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Sample records for pressure palsies hnpp

  1. Phenotype HNPP (Hereditary Neuropathy With Liability to Pressure Palsies) Induced by Medical Procedures.

    PubMed

    Kramer, Mark; Ly, Amy; Li, Jun

    2016-01-01

    The phenotype HNPP (hereditary neuropathy with liability to pressure palsies) is caused by heterozygous deletion of the PMP22 gene. HNPP is clinically characterized by asymmetric focal sensory loss and muscle weakness. Reports of HNPP have been rare. In this article, we report the case of an asymptomatic woman with the HNPP mutation. After undergoing total knee arthroplasty, she developed a footdrop with prolonged recovery. We concluded (a) that the HNPP mutation may carry a high risk for certain surgical procedures not expected to cause neurologic deficits in normal patients and (b) that humans with the HNPP mutation can be asymptomatic. Lack of symptoms can contribute to underrecognition of the disease. PMID:26761923

  2. Phenotype HNPP (Hereditary Neuropathy With Liability to Pressure Palsies) Induced by Medical Procedures

    PubMed Central

    Kramer, Mark; Ly, Amy; Li, Jun

    2016-01-01

    The phenotype HNPP (hereditary neuropathy with liability to pressure palsies) is caused by heterozygous deletion of the PMP22 gene. HNPP is clinically characterized by asymmetric focal sensory loss and muscle weakness. Reports of HNPP have been rare. In this article, we report the case of an asymptomatic woman with the HNPP mutation. After undergoing total knee arthroplasty, she developed a footdrop with prolonged recovery. We concluded (a) that the HNPP mutation may carry a high risk for certain surgical procedures not expected to cause neurologic deficits in normal patients and (b) that humans with the HNPP mutation can be asymptomatic. Lack of symptoms can contribute to underrecognition of the disease. PMID:26761923

  3. DNA analysis in Finnish patients with hereditary neuropathy with liability to pressure palsies (HNPP).

    PubMed Central

    Silander, K; Halonen, P; Sara, R; Kalimo, H; Falck, B; Savontaus, M L

    1994-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is a dominantly inherited disorder that presents as recurrent mononeuropathies precipitated by apparently trivial traumas. The presence of a deletion in 17p11.2 was analysed in 13 Finnish families with HNPP. The deletion was found in all patients who were neurologically and neurophysiologically confirmed to have HNPP. In the problematic cases the detection of the gene defect is the method of choice in the diagnosis of HNPP. Analysis of DNA can also be used to detect clinically unaffected family members. Images PMID:7931393

  4. Hereditary neuropathy with liability to pressure palsy (HNPP) in childhood: a case study emphasizing the relevance of detailed electrophysiological examination for suspected HNPP in the first decade.

    PubMed

    Bayrak, Ayşe Oytun; Battaloglu, Esra; Turker, Hande; Baris, Ibrahim; Oztas, Gurkan

    2009-06-01

    Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder characterized by recurrent mono-neuropathies secondary to minor trauma or compression. Whilst typical episodes of palsy generally become apparent during the second and the third decades, HNPP is rarely diagnosed in the first decade. We present the case of a 6-year-old patient to draw attention to the possibility of HNPP attacks in the first decade and the importance of detailed electrophysiological examination. PMID:18760885

  5. [Hereditary neuropathy with liability to pressure palsy (HNPP): a diagnostic trap].

    PubMed

    Ragois, P; Didailler, P; Rizzi, P

    2013-09-01

    The authors report two clinical cases of a rarely observed pathology in orthopedic surgery daily practice: hereditary neuropathy with liability to pressure palsy (HNPP), which leads to dysesthesiae, hypoesthesiae and regressive palsies. Onset, clinical signs and electromyographic abnormalities are described. Forensic consequences can occur in early postoperative period. Knowledge of this familial pathology allows precautionary measures at surgery and avoids unnecessary surgical revisions. PMID:23953745

  6. Characteristic features of hereditary neuropathy with liability to pressure palsy (HNPP) presenting with brachial plexopathy in soldiers.

    PubMed

    Kim, Kyoung-Eun

    2014-11-15

    A brachial plexus lesion is not common in hereditary neuropathy with liability to pressure palsy (HNPP). We report the clinical and electrodiagnostic features of young soldiers with HNPP presenting with brachial plexopathy. By reviewing 2year medical records from Korean military hospitals, we identified soldiers with brachial plexus lesions. Among them, patients diagnosed with HNPP were determined and clinical and electrophysiological findings were compared between HNPP and non-HNPP patients with a brachial plexus lesion. Thirteen patients (6.8%) were diagnosed with HNPP among 189 patients with a brachial plexus lesion. Push-ups, as either a punishment or an exercise, was the most frequent preceding event in HNPP patients (76.9%), whereas it was rare in non-HNPP patients. The distal motor latency of the median nerve showed the highest sensitivity (90.9%) and specificity (100%) for HNPP in patients with a brachial plexus lesion. In conclusion, HNPP should be suspected in patients with brachial plexopathy if brachial plexopathy develops after push-ups or if the distal motor latency of median nerves is prolonged. PMID:25175852

  7. Mutational analysis of Greek patients with suspected hereditary neuropathy with liability to pressure palsies (HNPP): a 15-year experience.

    PubMed

    Karadima, Georgia; Koutsis, Georgios; Raftopoulou, Maria; Karletidi, Karolina-Maria; Zambelis, Thomas; Karandreas, Nikolaos; Panas, Marios

    2015-06-01

    There has been limited information from population studies regarding the overall frequency of the common 1.5-Mb 17p11.2 deletion and even scarcer data regarding the overall frequency of PMP22 micromutations in patients with a clinical suspicion of hereditary neuropathy with liability to pressure palsies (HNPP). We have analysed 100 consecutive Greek patients referred for HNPP genetic testing over a 15-year period to our Neurogenetics Unit in Athens, a reference centre for all regions of Greece. All patients were screened for the 1.5-Mb deletion and a selected subgroup of deletion-negative patients for PMP22 micromutations. Mutation-positive and mutation-negative patients were compared for various clinical parameters. In total, 54 mutation-positive patients were identified. In index cases, the deletion frequency was 47.8%, and the PMP22 micromutation frequency was 2.2%. Within mutation-positive patients, the common deletion represented 95.7% and PMP22 micromutations 4.3% of cases. Two previously reported PMP22 micromutations (c.364_365delCC and c.79-2A>G) were detected. HNPP index cases had a 2.8-1 male-to-female ratio, similar to mutation-negative patients. A typical phenotype (recurrent or isolated palsies) was present in 82.4% of symptomatic HNPP cases, significantly higher than mutation-negative patients. Sensitivity of proposed electrophysiological diagnostic criteria for HNPP was calculated at 95.7% and specificity at 80.5%. In conclusion, the common HNPP deletion accounts for ∼50% and PMP22 micromutations for ∼2% of cases in a large consecutive cohort of patients with suspected HNPP. The mutational and phenotypic spectrum of HNPP is similar in the Greek population compared with other populations. Proposed electrophysiological diagnostic criteria perform satisfactorily in everyday clinical practice. PMID:26110377

  8. A report of hereditary neuropathy with liability to pressure palsy (HNPP) presenting with brachial plexopathy: the value of complete electrodiagnostic testing.

    PubMed

    Bulusu, Srinivas; McMillan, Hugh J

    2011-09-01

    Patients with hereditary neuropathy with liability to pressure palsy (HNPP) typically present with a mononeuropathy (particularly peroneal or ulnar palsy) or a brachial plexopathy. Careful electrodiagnostic testing has an important role in establishing the diagnosis of HNPP differentiating this condition from other inherited or acquired neuropathies as well as obviating the need for unnecessary surgeries. We present a case of a patient who presented with a painless brachial plexopathy who was found to have multiple sites of segmental demyelination on nerve conduction studies, consistent with HNPP. We review the clinical and electrodiagnostic features of HNPP including the key electrodiagnostic findings to screen for this disorder. PMID:21988036

  9. DTI Study of Cerebral Normal-Appearing White Matter in Hereditary Neuropathy With Liability to Pressure Palsies (HNPP).

    PubMed

    Wang, Wei-Wei; Song, Chun-Li; Huang, Liang; Song, Qing-Wei; Liang, Zhan-Hua; Wei, Qiang; Hu, Jia-Ni; Miao, Yan-Wei; Wu, Bing; Xie, Lizhi

    2015-10-01

    The majority of previous studies on hereditary neuropathy with liability to pressure palsies (HNPP) were focused on peripheral nerves, whereas cerebral alterations in HNPP have been less attended to. In this work, Diffusion tensor imaging (DTI) was used to detect the changes in WM, especially in the normal-appearing white matter (NAWM) in HNPP patients for its sensitivity in probing the microstructure of WM, the sensitive metric was searched for probing cerebral alterations and the regional distribution of cerebral abnormalities was identified. Twelve HNPP patients and 12 age- and gender-matched healthy controls underwent the conventional MRI, DTI scan, and electrophysiological examination. The conventional MRI images were first analyzed to identify abnormal intense regions and the NAWM regions. NAWM refers to the white matter regions that do not include the lesions on conventional MRI. The apparent diffusion coefficient and fractional anisotropy (FA) values of the NAWM were then measured and compared between patient and control groups. The sensitivity and specificity of 3 methods and the cerebral regional distribution of MR signal abnormalities were further analyzed. Hyperintense foci were observed on T2 weighted image and fluid attenuated inversion recovery images in 6 patients. Compared to the controls, FA values of the patients were significantly lower in bilateral frontal, orbitofrontal, and temporal NAWMs; whereas the electrophysiological examination results of patients and controls exhibited no statistically significant difference. The sensitivity of FA value was higher than that of electrophysiological examination and conventional MRI. The majority of abnormal signals on conventional MRI images and abnormal FA values were located in the frontal and temporal lobes. The results of our study show cerebral WM changes in HNPP patients. FA value in DTI has been shown to be sensitive to the cerebral microstructural changes in HNPP. The frontal lobe is the predilection site that is most involved in HNPP. PMID:26512614

  10. [Delayed paresis of the femoral nerve after total hip arthroplasty associated with hereditary neuropathy with liability to pressure palsies (HNPP)].

    PubMed

    Schuh, A; Dürr, V; Weier, H; Zeiler, G; Winterholler, M

    2004-07-01

    Delayed lesions of the femoral or sciatic nerve are a rare complication after total hip arthroplasty. Several cases in association with cement edges, scar tissue, broken cerclages, deep hematoma, or reinforcement rings have been published. We report about a 62-year-old female who developed a pure motor paresis of the quadriceps muscle 2 weeks after total hip arthroplasty. After electrophysiological evaluation had revealed an isolated femoral nerve lesion, revision of the femoral nerve was performed. During operative revision no pathologic findings could be seen. One week later the patient developed paralysis of the left wrist and finger extensors after using crutches. Electrophysiological evaluation revealed several nerve conduction blocks in physiological entrapments and the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP) was established. Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare disease with increased vulnerability of the peripheral nerve system with mostly reversible sensorimotor deficits. It should be taken into consideration in cases of atypical findings of compression syndromes of peripheral nerves or delayed neuropathy, e. g., after total hip arthroplasty. PMID:15083272

  11. Hereditary neuropathy with liability to pressure palsies in a Turkish patient (HNPP): a rare cause of entrapment neuropathies in young adults.

    PubMed

    Celik, Yahya; Kilinçer, Cumhur; Hamamcioğlu, M Kemal; Balci, Kemal; Birgili, Bariş; Cobanoğlu, Sebahattin; Utku, Ufuk

    2008-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant nerve disease usually caused by 1,5 Mb deletion on chromosome 17p11.2.2-p12, the region where the PMP-22 gene is located. The patients with HNPP usually have relapsing and remitting entrapment neuropathies due to compression. We present a 14-year-old male who had acute onset, right-sided ulnar nerve entrapment at the elbow. He had electrophysiological findings of bilateral ulnar nerve entrapments (more severe at the right side) at the elbow and bilateral median nerve entrapment at the wrist. Genetic tests of the patient demonstrated deletions in the 17p11.2 region. The patient underwent decompressive surgery for ulnar nerve entrapment at the elbow and completely recovered two months after the event. Although HNPP is extremely rare, it should be taken into consideration in young adults with entrapment neuropathies. PMID:18382985

  12. Hereditary neuropathy with liability to pressure palsy.

    PubMed

    Paprocka, Justyna; Kajor, Maciej; Jamroz, Ewa; Jezela-Stanek, Aleksandra; Seeman, Pavel; Marszał, Elzbieta

    2006-01-01

    Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disease with sensory and motor nerve palsies usually precipitated by trivial trauma or compression. In the majority of cases HNPP is caused by deletion of the peripheral myelin protein 22 gene (PMP22) on chromosome 17p11.2. The authors present a family case with genetically proven HNPP. PMID:17183456

  13. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated to hereditary neuropathy with liability to pressure palsies (HNPP) and revealed after influenza AH1N1 vaccination.

    PubMed

    Remiche, Gauthier; Abramowicz, Marc; Mavroudakis, Nicolas

    2013-12-01

    Neurological complications of AH1N1 vaccination such as Guillain-Barré syndrome were described in the previous years. Several reports suggest that hereditary neuropathies may be a predisposing factor for immune-mediated neuropathies. We report the case of a 54-year-old female who developed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) 5 weeks after AH1N1 vaccination. She had no previous neurological history, but neurophysiological features led us to suspect an underlying hereditary neuropathy. PMP22 gene analysis showed a typical deletion, confirming the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). We observed a significant clinical and neurophysiological improvement of the neuropathy after intravenous immunoglobulin treatment. This is, to our knowledge, the first reported case of CIDP potentially triggered by AH1N1 vaccination. This and previous observations suggest that genetic-determined neuropathies could predispose to the occurrence of immune-mediated neuropathies. One must recall the possibility of a superimposed hereditary neuropathy like HNPP in patients with a clinical presentation of CIDP, especially when positive family history or unexpected neurophysiological features are present. PMID:24146347

  14. Fibular nerve palsy after hip replacement: Not only surgeon responsibility. Hereditary neuropathy with liability to pressure palsies (HNPP) a rare cause of nerve liability.

    PubMed

    Logroscino, G; Del Tedesco, F; Cambise, C; Coraci, D; Donati, F; Santilli, V; Padua, L

    2016-06-01

    Mononeuropathy after surgery may occur and hereditary neuropathy with liability to pressure palsies is a possible pathological condition related to paresis after hip surgery. We present a case of 66-year-old man presenting severe weakness at inferior limb muscles after hip prosthesis revision. Clinic and electrophysiology showed severe right fibular nerve damage and ultrasound found a marked enlargement of the same nerve, associated with focal enlargements in other nerves. A diagnosis of hereditary neuropathy with liability to pressure palsies was suspected and confirmed by genetic test. The patient gradually recovered returning to a normal daily active life. Ultrasound was crucial for diagnosis. The suspicion and diagnosis of latent neuropathy, which can occur after surgical intervention, may lead to a better understand of the risks of the surgery, specific for the patient, and avoid the wrong attribution to surgical malpractice. PMID:27084090

  15. [Hereditary neuropathy with liability to pressure palsies (HNPP) in hand surgery: reminds and warn against a usually unrecognised disease].

    PubMed

    Lazar, C-C; Auquit-Auckbur, I; Milliez, P-Y

    2007-12-01

    Tomacula is a rare hereditary disease due to a deletion on chromosome 17. Clinical presentation varies but patients usually complain of recurrent paraesthesiaes and palsies related to compression or trauma of a peripheral nerve. Diagnosis is based on electrophysiological studies, nerve biopsies and genetic tests. Implications for the patient and family members are a genetic counselling and some simple preventive measures. Although there is no curative treatment for this neuropathy, surgery can be useful for decompression of nerves and neurolysis. However, the surgical act increases the risk of nerve damage. Knowing about the diagnosis can help the patient and the surgical team avoid causing lesions. PMID:17030391

  16. Hereditary Neuropathy with Liability to Pressure Palsies.

    PubMed

    Choi, Hyoung Won; Kuntz, Nancy L

    2015-11-01

    Investigators from 4 pediatric hospitals in Canada analyzed the clinical presentation and electrophysiological data of 12 children with hereditary neuropathy with liability to pressure palsies (HNPP), caused by PMP22 gene deletion. PMID:26933540

  17. Hereditary neuropathy with liability to pressure palsies occurring during military training.

    PubMed

    Delacour, H; Bompaire, F; Biale, L; Sallansonnet-Froment, M; Ceppa, F; Burnat, P

    2012-03-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant peripheral neuropathy characterized by recurrent isolated nerve palsies, which are precipitated by trivial compression and trauma. Although HNPP has been well-described in literature, it often goes unrecognized. We report a case of HNPP occurring during military training to promote recognition and proper management of this entity. PMID:22545374

  18. Screening of the 17p11.2--p12 region in a large cohort of patients with Charcot-Marie-Tooth (CMT) disease or hereditary neuropathy with liability to pressure palsies (HNPP).

    PubMed

    Kabzinska, D; Pierscinska, J; Kochanski, A

    2009-01-01

    Within the last decade, numerous methods have been applied to detect the most common mutation in patients affected with Charcot-Marie-Tooth (CMT) disease, i.e. submicroscopic duplication in the 17p11.2--p12 region. In 1993, another neuropathy - known as hereditary neuropathy with liability to pressure palsies (HNPP) - has been shown to be caused by a 17p11.2--p12 deletion. Historically, Southern blot analysis was the first approach to identify CMT1A duplication or HNPP deletion. This time- and labor-consuming method requires prior selection of DNA samples. In fact, only CMT patients affected with the demyelinating form of CMT1 have been screened for CMT1A duplication. After the 17p11.2--p12 duplication was identified in the CMT1 families, subsequent studies revealed additional axonal features in the patients harboring the 17p11.2--p12 duplication. Thus it seems reasonable to test all patients affected with CMT for the presence of the 17p11.2--p12 duplication. To evaluate the utility of real-time polymerase chain reaction (Q-PCR) and restriction fragment length polymorphism PCR (RFLP-PCR), we screened a large group of 179 families with the diagnosis of CMT/HNPP for the presence of the 17p11.2--p12 duplication/deletion. Due to a high frequency of CMT1A duplication in familial cases of CMT, we propose (in contrast to the previous studies) to perform Q-PCR analysis in all patients diagnosed with CMT. PMID:19638685

  19. Is carpal tunnel decompression warranted for HNPP?

    PubMed

    Earle, Nicholas; Zochodne, Douglas W

    2013-12-01

    The role of carpal tunnel decompression surgery for patients that have hereditary neuropathy with liability to pressure palsy (HNPP) is currently unknown. Since recovery from carpal tunnel compression is often associated with remyelination or nodal reconstruction rather than axonal regeneration, it is uncertain whether the PMP22 deletion associated with HNPP interrupts myelin or nodal reconstitution. We describe two patients with genetically confirmed HNPP and symptomatic carpal tunnel syndrome that had clinical and electrophysiological improvement after surgical decompression. The findings indicate a capacity for conduction repair in HNPP. They also suggest a need for further investigation and discussion around whether to offer carpal tunnel decompression to symptomatic HNPP patients. PMID:24171697

  20. Two cases of elderly-onset hereditary neuropathy with liability to pressure palsy manifesting bilateral peroneal nerve palsies.

    PubMed

    Kawaguchi, Norihiko; Suzuki, Naoki; Tateyama, Maki; Takai, Yoshiki; Misu, Tatsuro; Nakashima, Ichiro; Itoyama, Yasuto; Aoki, Masashi

    2012-09-01

    Hereditary neuropathy with liability to pressure palsy (HNPP) is characterized by recurrent focal neuropathies, which usually become symptomatic in the second or third decade of life. However, clinical phenotypic heterogeneity among patients with HNPP has recently been reported. Certain patients show polyneuropathy-type diffuse nerve injuries, whereas others remain asymptomatic at older ages. We present two cases of elderly-onset bilateral peroneal nerve palsies with diffuse muscle weakness in the lower limbs and glove-and-stocking type sensory disturbance. Both patients were diagnosed with HNPP by genetic analyses that detected deletions of chromosome 17p11.2 in peripheral myelin protein 22 genes. Their clinical courses suggested that the Japanese sitting style termed 'seiza', a way of sitting on the floor with the lower legs crossed under the thighs, was a precipitating factor for the bilateral peroneal nerve palsies. PMID:23185166

  1. Sonographic evaluation of the peripheral nerves in hereditary neuropathy with liability to pressure palsies: a case report.

    PubMed

    Kim, Se Hwa; Yang, Seung Nam; Yoon, Joon Shik; Park, Bum Jun

    2014-02-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominantly inherited disorder that affects peripheral nerves by repeated focal pressure. HNPP can be diagnosed by clinical findings, electrodiagnostic studies, histopathological features, and genetic analysis. Ultrasonography is increasingly used for the diagnosis of neuromuscular diseases; however, sonographic features of HNPP have not been clearly defined. We report the sonographic findings and comparative electrodiagnostic data in a 73-year-old woman with HNPP, confirmed by genetic analysis. The cross-sectional areas of peripheral nerves were enlarged at typical nerve entrapment sites, but enlargement at non-entrapment sites was uncommon. These sonographic features may be helpful for diagnosis of HNPP when electrodiagnostic studies are suspicious of HNPP and/or gene study is not compatible. PMID:24639934

  2. Sonographic Evaluation of the Peripheral Nerves in Hereditary Neuropathy With Liability to Pressure Palsies: A Case Report

    PubMed Central

    Kim, Se Hwa; Yoon, Joon Shik; Park, Bum Jun

    2014-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominantly inherited disorder that affects peripheral nerves by repeated focal pressure. HNPP can be diagnosed by clinical findings, electrodiagnostic studies, histopathological features, and genetic analysis. Ultrasonography is increasingly used for the diagnosis of neuromuscular diseases; however, sonographic features of HNPP have not been clearly defined. We report the sonographic findings and comparative electrodiagnostic data in a 73-year-old woman with HNPP, confirmed by genetic analysis. The cross-sectional areas of peripheral nerves were enlarged at typical nerve entrapment sites, but enlargement at non-entrapment sites was uncommon. These sonographic features may be helpful for diagnosis of HNPP when electrodiagnostic studies are suspicious of HNPP and/or gene study is not compatible. PMID:24639934

  3. Hereditary neuropathy with liability to pressure palsies: the first publication (1947).

    PubMed

    Koehler, Peter J

    2003-04-01

    The first report of hereditary neuropathy with liability to pressure palsies (HNPP) was published in Dutch in 1947. The present paper makes it accessible in the English language. de Jong described two families, but only the cases from the first family may be considered to have had HNPP. Five persons from three generations had recurring peripheral neuropathies. de Jong hypothesized a hereditary disposition for the occurrence of neuropathies, but suggested a relationship with low vitamin B(1) levels. PMID:12682341

  4. Progress in molecular diagnosis of Charcot-Marie-Tooth-disease type 1 (CMT 1, HMSN I) and hereditary neuropathy with liability to pressure palsies (HNPP) by fluorescence in situ hybridization (FISH)-detection of a potential genetic mosaicism

    SciTech Connect

    Bathke, K.; Liehr. T.; Ekici, A.

    1994-09-01

    We tested 20 CMT 1 patients characterized according to the criteria of the European CMT consortium by Southern hybridization of MspI restricted genomic DNA with probes pVAW409R1, pVAW412Hec and pEW401HE. In 11 of the 20 CMT 1 cases (55%), we observed a duplication in 17q11.2; one patient had a dinucleotide insertion in exon 6 of the PO-gene (5%). One HNPP case had a typical 17p11.2 deletion. Analysis of CA-repeats was performed with primers RM11GT and Mfd41; SSCP-analysis of the PO, PMP22 and Cx32-genes is in progress. FISH was carried out with probe pVAW409R1. 125 interphase nuclei were analyzed for each proband by counting the signals per nucleus. Normal cells show a characteristic distribution of signals: 1 signal in 5.9% of nuclei, 2 in 86.3% and 3 in 7.8%. A duplication is indicated by a shift to 3 signals in more than approximately 60% and 2 in less than 25% of the nuclei. In contrast, the 17p11.2 deletion of the HNPP patient shifts to 82.4% of nuclei with a single hybridization signal versus 14.4% with 2 signals. We detected one case with significantly abnormal distribution of interphase nuclei hybridization signals compared to cultures of normal cells and to those with 17p11.2 duplication or deletion: 3.2% nuclei revealed 1 signal, 48.0% two signals and 48.8% 3 signals, indicating a pathogenic but moderate dosis increase compared to the throughout duplicated cases. FISH with probe pVAW409R1 is a versatile tool to detect the HNPP deletion both in interphase nuclei and in metaphase chromosomes. In CMT 1 disease interphase nuclei are required for FISH analysis due to the small duplication of 1.5 Mbp. In contrast to Southern techniques, FISH is able to detect genetic mosaicism.

  5. Hereditary neuropathy with liability to pressure palsy: two cases of difficult diagnosis.

    PubMed

    Beydoun, Said R; Cho, Justin

    2013-09-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an inherited autosomal dominant disorder that causes a polyneuropathy with predisposition for involvement at sites of compression and is often underdiagnosed or misdiagnosed due to its heterogeneity in clinical and electrophysiological presentation. We report 2 cases of HNPP, which were initially diagnosed and treated as either an acquired demyelinating disorder or alternative inherited demyelinating disorder. Thorough evaluation of repeat electrodiagnostic studies and genetic testing confirmed the diagnosis of HNPP in both cases. One case showed the classic peripheral myelin protein 22 (PMP22) deletion and the other case showed a previously reported single base pair deletion at Leu145 causing a frameshift mutation at the PMP22 gene. These cases underscore the difficulty of diagnosing HNPP, because of the variations in clinical and electrophysiological findings and reinforce the importance of a combination high index of clinical suspicion, electrodiagnostic testing, and genetic testing to make the diagnosis. PMID:23965407

  6. Beneficial use of steroids in hereditary neuropathy with liability to pressure palsy.

    PubMed

    Heng, Hock S; Tang, Shan S; Goyal, Sushma; Wraige, Elizabeth A; Lim, Ming J

    2012-02-01

    Management of hereditary neuropathy with liability to pressure palsy (HNPP) is primarily conservative, aimed at preventing nerve injury by avoiding trauma or other potential aggravating factors. No pharmacological treatment is known to be beneficial. We describe two adolescents, one with HNPP (male; aged 15y) and another with a clinical picture suggestive of HNPP (genetically unconfirmed; female; aged 14y), who showed considerable improvement of their symptoms after receiving corticosteroid therapy. Both individuals were symptomatic for at least 5 months before the treatment. Following corticosteroids, both individuals demonstrated rapid improvement leading to near-complete recovery of muscle power. Clinical improvement after corticosteroid therapy has been reported in some individuals with other hereditary neuropathies. Our cases demonstrate that corticosteroid therapy may also be beneficial in individuals with HNPP who have a protracted or incomplete course of recovery. PMID:22098098

  7. Hereditary neuropathy with liability to pressure palsies. Diagnosis in the first family (1947) confirmed.

    PubMed

    Koehler, Peter J; Baas, Frank

    2012-12-01

    In 1947, the Dutch neurologist De Jong published the first family with, what later would be called, hereditary neuropathy with liability to pressure palsies (HNPP). We recently found a new case from this family and were able to confirm the diagnosis by DNA analysis. PMID:23279343

  8. Hereditary neuropathy with liability to pressure palsies: case report and discussion.

    PubMed

    Grossman, Marc J; Feinberg, Joseph; DiCarlo, Edward F; Birchansky, Sherri B; Wolfe, Scott W

    2007-09-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an uncommon diagnosis that should be considered in patients with multiple compressive neuropathies. We present the case of a woman who presented with bilateral hand numbness and weakness. Electrodiagnostic testing revealed bilateral carpal tunnel syndrome, bilateral ulnar neuropathy at the elbow, left peroneal neuropathy at the fibular head, and a primarily demyelinating generalized sensorimotor neuropathy. Subsequent genetic testing identified a deletion at chromosome 17p11.2 to confirm the diagnosis of HNPP. Treatment of this largely self-limiting disease is controversial, and this patient suffered minimal disability with treatment including splinting and surgical releases. PMID:18751796

  9. Electrophysiological comparison between males and females in HNPP.

    PubMed

    Manganelli, Fiore; Pisciotta, Chiara; Dubbioso, Raffaele; Maruotti, Valerio; Iodice, Rosa; Notturno, Francesca; Ruggiero, Lucia; Vitale, Carmine; Nolano, Maria; Uncini, Antonino; Santoro, Lucio

    2013-08-01

    Some evidences highlighted a higher clinical expression of hereditary neuropathy with liability to pressure palsy (HNPP) in males, and a higher load of traumatic nerve injuries due to different occupational activity has been invoked to explain this observation. It is unknown whether this increased clinical impairment corresponds to a greater electrophysiological involvement. Thus, we compared clinical and electrophysiological features between men and women in a large cohort of HNPP patients. Nerve palsies and electrophysiological abnormalities were more frequent in men, and electrophysiological findings which differentiated males from females did not show any age-related worsening. In conclusion, our findings showed a higher clinical and electrophysiological involvement in males which does not seem related to different cumulative nerve damage over time. We believe that the higher disease expression may increase the chance to detect the disease in males and, thereby, to underestimate the HNPP diagnosis in females. PMID:23207550

  10. Inherited focal, episodic neuropathies: hereditary neuropathy with liability to pressure palsies and hereditary neuralgic amyotrophy.

    PubMed

    Chance, Phillip F

    2006-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or "sausage-like" formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found. PMID:16775374

  11. Clinical, electrophysiological and magnetic resonance findings in a family with hereditary neuropathy with liability to pressure palsies caused by a novel PMP22 mutation.

    PubMed

    Yurrebaso, Izaskun; Casado, Oscar L; Barcena, Joseba; Perez de Nanclares, Guiomar; Aguirre, Urko

    2014-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is a disorder mainly caused by a 1.5-Mb deletion at 17p11.2-12 (and in some rare cases by point mutations) and clinically associated with recurrent painless palsies. Here, we performed electrophysiological (motor, sensory and terminal latency index), MRI and genetic studies in a family referred for ulnar neuropathy with pain. Surprisingly, we found typical neurophysiological features of HNPP (prolongation of distal motor latencies and diffuse SNCV slowing with significant slowing of motor nerve conduction velocities). Besides, the proband presented conduction block in left ulnar, left median and both peroneal nerves. MRI findings were consistent with an underlying neuropathy. Molecular studies identified a novel frameshift mutation in PMP22 confirming the diagnosis of HNPP. Our data suggest that neurophysiological studies are essential to characterize underdiagnosed HNPP patients referred for peripheral neuropathy. Our experience shows that MRI could be a complementary tool for the diagnosis of these patients. PMID:24239057

  12. Partial Gene Deletions of PMP22 Causing Hereditary Neuropathy with Liability to Pressure Palsies

    PubMed Central

    Cho, Sun-Mi; Kim, Yoonjung; Lee, Sang Guk; Yang, Jin-Young

    2014-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal neuropathy that is commonly caused by a reciprocal 1.5 Mb deletion on chromosome 17p11.2, at the site of the peripheral myelin protein 22 (PMP22) gene. Other patients with similar phenotypes have been shown to harbor point mutations or small deletions, although there is some clinical variation across these patients. In this report, we describe a case of HNPP with copy number changes in exon or promoter regions of PMP22. Multiplex ligation-dependent probe analysis revealed an exon 1b deletion in the patient, who had been diagnosed with HNPP in the first decade of life using molecular analysis. PMID:25506001

  13. Partial Gene Deletions of PMP22 Causing Hereditary Neuropathy with Liability to Pressure Palsies.

    PubMed

    Cho, Sun-Mi; Hong, Bo Young; Kim, Yoonjung; Lee, Sang Guk; Yang, Jin-Young; Kim, Juwon; Lee, Kyung-A

    2014-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal neuropathy that is commonly caused by a reciprocal 1.5 Mb deletion on chromosome 17p11.2, at the site of the peripheral myelin protein 22 (PMP22) gene. Other patients with similar phenotypes have been shown to harbor point mutations or small deletions, although there is some clinical variation across these patients. In this report, we describe a case of HNPP with copy number changes in exon or promoter regions of PMP22. Multiplex ligation-dependent probe analysis revealed an exon 1b deletion in the patient, who had been diagnosed with HNPP in the first decade of life using molecular analysis. PMID:25506001

  14. Hereditary neuropathy with liability to pressure palsies presenting with sciatic neuropathy.

    PubMed

    Topakian, Raffi; Wimmer, Sibylle; Pischinger, Barbara; Pichler, Robert

    2014-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant disorder associated with recurrent mononeuropathies following compression or trivial trauma. Reports on sciatic neuropathy as the presenting manifestation of HNPP are very scarce. We report on a 21-year-old previously healthy man who was admitted with sensorimotor deficits in his left leg. He had no history of preceding transient episodes of weakness or sensory loss. Clinical and electrophysiological examinations were consistent with sciatic neuropathy. Cerebrospinal fluid investigation and MRI of the nerve roots, plexus, and sciatic nerve did not indicate the underlying aetiology. When extended electrophysiological tests revealed multiple subclinical compression neuropathies in the upper limbs, HNPP was contemplated and eventually confirmed by genetic testing. PMID:25326571

  15. Hereditary neuropathy with liability to pressure palsy presenting with hand drop in a young child.

    PubMed

    Sobreira, Inês; Sousa, Cátia; Raposo, Ana; Soares, M Rita; Soudo, Ana; Dias, Ana Isabel

    2012-01-01

    Hereditary neuropathy with liability to pressure palsy (HNPP) results from the deletion of the PMP22 gene in chromosome 17p11.2. Clinically, it presents with painless pressure palsies, typically in the 2nd and 3rd decades of life, being a rare entity in childhood. We present the case study of a six-year-old male child who presented with left hand drop that he kept for over four weeks. Electrophysiological studies suggested HNPP and genetic studies confirmed it. With this paper, we pretend to create awareness to this entity as a diagnosis to be considered in a child with painless monoparesis and to emphasize the importance of electrophysiological studies in the diagnosis. PMID:22953141

  16. Hereditary Neuropathy with Liability to Pressure Palsy Presenting with Hand Drop in a Young Child

    PubMed Central

    Sobreira, Inês; Sousa, Cátia; Raposo, Ana; Soares, M. Rita; Soudo, Ana; Dias, Ana Isabel

    2012-01-01

    Hereditary neuropathy with liability to pressure palsy (HNPP) results from the deletion of the PMP22 gene in chromosome 17p11.2. Clinically, it presents with painless pressure palsies, typically in the 2nd and 3rd decades of life, being a rare entity in childhood. We present the case study of a six-year-old male child who presented with left hand drop that he kept for over four weeks. Electrophysiological studies suggested HNPP and genetic studies confirmed it. With this paper, we pretend to create awareness to this entity as a diagnosis to be considered in a child with painless monoparesis and to emphasize the importance of electrophysiological studies in the diagnosis. PMID:22953141

  17. Hereditary Neuropathy with Liability to Pressure Palsies Masked by Previous Gunshots and Tuberculosis.

    PubMed

    Gencik, Martin; Finsterer, Josef

    2015-01-01

    Objectives. Although hereditary neuropathy with liability to pressure palsies (HNPP) presents with a distinct phenotype on history, clinical exam, and nerve conduction studies, it may be masked if diagnostic work-up suggests other causes. Case Report. In a 37-year-old male with pseudoradicular lumbar pain, neurological exam revealed sore neck muscles, peripheral facial nerve palsy, right anacusis and left hypoacusis, hemihypesthesia of the right face, mild distal quadriparesis, diffuse wasting, and generally reduced tendon reflexes. He had a history of skull fracture due to a gunshot behind the right ear and tuberculosis for which he had received adequate treatment for 3 years; MRI revealed a disc prolapse at C6/7 and Th11/12. Nerve conduction studies were indicative of demyelinating polyneuropathy with conduction blocks. Despite elevated antinuclear antibodies and elevated CSF-protein, HNPP was diagnosed genetically after having excluded vasculitis, CIDP, radiculopathy, and the side effects of antituberculous treatment. Conclusions. HNPP may manifest with mild, painless, distal quadriparesis. The diagnosis of HNPP may be blurred by a history of tuberculosis, tuberculostatic treatment, hepatitis, and the presence of elevated CSF-protein. PMID:26640726

  18. Hereditary Neuropathy with Liability to Pressure Palsies Masked by Previous Gunshots and Tuberculosis

    PubMed Central

    Gencik, Martin; Finsterer, Josef

    2015-01-01

    Objectives. Although hereditary neuropathy with liability to pressure palsies (HNPP) presents with a distinct phenotype on history, clinical exam, and nerve conduction studies, it may be masked if diagnostic work-up suggests other causes. Case Report. In a 37-year-old male with pseudoradicular lumbar pain, neurological exam revealed sore neck muscles, peripheral facial nerve palsy, right anacusis and left hypoacusis, hemihypesthesia of the right face, mild distal quadriparesis, diffuse wasting, and generally reduced tendon reflexes. He had a history of skull fracture due to a gunshot behind the right ear and tuberculosis for which he had received adequate treatment for 3 years; MRI revealed a disc prolapse at C6/7 and Th11/12. Nerve conduction studies were indicative of demyelinating polyneuropathy with conduction blocks. Despite elevated antinuclear antibodies and elevated CSF-protein, HNPP was diagnosed genetically after having excluded vasculitis, CIDP, radiculopathy, and the side effects of antituberculous treatment. Conclusions. HNPP may manifest with mild, painless, distal quadriparesis. The diagnosis of HNPP may be blurred by a history of tuberculosis, tuberculostatic treatment, hepatitis, and the presence of elevated CSF-protein. PMID:26640726

  19. Hereditary neuropathy with liability to pressure palsy combined with schwannomas of the median and medial plantar nerves.

    PubMed

    Heckmann, Josef G; Dütsch, Matthias; Buslei, Ralf

    2007-01-01

    A 42-year-old woman was surgically treated for carpal tunnel syndrome, revealing schwannoma of the median nerve. A year later, she developed a tarsal tunnel syndrome. At time of this diagnosis, hereditary neuropathy with liability to pressure palsies (HNPP) was diagnosed genetically and a schwannoma of the medial plantar nerve was treated surgically. The occurrence of HNPP and schwannomas in the same patient might be purely coincidental, but it is tempting to speculate that they share a common genetic basis. PMID:16969831

  20. An abnormal mRNA produced by a novel PMP22 splice site mutation associated with HNPP

    PubMed Central

    Bellone, E; Balestra, P; Ribizzi, G; Schenone, A; Zocchi, G; Maria, E Di; Ajmar, F; Mandich, P

    2006-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant, demyelinating neuropathy. Point mutations in the PMP22 gene are a rare cause of HNPP. A novel PMP22 splice site mutation (c.179+1 G?C) is reported in an HNPP family. By reverse transcriptase?polymerase chain reaction experiments, this mutation was shown to cause the synthesis of an abnormal mRNA in which a premature stop codon probably produces a truncated non?functional protein. PMID:16199442

  1. Hereditary neuropathy with liability to pressure palsies in childhood: Case series and literature update.

    PubMed

    Chrestian, Nicolas; McMillan, Hugh; Poulin, Chantal; Campbell, Craig; Vajsar, Jiri

    2015-09-01

    Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) is a rare condition in childhood with a diverse range of clinical presentations. We analyzed the clinical presentation and electrophysiological data of 12 children with a confirmed PMP22 gene deletion and reviewed the published reports of HNPP in children and compared our data with the reports from the literature review. Peroneal palsy was the most common presentation (42%) followed by brachial plexus palsy in 25% of our cases. Nerve conduction studies were always suggestive of the diagnosis demonstrating 3 major patterns: multifocal demyelination at the area of entrapment, generalized sensory-motor polyneuropathy and a combination of the two first patterns in a vast majority (60%). Surprisingly, there was bilateral or unilateral electrophysiological entrapment of the median nerve at the carpal tunnel in all our patients. The clinical presentation of HNPP in childhood is heterogeneous and electrophysiological findings are helpful in establishing the diagnosis. Any unexplained mononeuropathy or multifocal neuropathy should lead to PMP22 gene testing to look for the deletion. Early diagnosis is important in order to facilitate appropriate genetic counseling and also for the appropriate care for these patients. PMID:26189194

  2. A novel point mutation in PMP22 gene in an Italian family with hereditary neuropathy with liability to pressure palsies.

    PubMed

    Muglia, Maria; Patitucci, Alessandra; Rizzi, Romana; Ungaro, Carmine; Conforti, Francesca Luisa; Gabriele, Anna Lia; Magariello, Angela; Mazzei, Rosalucia; Motti, Luisa; Sabadini, Rossella; Sprovieri, Teresa; Marcello, Norina; Quattrone, Aldo

    2007-12-15

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant inherited disorder characterized by recurrent sensory or motor dysfunction. In 85% of HNPP cases the genetic defect is a 1.4 Mb deletion on chromosome 17p11.2, encompassing the PMP22 gene. Point mutations in the PMP22 gene responsible for HNPP phenotypes are rare. We investigated a 17-years-old girl who led to our detecting a novel mutation in PMP22 gene. The mutation was also detected in her father and corresponded to a deletion of one tymidine at position 11 in exon2 (c.11delT). This novel mutation creates a shift on the reading frame starting at codon 4 and leads to the introduction of a premature stop at codon 6. PMID:17707409

  3. Sonographic and electrodiagnostic features of hereditary neuropathy with liability to pressure palsies.

    PubMed

    Ginanneschi, Federica; Filippou, Georgios; Giannini, Fabio; Carluccio, Maria A; Adinolfi, Antonella; Frediani, Bruno; Dotti, Maria T; Rossi, Alessandro

    2012-12-01

    In hereditary neuropathy with liability to pressure palsies (HNPP), the increase in distal motor latencies (DMLs) is often out of proportion to the slowing of conduction velocities, but the pathophysiological mechanism is still unclear. We used a combined electrophysiological and ultrasonographic (US) approach to provide insight into this issue. Twelve HNPP subjects underwent extensive electrophysiological studies and US measurements of the cross-sectional area (CSA) of several peripheral nerves. US nerve enlargement was only observed in the carpal tunnel, Guyon's canal, the elbow and the fibular head. We did not observe US abnormalities at sites where nerve entrapment is uncommon. An increase in DMLs was observed regardless of US nerve enlargement. The increased nerve CSA only in common sites of entrapment likely reflected the well-documented nerve vulnerability to mechanical stress in HNPP. No morphometric changes were seen in the distal nerve segments where compression/entrapment is unlikely, despite the fact that the DMLs were increased. These data suggest that factors other than mechanical stress are responsible for the distal slowing of action potential propagation. We speculate that a mixture of mechanical insults and an axon-initiated process in the distal nerves underlies the distal slowing and/or conduction failure in HNPP. PMID:23279340

  4. New mutations in CMT 1 and HNPP

    SciTech Connect

    Vandenberghe, A.; Boucherat, M.; Bonnebouche, C.

    1994-09-01

    The majority of mutations in CMT 1 (Charcot-Marie-Tooth disease type 1) are due to a duplication of a 1.5 Mb fragment from chromosome 17 containing the PMP22 myelin gene. In addition, micromutations are found in the genes for PMP22 and myelin Po. We collected data from over one hundred families with a duplication in 17p11.2. In about 10% of these families, a de novo mutation was observed. All parents were clinically examined as normal and correct paternity was confirmed. Some families were informative for polymorphic probes located in the duplicated region, and we could deduce a majority of new mutations to be from paternal origin. HNPP (hereditary neuropathy with liability to pressure palsies) is believed to be the reciprocal product of an unequal crossing over underlying the CMT 1 mutation and is due to a deletion of the 1.5 Mb fragment. One new HNPP mutation was found among 7 deleted HNPP families. This mutation is of paternal origin. Clinically assigned CMT 1 patients without a duplication are screened for micromutations applying the SSCP technique. In one family, a de novo mutation was found in the gene for Po.

  5. Hereditary neuropathy with liability to pressure palsies and amyotrophic lateral sclerosis.

    PubMed

    Bhatt, Archit; Farooq, Muhammad U; Aburashed, Rany; Kassab, Mounzer Y; Majid, Arshad; Bhatt, Shaila; Naravetla, Bharath; Dhaliwal, Gurmail

    2009-06-01

    A 56-year-old male with recurrent painless focal neuropathies and a family history of peripheral neuropathy of unknown etiology presented with progressively worsening of impaired sensations and weakness in his lower extremities. His initial electrodiagnostic evaluation was suggestive of severe sensory and motor peripheral polyneuropathy. The genetic testing was performed for familial causes of peripheral neuropathy as there was a family history of peripheral neuropathy of unknown etiology. The patient was found to have 1.5-Mb deletion in the PMP22 gene which was confirmatory of hereditary neuropathy with liability to pressure palsies (HNPP). He developed progressive upper and lower extremity weakness, bulbar dysfunction and widespread fasciculations during the course of his illness. He was subsequently diagnosed with amyotrophic lateral sclerosis (ALS). This is the second reported case of HNPP associated with ALS. We discuss significant clinical and electrodiagnostic findings of this interesting case. PMID:19238316

  6. A rare genetic disorder in the differential diagnosis of the entrapment neuropathies: hereditary neuropathy with liability to pressure palsies.

    PubMed

    Koc, Filiz; Güzel, Rengin; Benlidayi, Ilke Coskun; Yerdelen, Deniz; Güzel, Irfan; Sarica, Yakup

    2006-04-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant, slowly progressive neuromuscular disorder, which is characterized by recurrent acute peripheral nerve palsies. Electrophysiological studies show decreased motor and sensory conduction velocities in both clinically affected and unaffected nerves. Focal thickening of myelin sheath with sausage-like formation, also called tomacula, is seen in nerve biopsies. In genetic studies, 1.5-Mb deletion on chromosome 17p11.2 is detected in approximately 85% of HNPP cases and point mutations are determined in some cases. We describe a 26-year-old man who had a 6-month history of paresthesia in the little fingers of his hands. He was diagnosed with HNPP by neurologic examination, and electrophysiological and histopathologic studies. Studies in his mother and one brother also showed entrapment neuropathy. However, no deletions or point mutations were determined in this family. Other genetic defects apart from the known ones might be present in this disease. The most frequent entrapment syndrome, carpal tunnel syndrome, is also seen in this disease, so physicians dealing with musculoskeletal problems should be alert about this subject. Awareness of HNPP may help avoid unnecessary operative interventions. PMID:16601541

  7. Genetics Home Reference: hereditary neuropathy with liability to pressure palsies

    MedlinePlus

    ... hereditary neuropathy with liability to pressure palsies hereditary neuropathy with liability to pressure palsies Enable Javascript to ... Print All Open All Close All Description Hereditary neuropathy with liability to pressure palsies is a disorder ...

  8. A 1.5 Mb submicroscopic deletion in 17p11.2-p12 is frequently observed in Italian families with hereditary neuropathy with liability to pressure palsies

    SciTech Connect

    Lorenzetti, D.; Roa, B.B.; Abbas, N.E.

    1994-09-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder characterized by recurrent mononeuropathies that was recently associated with a 1.5 Mb deletion in chromosome 17p11.2-p12. Duplication of the same region is known to be associated with Charcot-Marie-Tooth disease type 1A (CMT1A), a more severe peripheral neuropathy characterized by symmetrically slowed nerve conduction velocity. The CMT1A duplication and HNPP deletion are reciprocal recombination products involving a repeat element (CMT1A-REP) which flanks the 1.5 Mb region involved in the duplication/deletion. Patients from 9 unrelated HNPP Italian families were clinically, electrophysiologically and histologically evaluated. Families were typed with a polymorphic (CA){sub n} repeat and with RFLPs corresponding to loci D17S122, D17S125 and D17S61, which all map within the deleted region. Lack of allelic transmission from affected parent to affected offspring was observed in four informative families, suggesting the presence of deletion. Southern blot analysis of EcoRI digested genomic DNA from HNPP patients and control subjects was performed using a probe mapping within the CMT1A-REP elements. A reduced hybridization signal of a 6.0 kb EcoRI fragment, mapping within the distal CMT1A-REP, was observed in all HNPP patients suggesting the loss of one copy of this fragment in the HNPP-deleted chromosome. PFGE analysis of SacII digested genomic DNA from selected HNPP subjects showed the presence of a junction fragment which has previously been found in association with the 1.5 Mb HNPP deletion. Evidence for deletion could be demonstrated in all 9 families suggesting that the 17p11.2-p12 deletion is commonly associated with HNPP.

  9. A 1.5-Mb deletion in 17p11.2-p12 is frequently observed in Italian families with hereditary neuropathy with liability to pressure palsies

    SciTech Connect

    Lorenzetti, D.; Pandolfo, M. |; Pareyson, D.; Sghirlanzoni, A.; Di Donato, S.; Roa, B.B.; Abbas, N.E.; Lupski, J.R.

    1995-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder characterized by recurrent mononeuropathies. A 1.5-Mb deletion in chromosome 17p11.2-p12 has been associated with HNPP. Duplication of the same 1.5-Mb region is known to be associated with Charcot-Marie-Tooth disease type 1 (CMT1A), a more severe peripheral neuropathy characterized by symmetrically slowed nerve conduction velocity (NCV). The CMT1A duplication and HNPP deletion appear to be the reciprocal products of a recombination event involving a repeat element (CMT1A-REP) that flanks the 1.5-Mb region involved in the duplication/deletion. Patients from nine unrelated Italian families who were diagnosed with HNPP on the basis of clinical, electrophysiological, and histological evaluations were analyzed by molecular methods for DNA deletion on chromosome 17p. In all nine families, Southern analysis using a CMT1A-REP probe detected a reduced hybridization signal of a 6.0-kb EcoRI fragment mapping within the distal CMT1A-REP, indicating deletion of one copy of CMT1A-REP in these HNPP patients. Families were also typed with a polymorphic (CA){sub n} repeat and with RFLPs corresponding to loci D17S122, D17S125, and D17S61, which all map within the deleted region. Lack of allelic transmission from affected parent to affected offspring was observed in four informative families, providing an independent indication for deletion. Furthermore, pulsed-field gel electrophoresis analysis of SacII-digested genomic DNA detected junction fragments specific to the 1.5-Mb HNPP deletion in seven of nine Italian families included in this study. These findings suggest that a 1.5-Mb deletion on 17p11.2-p12 is the most common mutation associated with HNPP. 51 refs., 5 figs., 1 tab.

  10. A 1.5-Mb deletion in 17p11.2-p12 is frequently observed in Italian families with hereditary neuropathy with liability to pressure palsies.

    PubMed

    Lorenzetti, D; Pareyson, D; Sghirlanzoni, A; Roa, B B; Abbas, N E; Pandolfo, M; Di Donato, S; Lupski, J R

    1995-01-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder characterized by recurrent mononeuropathies. A 1.5-Mb deletion in chromosome 17p11.2-p12 has been associated with HNPP. Duplication of the same 1.5-Mb region is known to be associated with Charcot-Marie-Tooth disease type 1 (CMT1A), a more severe peripheral neuropathy characterized by symmetrically slowed nerve conduction velocity (NCV). The CMT1A duplication and HNPP deletion appear to be the reciprocal products of a recombination event involving a repeat element (CMT1A-REP) that flanks the 1.5-Mb region involved in the duplication/deletion. Patients from nine unrelated Italian families who were diagnosed with HNPP on the basis of clinical, electrophysiological, and histological evaluations were analyzed by molecular methods for DNA deletion on chromosome 17p. In all nine families, Southern analysis using a CMT1A-REP probe detected a reduced hybridization signal of a 6.0-kb EcoRI fragment mapping within the distal CMT1A-REP, indicating deletion of one copy of CMT1A-REP in these HNPP patients. Families were also typed with a polymorphic (CA)n repeat and with RFLPs corresponding to loci D17S122, D17S125, and D17S61, which all map within the deleted region. Lack of allelic transmission from affected parent to affected offspring was observed in four informative families, providing an independent indication for deletion. Furthermore, pulsed-field gel electrophoresis analysis of SacII-digested genomic DNA detected junction fragments specific to the 1.5-Mb HNPP deletion in seven of nine Italian families included in this study. These findings suggest that a 1.5-Mb deletion on 17p11.2-p12 is the most common mutation associated with HNPP. PMID:7825607

  11. The LITAF/SIMPLE I92V sequence variant results in an earlier age of onset of CMT1A/HNPP diseases.

    PubMed

    Sinkiewicz-Darol, Elena; Lacerda, Andressa Ferreira; Kostera-Pruszczyk, Anna; Potulska-Chromik, Anna; Sokołowska, Beata; Kabzińska, Dagmara; Brunetti, Craig R; Hausmanowa-Petrusewicz, Irena; Kochański, Andrzej

    2015-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) represent the most common heritable neuromuscular disorders. Molecular diagnostics of CMT1A/HNPP diseases confirm clinical diagnosis, but their value is limited to the clinical course and prognosis. However, no biomarkers of CMT1A/HNPP have been identified. We decided to explore if the LITAF/SIMPLE gene shared a functional link to the PMP22 gene, whose duplication or deletion results in CMT1A and HNPP, respectively. By studying a large cohort of CMT1A/HNPP-affected patients, we found that the LITAF I92V sequence variant predisposes patients to an earlier age of onset of both the CMT1A and HNPP diseases. Using cell transfection experiments, we showed that the LITAF I92V sequence variant partially mislocalizes to the mitochondria in contrast to wild-type LITAF which localizes to the late endosome/lysosomes and is associated with a tendency for PMP22 to accumulate in the cells. Overall, this study shows that the I92V LITAF sequence variant would be a good candidate for a biomarker in the case of the CMT1A/HNPP disorders. PMID:25342198

  12. Major histocompatibility complex class II expression and macrophage responses in genetically proven Charcot-Marie-Tooth type 1 and hereditary neuropathy with liability to pressure palsies.

    PubMed

    Stoll, G; Gabreëls-Festen, A A; Jander, S; Müller, H W; Hanemann, C O

    1998-11-01

    This study examined major histocompatibility complex (MHC) class II expression and macrophage infiltration in sural nerve biopsies from patients with genetically proven Charcot-Marie-Tooth (CMT) 1A and 1B and hereditary neuropathy with liability to pressure palsies (HNPP) by immunocytochemistry. In both young and older patients with duplication of the PMP22 gene, MHC class II expression was consistently up-regulated and not closely related to the extent of macrophage infiltration. On the other hand, MHC class II expression was more variable in CMT1A and CMT1B caused by point mutations and in HNPP. The extent of nerve pathology as assessed by teased fiber preparations or electron microscopy was not predictive for the degree of MHC class II expression in CMT1/HNPP. We conclude that MHC class II up-regulation is a common feature in hereditary neuropathies. As shown for the animal model of globoid cell dystrophy, it is conceivable that increased expression of MHC class II molecules in CMT1 and HNPP accelerates nerve pathology. PMID:9771665

  13. Hereditary neuropathy with liability to pressure palsy: a brief review with a case report.

    PubMed

    Rana, Abdul Qayyum; Masroor, Mohamed Sufian

    2012-03-01

    Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) is an autosomal dominant disorder and is usually characterized by episodes of recurrent and painless focal motor and sensory peripheral mononeuropathy. This condition is usually localized around areas of entrapment (predominantly the wrists, knees, elbows, and shoulders). The genetic locus of the disease is chromosome 17p12. A deletion of the PMP22 gene results in the lack of peripheral myelin protein, a key component to the myelin sheet of peripheral nerves. However, this disease may be completely asymptomatic until an event, such as a minor trauma, triggers these episodes, as seen in our presented case report. The diagnosis of HNPP can be somewhat challenging, as other diseases, such as Charcot-Marie-Tooth disease type 1A (CMT) and Hereditary Neuralgic Amyotrophy (HNA) must be included in the differential diagnosis due to their overlapping clinical features. There are currently no treatments to cure the disease, but therapies seek to alleviate the symptoms and recurring episodes. PMID:22023293

  14. Prevalence of the 1.5-Mb 17p deletion in families with hereditary neuropathy with liability to pressure palsies.

    PubMed

    Mariman, E C; Gabreëls-Festen, A A; van Beersum, S E; Valentijn, L J; Baas, F; Bolhuis, P A; Jongen, P J; Ropers, H H; Gabreëls, F J

    1994-10-01

    Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder of the peripheral nerves leading to increased susceptibility to mechanical traction or compression. Some patients have been shown to be carriers of a 1.5-Mb deletion in chromosome 17p11.2, which corresponds to the duplicated region present in most patients with Charcot-Marie-Tooth disease type 1A. Recently, evidence has been presented that the deletion is not the only cause of HNPP. To determine the prevalence of the 1.5-Mb deletion, we have examined 22 unrelated families with HNPP in the following two ways: by polymerase chain reaction analysis of marker loci D17S122 and D17S261, and by gene dosage measurements with DNA probes for D17S122 (VAW409R3a) and D17S125 (VAW412R3a) and for the PMP-22 gene. The efficiency and sensitivity of these methods is discussed. Our results show that the prevalence of the 17p deletion in our families with HNPP is 68%. One patient, presenting as a sporadic case, was found to be affected by a de novo deletion in the paternal chromosome. Single-strand conformation analysis of the protein-coding region of the PMP-22 gene did not reveal any mutation in patients from the 7 families lacking the 17p deletion. As a group, these families could not be distinguished by clinical, electrophysiological, or morphological features from the families with the deletion. PMID:7944298

  15. Clinical and neurophysiological features of the hereditary neuropathy with liability to pressure palsy due to the 17p11.2 deletion.

    PubMed

    Oliveira, Aline Pinheiro Martins de; Pereira, Raquel Campos; Onofre, Patrícia Toscano; Marques, Vanessa Daccach; Andrade, Gilberto Brown de; Barreira, Amilton Antunes; Marques Junior, Wilson

    2016-02-01

    The hereditary neuropathy with liability to pressure palsies (HNPP) is an autossomal dominant disorder manifesting recurrent mononeuropathies. Objective Evaluate its clinical and nerve conduction studies (NCS) characteristics, searching for diagnostic particularities. Method We reviewed the neurological manifestations of 39 and the NCS of 33 patients. Results Family history was absent in 16/39 (41%). The onset complaints were weakness in 24, pain in 6, sensory deficit in 5 and paresthesias in 4. Pain was seen in 3 other patients. The following neuropathy patterns were found: multiple mononeuropathy (26), mononeuropathy (7), chronic sensorimotor polyneuropathy (4), chronic sensory polyneuropathy (1) and unilateral brachial plexopathy (1). NCS showed a sensorimotor neuropathy with focal conduction slowing in 31, two had mononeuropathy and another brachial plexopathy. Conclusion HNPP presentation is variable and may include pain. The most frequent pattern is of an asymmetrical sensory and motor neuropathy with focal slowing at specific topographies on NCS. PMID:26982985

  16. Overlap phenotype between CMT1A and hereditary neuropathy with liability to pressure palsies caused by the novel small in-frame deletion c.407_418del12 in PMP22 gene.

    PubMed

    Vill, Katharina; Kuhn, Marius; Gläser, Dieter; Müller-Felber, Wolfgang

    2015-02-01

    We report monozygotic twins, who presented with a clinical picture of Charcot-Marie-Tooth disease type 1 (CMT1) with bilateral foot drop, pes cavus, thoracic kyphosis, and scoliosis. Hereditary neuropathy with liability to pressure palsies (HNPP) showed up in one of them. Neurography showed demyelinating neuropathy, typical for CMT1, and transient conduction block in the ulnar nerve correlating with clinical ulnar palsy due to minor mechanical stress in only one of them. Genetic analysis revealed novel small de novo deletion c.407_418del12 in the PMP22 gene. Our patient shows the rarely reported combination of CMT1A and HNPP, caused by an in-frame deletion in the PMP22 gene. HNPP is in the majority of cases correlated with heterozygous deletion of the whole PMP22 gene or other mutations leading to functional haploinsufficiency. The cases give further evidence that pathogenesis of HNPP is not completely understood and can obviously result from existence of a defective protein, too. The intrafamiliar phenotypic variability, even in monozygotic twins, confirms the well-known fact that factors apart from genetics contribute to the clinical course. PMID:25265422

  17. Rapidly progressive amyotrophic lateral sclerosis in a young patient with hereditary neuropathy with liability to pressure palsies.

    PubMed

    Canali, Elena; Chiari, Annalisa; Sola, Patrizia; Fioravanti, Valentina; Valzania, Franco; Pentore, Roberta; Nichelli, Paolo; Mandrioli, Jessica

    2010-05-01

    We describe the rare case of a young woman with hereditary neuropathy with liability to compression palsy (HNPP), who developed a rapidly progressive ALS. We suggest that underexpression of PMP22 protein in the nervous system might interfere with motor neuron function by impairing myelin formation and exposure of the axon to injury. Patients with ALS and evidence of demyelination should be screened for HNPP. PMID:19437170

  18. PMP22 related neuropathies: Charcot-Marie-Tooth disease type 1A and Hereditary Neuropathy with liability to Pressure Palsies.

    PubMed

    van Paassen, Barbara W; van der Kooi, Anneke J; van Spaendonck-Zwarts, Karin Y; Verhamme, Camiel; Baas, Frank; de Visser, Marianne

    2014-01-01

    PMP22 related neuropathies comprise (1) PMP22 duplications leading to Charcot-Marie-Tooth disease type 1A (CMT1A), (2) PMP22 deletions, leading to Hereditary Neuropathy with liability to Pressure Palsies (HNPP), and (3) PMP22 point mutations, causing both phenotypes. Overall prevalence of CMT is usually reported as 1:2,500, epidemiological studies show that 20-64% of CMT patients carry the PMP22 duplication. The prevalence of HNPP is not well known. CMT1A usually presents in the first two decades with difficulty walking or running. Distal symmetrical muscle weakness and wasting and sensory loss is present, legs more frequently and more severely affected than arms. HNPP typically leads to episodic, painless, recurrent, focal motor and sensory peripheral neuropathy, preceded by minor compression on the affected nerve. Electrophysiological evaluation is needed to determine whether the polyneuropathy is demyelinating. Sonography of the nerves can be useful. Diagnosis is confirmed by finding respectively a PMP22 duplication, deletion or point mutation. Differential diagnosis includes other inherited neuropathies, and acquired polyneuropathies. The mode of inheritance is autosomal dominant and de novo mutations occur. Offspring of patients have a chance of 50% to inherit the mutation from their affected parent. Prenatal testing is possible; requests for prenatal testing are not common. Treatment is currently symptomatic and may include management by a rehabilitation physician, physiotherapist, occupational therapist and orthopaedic surgeon. Adult CMT1A patients show slow clinical progression of disease, which seems to reflect a process of normal ageing. Life expectancy is normal. PMID:24646194

  19. PMP22 related neuropathies: Charcot-Marie-Tooth disease type 1A and Hereditary Neuropathy with liability to Pressure Palsies

    PubMed Central

    2014-01-01

    PMP22 related neuropathies comprise (1) PMP22 duplications leading to Charcot-Marie-Tooth disease type 1A (CMT1A), (2) PMP22 deletions, leading to Hereditary Neuropathy with liability to Pressure Palsies (HNPP), and (3) PMP22 point mutations, causing both phenotypes. Overall prevalence of CMT is usually reported as 1:2,500, epidemiological studies show that 20-64% of CMT patients carry the PMP22 duplication. The prevalence of HNPP is not well known. CMT1A usually presents in the first two decades with difficulty walking or running. Distal symmetrical muscle weakness and wasting and sensory loss is present, legs more frequently and more severely affected than arms. HNPP typically leads to episodic, painless, recurrent, focal motor and sensory peripheral neuropathy, preceded by minor compression on the affected nerve. Electrophysiological evaluation is needed to determine whether the polyneuropathy is demyelinating. Sonography of the nerves can be useful. Diagnosis is confirmed by finding respectively a PMP22 duplication, deletion or point mutation. Differential diagnosis includes other inherited neuropathies, and acquired polyneuropathies. The mode of inheritance is autosomal dominant and de novo mutations occur. Offspring of patients have a chance of 50% to inherit the mutation from their affected parent. Prenatal testing is possible; requests for prenatal testing are not common. Treatment is currently symptomatic and may include management by a rehabilitation physician, physiotherapist, occupational therapist and orthopaedic surgeon. Adult CMT1A patients show slow clinical progression of disease, which seems to reflect a process of normal ageing. Life expectancy is normal. PMID:24646194

  20. Mutation analysis of PMP22 in Slovak patients with Charcot-Marie-Tooth disease and hereditary neuropathy with liability to pressure palsies.

    PubMed

    Resko, Peter; Radvansky, Jan; Odnogova, Zuzana; Baldovic, Marian; Minarik, Gabriel; Polakova, Helena; Palffy, Roland; Kadasi, Ludevit

    2011-12-01

    Charcot-Marie-Tooth disease (CMT) and related peripheral neuropathies are the most commonly inherited neurological disorders in humans, characterized by clinical and genetic heterogeneity. The most prevalent clinical entities belonging to this group of disorders are CMT type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP). CMT1A and HNPP are predominantly caused by a 1.5 Mb duplication and deletion in the chromosomal region 17p11.2, respectively, and less frequently by other mutations in the peripheral myelin protein 22 (PMP22) gene. Despite being relatively common diseases, they haven't been previously studied in the Slovak population. Therefore, the aim of this study was to identify the spectrum and frequency of PMP22 mutations in the Slovak population by screening 119 families with CMT and 2 families with HNPP for causative mutations in this gene. The copy number determination of PMP22 resulted in the detection of CMT1A duplication in 40 families and the detection of HNPP deletion in 7 families, 6 of which were originally diagnosed as CMT. Consequent mutation screening of families without duplication or deletion using dHPLC and sequencing identified 6 single base changes (3 unpublished to date), from which only c.327C>A (Cys109X) present in one family was provably causative. These results confirm the leading role of PMP22 mutation analysis in the differential diagnosis of CMT and show that the spectrum and frequency of PMP22 mutations in the Slovak population is comparable to that seen in the global population. PMID:22131320

  1. Hereditary Neuropathy with Liability to Pressure Palsy Presenting as an Acute Brachial Plexopathy: A Lover's Palsy.

    PubMed

    Wedderburn, Sarah; Pateria, Puraskar; Panegyres, Peter K

    2014-01-01

    It is generally regarded that patients with hereditary neuropathy to pressure palsies, due to a deletion in the PMP22 gene, show recurrent pressure palsy and generalised peripheral neuropathy (pes cavus and hammer toes sometimes develop). Brachial plexopathy is rarely identified as a first presentation of hereditary neuropathy to pressure palsies. We describe a young man who developed a painless flail upper limb with a clinical diagnosis of a brachial plexopathy after his partner slept on his arm - a PMP22 deletion was found. His father, who had a symmetrical polyneuropathy without recurrent mononeuropathies, shared the PMP22 deletion. PMID:25685136

  2. Hereditary Neuropathy with Liability to Pressure Palsy Presenting as an Acute Brachial Plexopathy: A Lover's Palsy

    PubMed Central

    Wedderburn, Sarah; Pateria, Puraskar; Panegyres, Peter K.

    2014-01-01

    It is generally regarded that patients with hereditary neuropathy to pressure palsies, due to a deletion in the PMP22 gene, show recurrent pressure palsy and generalised peripheral neuropathy (pes cavus and hammer toes sometimes develop). Brachial plexopathy is rarely identified as a first presentation of hereditary neuropathy to pressure palsies. We describe a young man who developed a painless flail upper limb with a clinical diagnosis of a brachial plexopathy after his partner slept on his arm – a PMP22 deletion was found. His father, who had a symmetrical polyneuropathy without recurrent mononeuropathies, shared the PMP22 deletion. PMID:25685136

  3. Application of multiplex ligation-dependent probe analysis to define a small deletion encompassing PMP22 exons 4 and 5 in hereditary neuropathy with liability to pressure palsies.

    PubMed

    Sutton, Ian J; Mocroft, A Paul; Lindley, Victoria H; Barber, Richard M; Bryon, R Jane; Winer, John B; MacDonald, Fiona

    2004-12-01

    Hereditary neuropathy with liability to pressure palsies arises as a result of defects at the chromosome 17p11.2-12 locus and in 84% of cases a 1.5 Mb deletion containing the PMP22 gene is detected by analysis that utilises polymorphic (CA)n repeat markers which flank this gene. We report the clinical and electrophysiological findings observed in a kindred with three members affected by HNPP due to a deletion containing exons 4 and 5 of the PMP22 gene. This small deletion cannot be detected using standard analysis with polymorphic (CA)n repeat markers and a definitive diagnosis was made by multiplex ligation-dependent probe analysis of PMP22 exons 1A-5. MLPA can be readily utilised as a routine diagnostic laboratory test to detect the common HNPP 1.5 Mb deletion, as well as the reciprocal 1.5 Mb insertion observed in CMT1A, but has the advantage over other diagnostic techniques of being able to define single exon deletions. PMID:15564036

  4. Diagnosis of CMT1A duplications and HNPP deletions by interphase FISH: Implications for testing in the cytogenetics laboratory

    SciTech Connect

    Shaffer, L.G.; Kennedy, G.M.; Spikes, A.S.

    1997-03-31

    Charcot-Marie-Tooth (CMT) disease type 1A is an inherited peripheral neuropathy characterized by slowly progressive distal muscle wasting and weakness, decreased nerve conduction velocities, and genetic linkage to 17p12. Most (>98%) CMT1A cases are caused by a DNA duplication of a 1.5-Mb region in 17p12 containing the PMP22 gene. The reciprocal product of the CMT1A duplication is a 1.5-Mb deletion which causes hereditary neuropathy with liability to pressure palsies (HNPP). The most informative current diagnostic testing requires pulsed-field gel electrophoresis to detect DNA rearrangement-specific junction fragments. We investigated the use of interphase FISH for the detection of duplications and deletions for these disorders in the clinical molecular cytogenetics laboratory. Established cell lines or blood specimens from 23 individuals with known molecular diagnoses and 10 controls were obtained and scored using a two-color FISH assay. At least 70%, of CMT1A cells displayed three signals consistent with duplications. Using this minimum expected percentile to make a CMT1A duplication diagnosis, all patients with CMT1A showed a range of 71-92% of cells displaying at least three signals. Of the HNPP cases, 88% of cells displayed only one hybridization signal, consistent with deletions. The PMP22 locus from normal control individuals displayed a duplication pattern in {approximately}9% of cells, interpreted as replication of this locus. The percentage of cells showing replication was significantly lower than in those cells displaying true duplications. We conclude that FISH can be reliably used to diagnose CMT1A and HNPP in the clinical cytogenetics laboratory and to readily distinguish the DNA rearrangements associated with these disorders from individuals without duplication or deletion of the PMP22 locus. 43 refs., 4 figs., 2 tabs.

  5. Recombination hot spot in 3.2-kb region of the Charcot-Marie Tooth type 1A repeat sequences: New tools for molecular diagnosis of hereditary neuropathy with liability to pressure palsies and of Charcot-Marie-Tooth type 1A

    SciTech Connect

    Lopes, J.; LeGuern, E.; Gouider, R.; Tardieu, S.; Abbas, N.

    1996-06-01

    Charcot-Marie-Tooth type 1A (CMT1A) disease and hereditary neuropathy with liability to pressure palsies (HNPP) are autosomal dominant neuropathies, associated, respectively, with duplications and deletions of the same 1.5-Mb region on 17p11.2-p12. These two rearrangements are the reciprocal products of an unequal meiotic crossover between the two chromosome 17 homologues, caused by the misalignment of the CMT1A repeat sequences (CMT1A-REPs), the homologous sequences flanking the 1.5-Mb CMT1A/HNPP monomer unit. In order to map recombination breakpoints within the CMT1A-REPs, a 12.9-kb restriction map was constructed from cloned EcoRI fragments of the proximal and distal CMT1A-REPs. Only 3 of the 17 tested restriction sites were present in the proximal CMT1A-REP but absent in the distal CMT1A-REP, indicating a high degree of homology between these sequences. The rearrangements were mapped in four regions of the CMT1A-REPs by analysis of 76 CMT1A index cases and 38 HNPP patients, who were unrelated. A hot spot of crossover breakpoints located in a 3.2-kb region accounted for three-quarters of the rearrangements, detected after EcoRI/SacI digestion, by the presence of 3.2-kb and 7.8-kb junction fragments in CMT1A and HNPP patients, respectively. These junction fragments, which can be detected on classical Southern blots, permit molecular diagnosis. Other rearrangements can also be detected by gene dosage on the same Southern blots. 25 refs., 4 figs., 2 tabs.

  6. Isolation of novel genes from the CMT1A duplication/HNPP deletion critical region in 17p11.2-p12.

    PubMed

    Murakami, T; Sun, Z S; Lee, C C; Lupski, J R

    1997-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with a 1.5-Mb tandem DNA duplication in chromosome 17p11.2-p12, while hereditary neuropathy with liability to pressure palsies (HNPP) is associated with a 1.5-Mb deletion at this locus. The 1.5-Mb CMT1A monomer unit duplicated in CMT1A and deleted in HNPP is flanked by low-copy repeats termed CMT1A-REPs. Both diseases appear to be caused by an altered copy number of the peripheral myelin protein 22 gene (PMP22), which lies within the critical region. To identify additional genes rapidly, we used a cosmid contig of this region and reciprocal probing of arrayed chromosome 17-specific cosmid and cDNA libraries. Three cDNA clones were identified within the CMT1A duplication/HNPP deletion region and one just proximal to the critical region. The cDNA for human heme A:farnesyltransferase (COX10) mapped 10 kb centromeric to the distal CMT1A-REP. The other two cDNA clones from within the critical interval mapped to cosmid 126D1 at the mfd41 (D17S261) DNA marker, and their conceptual translation showed homology to 60S ribosomal protein L9 (RPL9) and chromosomal protein RMSA-1 (RMSA-1). A gene that is homologous to human peroxisome proliferator activated receptor alpha (hPPARA) was identified near the proximal CMT1A-REP. PMID:9027492

  7. Foot pressure distribution in children with cerebral palsy while standing.

    PubMed

    Galli, Manuela; Cimolin, Veronica; Pau, Massimiliano; Leban, Bruno; Brunner, Reinald; Albertini, Giorgio

    2015-01-01

    Foot deformity is a major component of impaired functioning in cerebral palsy (CP). While gait and balance issues related to CP have been studied extensively, there is little information to date on foot-ground interaction (i.e. contact area and plantar pressure distribution). This study aimed to characterize quantitatively the foot-ground contact parameters during static upright standing in hemiplegia and diplegia. We studied 64 children with hemiplegia (mean age 8.2 years; SD 2.8 years) and 43 with diplegia (mean age 8.8 years; SD 2.3 years) while standing on both legs statically on a pressure sensitive mat. We calculated pressure data for the whole foot and sub-regions (i.e. rearfoot, midfoot and forefoot) and average contact pressure. The Arch Index (AI) served for classifying the feet as flat, normal or cavus feet. The data were compared with those from a sample of age- and gender-matched participants (control group, 68 children). Most of the feet showed very high AI values, thus indicating a flat foot. This deformity was more common in diplegia (74.4%) than in hemiplegia (54.7%). In both diplegic and hemiplegic children, average plantar pressure was significantly increased in the forefoot and midfoot and decreased in the rearfoot (p<0.001). The present data indicate an increased load on the front parts of the foot, which may be due to plantarflexor overactivity or knee flexion, combined with an increased incidence of low foot arches. As a low foot arch does not necessarily increase forefoot load, this deformity can be regarded as secondary. PMID:26057837

  8. A 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region in 17p11.2-p12

    SciTech Connect

    Murakami, Tatsufumi; Lupski, J.R.

    1996-05-15

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with a 1.5-Mb tandem duplication in chromosome 17p11.2-p12, and hereditary neuropathy with liability to pressure palsies (HNPP) is associated with a 1.5-Mb deletion at this locus. Both diseases appear to result from an altered copy number of the peripheral myelin protein-22 gene, PMP22, which maps within the critical region. To identify additional genes and characterize chromosomal elements, a 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region was assembled using a yeast artificial chromosome (YAC)-based isolation and binning strategy. Whole YAC probes were used for screening a high-density arrayed chromosome 17-specific cosmid library. Selected cosmids were spotted on dot blots and assigned to bins defined by YACs. This binning of cosmids facilitated the subsequent fingerprint analysis. The 1.5-Mb region was covered by 137 cosmids with a minimum overlap set of 52 cosmids assigned to 17 bins and 9 contigs. 20 refs., 2 figs.

  9. Mechanisms for nonrecurrent genomic rearrangements associated with CMT1A or HNPP: rare CNVs as a cause for missing heritability.

    PubMed

    Zhang, Feng; Seeman, Pavel; Liu, Pengfei; Weterman, Marian A J; Gonzaga-Jauregui, Claudia; Towne, Charles F; Batish, Sat Dev; De Vriendt, Els; De Jonghe, Peter; Rautenstrauss, Bernd; Krause, Klaus-Henning; Khajavi, Mehrdad; Posadka, Jan; Vandenberghe, Antoon; Palau, Francesc; Van Maldergem, Lionel; Baas, Frank; Timmerman, Vincent; Lupski, James R

    2010-06-11

    Genomic rearrangements involving the peripheral myelin protein gene (PMP22) in human chromosome 17p12 are associated with neuropathy: duplications cause Charcot-Marie-Tooth disease type 1A (CMT1A), whereas deletions lead to hereditary neuropathy with liability to pressure palsies (HNPP). Our previous studies showed that >99% of these rearrangements are recurrent and mediated by nonallelic homologous recombination (NAHR). Rare copy number variations (CNVs) generated by nonrecurrent rearrangements also exist in 17p12, but their underlying mechanisms are not well understood. We investigated 21 subjects with rare CNVs associated with CMT1A or HNPP by oligonucleotide-based comparative genomic hybridization microarrays and breakpoint sequence analyses, and we identified 17 unique CNVs, including two genomic deletions, ten genomic duplications, two complex rearrangements, and three small exonic deletions. Each of these CNVs includes either the entire PMP22 gene, or exon(s) only, or ultraconserved potential regulatory sequences upstream of PMP22, further supporting the contention that PMP22 is the critical gene mediating the neuropathy phenotypes associated with 17p12 rearrangements. Breakpoint sequence analysis reveals that, different from the predominant NAHR mechanism in recurrent rearrangement, various molecular mechanisms, including nonhomologous end joining, Alu-Alu-mediated recombination, and replication-based mechanisms (e.g., FoSTeS and/or MMBIR), can generate nonrecurrent 17p12 rearrangements associated with neuropathy. We document a multitude of ways in which gene function can be altered by CNVs. Given the characteristics, including small size, structural complexity, and location outside of coding regions, of selected rare CNVs, their identification remains a challenge for genome analysis. Rare CNVs may potentially represent an important portion of "missing heritability" for human diseases. PMID:20493460

  10. Abnormal sitting pressures of hemiplegic cerebral palsy children on a school chair

    PubMed Central

    Lee, In-Hee; Park, Sang-young

    2015-01-01

    [Purpose] The purpose of this study was to investigate the differences in symmetry of sitting posture between typical developmental (TD) children and hemi-cerebral palsy (CP) children. [Subjects and Methods] A school chair mounted on a force platform was used to assess the quiet-sitting pressure distribution of 10 TD and 10 CP children. [Results] The symmetry index of the TD children was significantly closer to zero than that of the CP children irrespective of the latter group’s hemiparetic side. [Conclusions] Sitting posture on school chairs of CP children was more asymmetrical than that of TD children. PMID:25729201

  11. Hereditary Neuropathy with Liability to Pressure Palsy: A Recurrent and Bilateral Foot Drop Case Report

    PubMed Central

    Flor-de-Lima, Filipa; Taipa, Ricardo; Melo-Pires, Manuel; Rodrigues, Maria Lurdes

    2013-01-01

    Hereditary neuropathy with liability to pressure palsy is characterized by acute, painless, recurrent mononeuropathies secondary to minor trauma or compression. A 16-year-old boy had the first episode of right foot drop after minor motorcycle accident. Electromyography revealed conduction block and slowing velocity conduction of the right deep peroneal nerve at the fibular head. After motor rehabilitation, he fully recovered. Six months later he had the second episode of foot drop in the opposite site after prolonged squatting position. Electromyography revealed sensorimotor polyneuropathy of left peroneal, sural, posterior tibial, and deep peroneal nerves and also of ulnar, radial, and median nerves of both upper limbs. Histological examination revealed sensory nerve demyelination and focal thickenings of myelin fibers. The diagnosis of hereditary neuropathy with liability to pressure palsy was confirmed by PMP22 deletion of chromosome 17p11.2. He started motor rehabilitation and avoidance of stressing factors with progressive recovery. After one-year followup, he was completely asymptomatic. Recurrent bilateral foot drop history, “sausage-like” swellings of myelin in histological examination, and the results of electromyography led the authors to consider the diagnosis despite negative family history. The authors highlight this rare disease in pediatric population and the importance of high index of clinical suspicion for its diagnosis. PMID:24251057

  12. Hereditary neuropathy with liability to pressure palsy: a recurrent and bilateral foot drop case report.

    PubMed

    Flor-de-Lima, Filipa; Macedo, Liliana; Taipa, Ricardo; Melo-Pires, Manuel; Rodrigues, Maria Lurdes

    2013-01-01

    Hereditary neuropathy with liability to pressure palsy is characterized by acute, painless, recurrent mononeuropathies secondary to minor trauma or compression. A 16-year-old boy had the first episode of right foot drop after minor motorcycle accident. Electromyography revealed conduction block and slowing velocity conduction of the right deep peroneal nerve at the fibular head. After motor rehabilitation, he fully recovered. Six months later he had the second episode of foot drop in the opposite site after prolonged squatting position. Electromyography revealed sensorimotor polyneuropathy of left peroneal, sural, posterior tibial, and deep peroneal nerves and also of ulnar, radial, and median nerves of both upper limbs. Histological examination revealed sensory nerve demyelination and focal thickenings of myelin fibers. The diagnosis of hereditary neuropathy with liability to pressure palsy was confirmed by PMP22 deletion of chromosome 17p11.2. He started motor rehabilitation and avoidance of stressing factors with progressive recovery. After one-year followup, he was completely asymptomatic. Recurrent bilateral foot drop history, "sausage-like" swellings of myelin in histological examination, and the results of electromyography led the authors to consider the diagnosis despite negative family history. The authors highlight this rare disease in pediatric population and the importance of high index of clinical suspicion for its diagnosis. PMID:24251057

  13. The 1.4-Mb CMT1A Duplication/HNPP Deletion Genomic Region Reveals Unique Genome Architectural Features and Provides Insights into the Recent Evolution of New Genes

    PubMed Central

    Inoue, Ken; Dewar, Ken; Katsanis, Nicholas; Reiter, Lawrence T.; Lander, Eric S.; Devon, Keri L.; Wyman, Dudley W.; Lupski, James R.; Birren, Bruce

    2001-01-01

    Duplication and deletion of the 1.4-Mb region in 17p12 that is delimited by two 24-kb low copy number repeats (CMT1A–REPs) represent frequent genomic rearrangements resulting in two common inherited peripheral neuropathies, Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsy (HNPP). CMT1A and HNPP exemplify a paradigm for genomic disorders wherein unique genome architectural features result in susceptibility to DNA rearrangements that cause disease. A gene within the 1.4-Mb region, PMP22, is responsible for these disorders through a gene-dosage effect in the heterozygous duplication or deletion. However, the genomic structure of the 1.4-Mb region, including other genes contained within the rearranged genomic segment, remains essentially uncharacterized. To delineate genomic structural features, investigate higher-order genomic architecture, and identify genes in this region, we constructed PAC and BAC contigs and determined the complete nucleotide sequence. This CMT1A/HNPP genomic segment contains 1,421,129 bp of DNA. A low copy number repeat (LCR) was identified, with one copy inside and two copies outside of the 1.4-Mb region. Comparison between physical and genetic maps revealed a striking difference in recombination rates between the sexes with a lower recombination frequency in males (0.67 cM/Mb) versus females (5.5 cM/Mb). Hypothetically, this low recombination frequency in males may enable a chromosomal misalignment at proximal and distal CMT1A–REPs and promote unequal crossing over, which occurs 10 times more frequently in male meiosis. In addition to three previously described genes, five new genes (TEKT3, HS3ST3B1, NPD008/CGI-148, CDRT1, and CDRT15) and 13 predicted genes were identified. Most of these predicted genes are expressed only in embryonic stages. Analyses of the genomic region adjacent to proximal CMT1A–REP indicated an evolutionary mechanism for the formation of proximal CMT1A–REP and the creation of novel genes by DNA rearrangement during primate speciation. PMID:11381029

  14. Bell's Palsy

    MedlinePlus

    ... Skiing, Snowboarding, Skating Crushes What's a Booger? Bell's Palsy KidsHealth > For Kids > Bell's Palsy Print A A ... goes away on its own. What Is Bell's Palsy? Bell's palsy weakens or paralyzes the muscles on ...

  15. [Acute negative pressure pulmonary edema: illustration from a unusual clinical case following a postoperative recurrent laryngeal nerve palsy].

    PubMed

    Aissaoui, Y; Kichna, H; Boughalem, M; Drissi Kamili, N

    2012-05-01

    Acute postoperative negative pressure pulmonary edema (NPPE) is a respiratory complication due to upper airway obstruction occurring most commonly in the postoperative period. Pathophysiologically, NPPE is explained by the abrupt generation of highly negative intrathoracic pressure that lead to fluid transudation from the pulmonary capillaries. This observation reports an unusual case of a young patient who has undergone total thyroidectomy for multinodular goiter. The postoperative period was marked by the occurrence of bilateral recurrent laryngeal nerve palsy that was complicated by a NPPE. The outcome was favorable after mechanical ventilation with reversal of NPPE and recovery of the recurrent laryngeal nerve function. This article stresses the importance of prevention of recurrent nerve palsy during thyroid surgery. It also highlights a little known respiratory complication: the NPPE. Understanding the pathophysiology of NPPE, rapid diagnosis and appropriate therapeutic measures could prevent its potential lethal consequences. PMID:22465642

  16. Changes of Plantar Pressure and Gait Parameters in Children with Mild Cerebral Palsy Who Used a Customized External Strap Orthosis: A Crossover Study

    PubMed Central

    Chang, Wen-Dien; Chang, Nai-Jen; Lin, Hung-Yu; Lai, Ping-Tung

    2015-01-01

    Toe-in gait and crouch gait can make children with mild cerebral palsy fall and suffer improper balance during walking or ambulation training. A customized external strap orthosis for correcting leg alignment was used to resolve this problem. The purpose of this study was to research the immediate effects while wearing the customized external strap orthosis. Pressure platform was used to assess the plantar pressure through static and dynamic assessments and to record the changes in path of pressure trajectory. Motion image analysis system was used to record the gait parameters, which included gait speed, stride length, and cadence. The influence of both wearing and removing the orthosis on the dominant leg of children with mild cerebral palsy was analyzed. Nine children with mild cerebral palsy, who all had a dominant right leg, were recruited. After wearing the orthosis, all gait parameters improved, and foot motion changed in the stance phase of the gait cycle. The path of pressure trajectory closing to the midline was also observed during dynamic assessment. Changes in plantar pressure and path of pressure trajectory were observed and the orthosis device could provide immediate assistance to correct the leg alignment and improve the gait performance in children with mild cerebral palsy. PMID:26640796

  17. Rapid genetic screening of Charcot-Marie-Tooth disease type 1A and hereditary neuropathy with liability to pressure palsies patients.

    PubMed

    Li, Xiaobo; Zi, Xiaohong; Li, Lin; Zhan, Yajing; Huang, Shunxiang; Li, Jin; Li, Xuning; Li, Xigui; Hu, Zhengmao; Xia, Kun; Tang, Beisha; Zhang, Ruxu

    2012-11-15

    We used the allele-specific PCR-double digestion method on peripheral myelin protein 22 (PMP22) to determine duplication and deletion mutations in the proband and family members of one family with Charcot-Marie-Tooth disease type 1 and one family with hereditary neuropathy with liability to pressure palsies. The proband and one subclinical family member from the Charcot-Marie-Tooth disease type 1 family had a PMP22 gene duplication; one patient from the hereditary neuropathy with liability to pressure palsies family had a PMP22 gene deletion. Electron microscopic analysis of ultrathin sections of the superficial peroneal nerve from the two probands demonstrated demyelination and myelin sheath hyperplasia, as well as an 'onion-like' structure in the Charcot-Marie-Tooth disease type 1A patient. We observed an irregular thickened myelin sheath and 'mouse-nibbled'-like changes in the patient with hereditary neuropathy with liability to pressure palsies. In the Charcot-Marie-Tooth disease type 1A patient, nerve electrophysiological examination revealed moderate-to-severe reductions in the motor and sensory conduction velocities of the bilateral median nerve, ulnar nerve, tibial nerve, and sural nerve. Moreover, the compound muscle action potential amplitude was decreased. In the patient with hereditary neuropathy with liability to pressure palsies, the nerve conduction velocity of the bilateral tibial nerve and sural nerve was moderately reduced, and the nerve conduction velocity of the median nerve and ulnar nerve of both upper extremities was slightly reduced. PMID:25337104

  18. Rapid genetic screening of Charcot-Marie-Tooth disease type 1A and hereditary neuropathy with liability to pressure palsies patients★

    PubMed Central

    Li, Xiaobo; Zi, Xiaohong; Li, Lin; Zhan, Yajing; Huang, Shunxiang; Li, Jin; Li, Xuning; Li, Xigui; Hu, Zhengmao; Xia, Kun; Tang, Beisha; Zhang, Ruxu

    2012-01-01

    We used the allele-specific PCR-double digestion method on peripheral myelin protein 22 (PMP22) to determine duplication and deletion mutations in the proband and family members of one family with Charcot-Marie-Tooth disease type 1 and one family with hereditary neuropathy with liability to pressure palsies. The proband and one subclinical family member from the Charcot-Marie-Tooth disease type 1 family had a PMP22 gene duplication; one patient from the hereditary neuropathy with liability to pressure palsies family had a PMP22 gene deletion. Electron microscopic analysis of ultrathin sections of the superficial peroneal nerve from the two probands demonstrated demyelination and myelin sheath hyperplasia, as well as an ‘onion-like’ structure in the Charcot-Marie-Tooth disease type 1A patient. We observed an irregular thickened myelin sheath and ‘mouse-nibbled’-like changes in the patient with hereditary neuropathy with liability to pressure palsies. In the Charcot-Marie-Tooth disease type 1A patient, nerve electrophysiological examination revealed moderate-to-severe reductions in the motor and sensory conduction velocities of the bilateral median nerve, ulnar nerve, tibial nerve, and sural nerve. Moreover, the compound muscle action potential amplitude was decreased. In the patient with hereditary neuropathy with liability to pressure palsies, the nerve conduction velocity of the bilateral tibial nerve and sural nerve was moderately reduced, and the nerve conduction velocity of the median nerve and ulnar nerve of both upper extremities was slightly reduced. PMID:25337104

  19. Bell's Palsy

    MedlinePlus

    ... Palsy? Bell's palsy is a temporary weakness or paralysis of the muscles on one side of the ... sent correctly. That's what causes weakness or temporary paralysis on one side of the face. When the ...

  20. Cerebral palsy

    MedlinePlus

    Cerebral palsy is a group of disorders that can involve brain and nervous system functions, such as movement, ... and thinking. There are several different types of cerebral palsy, including spastic, dyskinetic, ataxic, hypotonic, and mixed.

  1. Cerebral Palsy

    MedlinePlus

    ... Sledding, Skiing, Snowboarding, Skating Crushes What's a Booger? Cerebral Palsy KidsHealth > For Kids > Cerebral Palsy Print A A ... the things that kids do every day. What's CP? Some kids with CP use wheelchairs and others ...

  2. Cerebral Palsy

    MedlinePlus

    Cerebral palsy is a group of disorders that affect a person's ability to move and to maintain balance ... do not get worse over time. People with cerebral palsy may have difficulty walking. They may also have ...

  3. Cerebral Palsy

    MedlinePlus

    ... Awards Enhancing Diversity Find People About NINDS NINDS Cerebral Palsy Information Page Clinical Trials Trial of Erythropoietin Neuroprotection ... en Español Additional resources from MedlinePlus What is Cerebral Palsy? The term cerebral palsy refers to a group ...

  4. Transgenic mouse models of CMT1A and HNPP.

    PubMed

    Suter, U; Nave, K A

    1999-09-14

    We have generated several PMP22 animal mutants with altered PMP22 gene dosage. A moderate increase in the number of PMP22 genes led to hypomyelination comparable to CMT1A, whereas high copy numbers of transgenic PMP22 resulted in phenotypes resembling more severe forms of hereditary motor and sensory neuropathies. In contrast, eliminating one of the two normal PMP22 genes by gene targeting caused unstable focal hypermyelination (tomacula) similar to the pathology in HNPP. A related but more severe phenotype was observed in mice that lack PMP22 completely. Detailed analysis of the different PMP22 mutants revealed, in addition to the obvious myelinopathy, distal axonopathy as a characteristic feature. We conclude that the maintenance of axons might be a promising target for therapeutic interventions in these demyelinating hereditary neuropathies. Furthermore, our results strongly support the concept that PMP22-related neuropathies (and most likely also other forms of inherited motor and sensory neuropathies) should be viewed as the consequence of impaired neuron-Schwann cell interactions that are likely already to be operative during development. Such considerations should be taken into account in the design of potential novel treatment strategies. PMID:10586249

  5. A Case of Apoplexy Attack-Like Neuropathy due to Hereditary Neuropathy with Liability to Pressure Palsies in a Patient Diagnosed with Chronic Cerebral Infarction.

    PubMed

    Hachisuka, Akiko; Matsushima, Yasuyuki; Hachisuka, Kenji; Saeki, Satoru

    2016-06-01

    Hereditary neuropathy with liability to pressure palsies is an inherited disease associated with the loss of a copy of the PMP22 gene. The condition leads to mononeuropathy due to compression and easy strangulation during daily life activities, resulting in sudden muscle weakness and sensory disturbance, and displaying symptoms similar to cerebrovascular diseases. We report the case of an 80-year-old man with left paralysis due to chronic cerebral infarction. His medical history indicated remarkable recovery from about 4 months after the onset of left hemiplegia with predominant involvement of the fingers. Despite subsequent recurrent monoplegia of the upper or lower limbs, brain magnetic resonance imaging consistently revealed only previous cerebral infarction in the right corona radiata without new lesions. Medical examination showed reduced deep tendon reflexes in his extremities on both the healthy and hemiplegic sides. Nerve conduction studies showed delayed conduction at the bilateral carpal and cubital tunnels and near the right caput fibulae. Genetic analysis revealed loss of a copy of the PMP22 gene. Thus, he was diagnosed with a cerebral infarction complicated by hereditary neuropathy with liability to pressure palsies. Stroke patients develop sudden muscle weakness and sensory disturbance. However, if such patients have no hyperactive deep tendon reflexes and show atypical recovery of paralysis that does not correspond to findings of imaging modalities, nerve conduction studies and genetic analysis may be necessary, considering the complication of hereditary neuropathy with liability to pressure palsies. PMID:27080157

  6. Cerebral palsy.

    PubMed

    Wimalasundera, Neil; Stevenson, Valerie L

    2016-06-01

    Cerebral palsy has always been known as a disorder of movement and posture resulting from a non-progressive injury to the developing brain; however, more recent definitions allow clinicians to appreciate more than just the movement disorder. Accurate classification of cerebral palsy into distribution, motor type and functional level has advanced research. It also facilitates appropriate targeting of interventions to functional level and more accurate prognosis prediction. The prevalence of cerebral palsy remains fairly static at 2-3 per 1000 live births but there have been some changes in trends for specific causal groups. Interventions for cerebral palsy have historically been medical and physically focused, often with limited evidence to support their efficacy. The use of more appropriate outcome measures encompassing quality of life and participation is helping to deliver treatments which are more meaningful for people with cerebral palsy and their carers. PMID:26837375

  7. Associations of Sedentary Behaviour, Physical Activity, Blood Pressure and Anthropometric Measures with Cardiorespiratory Fitness in Children with Cerebral Palsy

    PubMed Central

    Ryan, Jennifer M.; Hensey, Owen; McLoughlin, Brenda; Lyons, Alan; Gormley, John

    2015-01-01

    Background Children with cerebral palsy (CP) have poor cardiorespiratory fitness in comparison to their peers with typical development, which may be due to low levels of physical activity. Poor cardiorespiratory fitness may contribute to increased cardiometabolic risk. Purpose The aim of this study was to determine the association between sedentary behaviour, physical activity and cardiorespiratory fitness in children with CP. An objective was to determine the association between cardiorespiratory fitness, anthropometric measures and blood pressure in children with CP. Methods This study included 55 ambulatory children with CP [mean (SD) age 11.3 (0.2) yr, range 6-17 yr; Gross Motor Function Classification System (GMFCS) levels I and II]. Anthropometric measures (BMI, waist circumference and waist-height ratio) and blood pressure were taken. Cardiorespiratory fitness was measured using a 10 m shuttle run test. Children were classified as low, middle and high fitness according to level achieved on the test using reference curves. Physical activity was measured by accelerometry over 7 days. In addition to total activity, time in sedentary behaviour and light, moderate, vigorous, and sustained moderate-to-vigorous activity (≥10 min bouts) were calculated. Results Multiple regression analyses revealed that vigorous activity (β = 0.339, p<0.01), sustained moderate-to-vigorous activity (β = 0.250, p<0.05) and total activity (β = 0.238, p<0.05) were associated with level achieved on the shuttle run test after adjustment for age, sex and GMFCS level. Children with high fitness spent more time in vigorous activity than children with middle fitness (p<0.05). Shuttle run test level was negatively associated with BMI (r2 = -0.451, p<0.01), waist circumference (r2 = -0.560, p<0.001), waist-height ratio (r2 = -0.560, p<0.001) and systolic blood pressure (r2 = -0.306, p<0.05) after adjustment for age, sex and GMFCS level. Conclusions Participation in physical activity, particularly at a vigorous intensity, is associated with high cardiorespiratory fitness in children with CP. Low cardiorespiratory fitness is associated with increased cardiometabolic risk. PMID:25835955

  8. Cerebral Palsy (For Parents)

    MedlinePlus

    ... Caring for Your Child All About Food Allergies Cerebral Palsy KidsHealth > For Parents > Cerebral Palsy Print A A ... kids who are living with the condition. About Cerebral Palsy Cerebral palsy is one of the most common ...

  9. Cerebral palsy - resources

    MedlinePlus

    Resources - cerebral palsy ... The following organizations are good resources for information on cerebral palsy : National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/disorders/cerebral_palsy/cerebral_palsy. ...

  10. Bell's Palsy.

    PubMed

    Vakharia, Kavita; Vakharia, Kalpesh

    2016-02-01

    Bell's palsy is unilateral, acute onset facial paralysis that is a common condition. One in every 65 people experiences Bell's palsy in the course of their lifetime. The majority of patients afflicted with this idiopathic disorder recover facial function. Initial treatment involves oral corticosteroids, possible antiviral drugs, and protection of the eye from desiccation. A small subset of patients may be left with incomplete recovery, synkinesis, facial contracture, or hemifacial spasm. A combination of medical and surgical treatment options exist to treat the long-term sequelae of Bell's palsy. PMID:26611696

  11. Four novel point mutations in the PMP22 gene with phenotypes of HNPP and Dejerine-Sottas neuropathy.

    PubMed

    Brožková, Dana; Mazanec, Radim; Rychlý, Zdeněk; Haberlová, Jana; Böhm, Jiří; Staněk, Jan; Plevová, Pavlína; Lisoňová, Jana; Sabová, Jana; Sakmaryová, Iva; Seeman, Pavel

    2011-11-01

    We report four novel point mutations in the PMP22 gene with two different phenotypes: mutation p.Ser79Thr arose de novo in a patient with the Dejerine-Sottas neuropathy (DSN) phenotype; and mutations c.78+5 G>A, c.320-1 G>C, and p.Trp140Stop segregated with HNPP in 5 families.Our findings show that point mutations in PMP22 may be more likely in HNPP patients than in CMT1 patients after exclusion of CMT1A/HNPP. PMID:22006697

  12. Bell's Palsy

    MedlinePlus

    ... conditions that cause injuries and damage to nerves. Knowledge gained from this research may help scientists find the definitive cause of Bell's palsy, leading to the discovery of new effective treatments for the disorder. Other ...

  13. Bell's Palsy

    MedlinePlus

    ... works and circumstances that lead to nerve damage. Knowledge gained from this research may help scientists find the definitive cause of Bell's palsy, leading to the discovery of new effective treatments for the disorder. Other ...

  14. Cerebral Palsy

    MedlinePlus

    ... Español (Spanish) Recommend on Facebook Tweet Share Compartir Cerebral palsy (CP) is a group of disorders that affect a ... ability to move and maintain balance and posture. CP is the most common motor disability in childhood. ...

  15. Bell's Palsy

    MedlinePlus

    Bell's palsy is the most common cause of facial paralysis. It usually affects just one side of the face. ... from mild to severe and include Twitching Weakness Paralysis Drooping eyelid or corner of mouth Drooling Dry ...

  16. Bell's palsy

    PubMed Central

    2014-01-01

    Introduction Bell's palsy is characterised by an acute, unilateral, partial, or complete paralysis of the face. Bell's palsy occurs in a lower motor neurone pattern. The weakness may be partial or complete, and may be associated with mild pain, numbness, increased sensitivity to sound, and altered taste. Bell's palsy is idiopathic, but a proportion of cases may be caused by re-activation of herpes virus at the geniculate ganglion of the facial nerve. Bell's palsy is most common in people aged 15 to 40 years, with a 1 in 60 lifetime risk. Most people make a spontaneous recovery within 1 month, but up to 30% show delayed or incomplete recovery. Methods and outcomes We conducted a systematic review to answer the following clinical questions: What are the effects of drug treatments for Bell's palsy in adults and children? What are the effects of physical treatments for Bell's palsy in adults and children? We searched: Medline, Embase, The Cochrane Library, and other important databases up to October 2013 (Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 13 studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: antiviral treatment, corticosteroids (alone or with antiviral treatment), hyperbaric oxygen therapy, and facial re-training. PMID:24717284

  17. Cerebral Palsy

    MedlinePlus

    ... brain is affected and which parts of the body that section of the brain controls. If CP affects both arms and both legs, ... the case of spastic CP) or to help control seizures. And some might have special surgeries to keep their arms or legs straighter and more ... Coping With Cerebral Palsy Puberty can ...

  18. Employees with Cerebral Palsy

    MedlinePlus

    ... Resources Home | Accommodation and Compliance Series: Employees with Cerebral Palsy (CP) By Eddie Whidden, MA Preface Introduction Information About ... SOAR) at http://AskJAN.org/soar. Information about Cerebral Palsy (CP) What is CP? Cerebral palsy is a ...

  19. Progressive Supranuclear Palsy

    MedlinePlus

    ... Diversity Find People About NINDS NINDS Progressive Supranuclear Palsy Information Page Synonym(s): Steele-Richardson-Olszewski Syndrome Condensed ... Additional resources from MedlinePlus What is Progressive Supranuclear Palsy? Progressive supranuclear palsy (PSP) is a rare brain ...

  20. Botulinum Toxin Type A Injection for Spastic Equinovarus Foot in Children with Spastic Cerebral Palsy: Effects on Gait and Foot Pressure Distribution

    PubMed Central

    Choi, Ja Young; Jung, Soojin; Rha, Dong-wook

    2016-01-01

    Purpose To investigate the effect of intramuscular Botulinum toxin type A (BoNT-A) injection on gait and dynamic foot pressure distribution in children with spastic cerebral palsy (CP) with dynamic equinovarus foot. Materials and Methods Twenty-five legs of 25 children with CP were investigated in this study. BoNT-A was injected into the gastrocnemius (GCM) and tibialis posterior (TP) muscles under the guidance of ultrasonography. The effects of the toxin were clinically assessed using the modified Ashworth scale (MAS) and modified Tardieu scale (MTS), and a computerized gait analysis and dynamic foot pressure measurements using the F-scan system were also performed before injection and at 1 and 4 months after injection. Results Spasticity of the ankle plantar-flexor in both the MAS and MTS was significantly reduced at both 1 and 4 months after injection. On dynamic foot pressure measurements, the center of pressure index and coronal index, which represent the asymmetrical weight-bearing of the medial and lateral columns of the foot, significantly improved at both 1 and 4 months after injection. The dynamic foot pressure index, total contact area, contact length and hind foot contact width all increased at 1 month after injection, suggesting better heel contact. Ankle kinematic data were significantly improved at both 1 and 4 months after injection, and ankle power generation was significantly increased at 4 months after injection compared to baseline data. Conclusion Using a computerized gait analysis and foot scan, this study revealed significant benefits of BoNT-A injection into the GCM and TP muscles for dynamic equinovarus foot in children with spastic CP. PMID:26847306

  1. Bell's palsy

    PubMed Central

    2011-01-01

    Introduction Bell's palsy is characterised by an acute, unilateral, partial, or complete paralysis of the face (i.e., lower motor neurone pattern). The weakness may be partial (paresis) or complete (paralysis), and may be associated with mild pain, numbness, increased sensitivity to sound, and altered taste. Bell's palsy remains idiopathic, but a proportion of cases may be caused by reactivation of herpes viruses from the geniculate ganglion of the facial nerve. Bell's palsy is most common in people aged 15 to 40 years, with a 1 in 60 lifetime risk. Most make a spontaneous recovery within 1 month, but up to 30% show delayed or incomplete recovery. Methods and outcomes We conducted a systematic review to answer the following clinical question: What are the effects of treatments in adults and children? We searched: Medline, Embase, The Cochrane Library, and other important databases up to June 2010 (Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 14 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review we present information relating to the effectiveness and safety of the following interventions: antiviral treatment, corticosteroids (alone or plus antiviral treatment), hyperbaric oxygen therapy, facial nerve decompression surgery, and facial retraining. PMID:21375786

  2. [Cerebral palsy].

    PubMed

    Malagón Valdez, Jorge

    2007-01-01

    The term cerebral palsy (CP), is used for a great number of clinical neurological syndromes. The syndromes are characterized by having a common cause, motor defects. It is important, because they can cause a brain damage by presenting motor defects and some associated deficiencies, such as mental deficiency, epilepsy, language and visual defects and pseudobulbar paralysis, with the non-evolving fact. Some authors prefer using terms such as "non-evolving encephalopathies". In the treatment the utility of prevention programs of early stimulation and special rehabilitation methods, and treatment of associated deficiencies such as epilepsy, mental deficiency, language, audition and visual problems, and the attention deficit improve the prognosis in an important way. The prognosis depends on the severity of the disease and the associated manifestations. PMID:18422084

  3. United Cerebral Palsy

    MedlinePlus

    ... of UCP blog for the latest updates. United Cerebral Palsy UCP educates, advocates and provides support services to ... Special Needs Planning Sprint Relay Copyright © 2015 United Cerebral Palsy 1825 K Street NW Suite 600 Washington, DC ...

  4. Progressive supranuclear palsy

    MedlinePlus

    Dementia-nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear ... Progressive supranuclear palsy is a condition that causes symptoms similar to those of Parkinson disease . It involves damage to many cells ...

  5. Aging and Cerebral Palsy.

    ERIC Educational Resources Information Center

    Networker, 1993

    1993-01-01

    This special edition of "The Networker" contains several articles focusing on aging and cerebral palsy (CP). "Aging and Cerebral Palsy: Pathways to Successful Aging" (Jenny C. Overeynder) reports on the National Invitational Colloquium on Aging and Cerebral Palsy held in April 1993. "Observations from an Observer" (Kathleen K. Barrett) describes…

  6. [Etiology of cerebral palsy].

    PubMed

    Jaisle, F

    1996-01-01

    The "perinatal asphyxia" is regarded to be one of the causes of cerebral palsy, though in the very most of the children with cerebral palsy there is found no hypoxia during labour. It should be mentioned, that the definition of "perinatal" and "asphyxia" neither are unic nor concret. And also there is no correlation between nonreassuring fetal heart rate patterns and acidosis in fetal blood with the incidence of cerebral palsy. Numerous studies in pregnant animals failed in proving an acute intrapartal hypoxia to be the origin of the cerebral palsy. Myers (1975) describes four patterns of anatomic brain damage after different injuries. Only his so called oligo-acidotic hypoxia, which is protracted and lasts over a longer time is leading to brain injury, which can be regarded in analogy to the injury of children with cerebral palsy. Summarising the update publications about the causes of cerebral palsy and the studies in pregnant animals there is no evidence that hypoxia during labour may be the cause of cerebral palsy. There is a great probability of a pre(and post-)natal origin of brain injury (for instance a periventricular leucomalacia found after birth) which leads to cerebral palsy. Short after labour signs of a so called "asphyxia" may occur in addition to this preexisting injury and misrepresent the cause of cerebral palsy. Finally the prepartal injury may cause both: Cerebral palsy and hypoxia. PMID:9035826

  7. Charcot-Marie-Tooth Disease

    MedlinePlus

    ... a different neuropathy distinct from CMT1A called hereditary neuropathy with predisposition to pressure palsy (HNPP) is caused ... PMP-22 gene result in episodic, recurrent demyelinating neuropathy. CMT1B is an autosomal dominant disease caused by ...

  8. Cerebral Palsy (CP) Quiz

    MedlinePlus

    ... Submit Button Past Emails CDC Features Pop Quiz: Cerebral Palsy Language: English Español (Spanish) Recommend on Facebook Tweet ... Sandy is the parent of a child with cerebral palsy and the Board President of Gio’s Garden , a ...

  9. Rehabilitation in cerebral palsy.

    PubMed Central

    Molnar, G. E.

    1991-01-01

    Cerebral palsy is the most frequent physical disability of childhood onset. Over the past four decades, prevalence has remained remarkably constant at 2 to 3 per 1,000 live births in industrialized countries. In this article I concentrate on the rehabilitation and outcome of patients with cerebral palsy. The epidemiologic, pathogenetic, and diagnostic aspects are highlighted briefly as they pertain to the planning and implementation of the rehabilitation process. PMID:1866952

  10. Nanomedicine in cerebral palsy

    PubMed Central

    Balakrishnan, Bindu; Nance, Elizabeth; Johnston, Michael V; Kannan, Rangaramanujam; Kannan, Sujatha

    2013-01-01

    Cerebral palsy is a chronic childhood disorder that can have diverse etiologies. Injury to the developing brain that occurs either in utero or soon after birth can result in the motor, sensory, and cognitive deficits seen in cerebral palsy. Although the etiologies for cerebral palsy are variable, neuroinflammation plays a key role in the pathophysiology of the brain injury irrespective of the etiology. Currently, there is no effective cure for cerebral palsy. Nanomedicine offers a new frontier in the development of therapies for prevention and treatment of brain injury resulting in cerebral palsy. Nanomaterials such as dendrimers provide opportunities for the targeted delivery of multiple drugs that can mitigate several pathways involved in injury and can be delivered specifically to the cells that are responsible for neuroinflammation and injury. These materials also offer the opportunity to deliver agents that would promote repair and regeneration in the brain, resulting not only in attenuation of injury, but also enabling normal growth. In this review, the current advances in nanotechnology for treatment of brain injury are discussed with specific relevance to cerebral palsy. Future directions that would facilitate clinical translation in neonates and children are also addressed. PMID:24204146

  11. Cerebral Palsy Litigation

    PubMed Central

    Sartwelle, Thomas P.

    2015-01-01

    The cardinal driver of cerebral palsy litigation is electronic fetal monitoring, which has continued unabated for 40 years. Electronic fetal monitoring, however, is based on 19th-century childbirth myths, a virtually nonexistent scientific foundation, and has a false positive rate exceeding 99%. It has not affected the incidence of cerebral palsy. Electronic fetal monitoring has, however, increased the cesarian section rate, with the expected increase in mortality and morbidity risks to mothers and babies alike. This article explains why electronic fetal monitoring remains endorsed as efficacious in the worlds’ labor rooms and courtrooms despite being such a feeble medical modality. It also reviews the reasons professional organizations have failed to condemn the use of electronic fetal monitoring in courtrooms. The failures of tort reform, special cerebral palsy courts, and damage limits to stem the escalating litigation are discussed. Finally, the authors propose using a currently available evidence rule—the Daubert doctrine that excludes “junk science” from the courtroom—as the beginning of the end to cerebral palsy litigation and electronic fetal monitoring’s 40-year masquerade as science. PMID:25183322

  12. Genetics Home Reference: progressive supranuclear palsy

    MedlinePlus

    ... Home Health Conditions progressive supranuclear palsy progressive supranuclear palsy Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Progressive supranuclear palsy is a brain disorder that affects movement, vision, ...

  13. Therapeutic interventions in cerebral palsy.

    PubMed

    Patel, Dilip R

    2005-11-01

    Various therapeutic interventions have been used in the management of children with cerebral palsy. Traditional physiotherapy and occupational therapy are widely used interventions and have been shown to be of benefit in the treatment of cerebral palsy. Evidence in support of the effectiveness of the neurodevelopmental treatment is equivocal at best. There is evidence to support the use and effectiveness of neuromuscular electrical stimulation in children with cerebral palsy. The effectiveness of many other interventions used in the treatment of cerebral palsy has not been clearly established based on well-controlled trials. These include: sensory integration, body-weight support treadmill training, conductive education, constraint-induced therapy, hyperbaric oxygen therapy, and the Vojta method. This article provides an overview of salient aspects of popular interventions used in the management of children with cerebral palsy. PMID:16391455

  14. Short-Term Effect of Botulinum Toxin A Injection on Spastic Equinovarus Foot in Cerebral Palsy Patients: A Study Using the Foot Pressure Measurement System

    PubMed Central

    Son, Su Min; Park, In Sik

    2015-01-01

    Objective To evaluate the therapeutic effect of botulinum toxin A (BTX-A) injection on spastic gastrocnemius (GCM) and tibialis posterior muscles (TPo) by using the foot pressure measurement system (FPMS). Methods Eighteen ambulatory CP patients were recruited in this study. BTX-A was injected into the GCM at a dose of 6-12 units/kg and TPo at a dose of 4-9 units/kg according to the severity of equinus and varus deformity. Foot contact pattern, pressure time integral (PTI), coronal index using the FPMS and Modified Ashworth Scale (MAS), and visual inspection of gait pattern were used for evaluation of the therapeutic effect of BTX-A injection. Clinical and FPMS data were statistically analyzed according to the muscle group. Results A significant decrease in the MAS score of the GCM and TPo was observed, and spastic equinovarus pattern during gait showed improvement after injection. The GCM+TPo injection group showed a significant decrease in forefoot, lateral forefoot pad, and lateral column PTI, and a significant increase in hindfoot PTI and coronal index. In the GCM only injection group, forefoot PTI and lateral column PTI were significantly decreased and hindfoot PTI was significantly increased. The TPo only injection group showed a significant decrease in lateral column PTI and a significant increase in the coronal index. Change in PTI in the hindfoot showed a significant correlation with the change in MAS score of the GCM. Change in PTI of the lateral column and coronal index showed a significant correlation with the change in MAS score of the TPo. Conclusion The FPMS demonstrated the quantitative therapeutic effect of BTX-A on abnormal pressure distribution in equinovarus foot in detail. The FPMS can be a useful additional tool for evaluation of the effect of BTX-A injection. PMID:25750865

  15. Cranial nerve palsies in childhood

    PubMed Central

    Lyons, C J; Godoy, F; ALQahtani, E

    2015-01-01

    We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the globe in adduction related to the innervation of the lateral rectus by the III nerve causing co-contraction in adduction. Clinical features that may be of concern in adulthood may not be relevant in childhood; whereas the presence of mydriasis in III palsy suggests a compressive aetiology in adults, this is not the case in children. However, the frequency of associated CNS abnormalities in III palsy and the risk of tumour in VI palsy can be indications for early neuroimaging depending on presenting features elicited through a careful history and clinical examination. The latter should include the neighbouring cranial nerves. We discuss the impact of our evolving knowledge of congenital cranial dysinnervation syndromes on this field. PMID:25572578

  16. The Effect of Treatment of Obstructive Sleep Apnea on Quality of Life in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Hsiao, Kai Hsun; Nixon, Gillian M.

    2008-01-01

    Benefits of treatment for obstructive sleep apnea (OSA) in children with cerebral palsy could differ from those in otherwise healthy children. We examined the effects of OSA treatment by comparing a group of children with cerebral palsy treated with adenotonsillectomy or continuous positive airway pressure (CPAP) by nasal mask with controls who…

  17. Palsies of Cranial Nerves That Control Eye Movement

    MedlinePlus

    ... Medical News Palsies of Cranial Nerves That Control Eye Movement By Michael Rubin, MDCM NOTE: This is the ... Gaze Palsies Palsies of Cranial Nerves That Control Eye Movement Third Cranial Nerve (Oculomotor Nerve) Palsy Fourth Cranial ...

  18. Progressive supranuclear palsy.

    PubMed

    Boeve, Bradley F

    2012-01-01

    Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy which can manifest clinically in a variety of syndromes. In this review, the classic and most common variant syndrome -PSP-Richardson's syndrome (PSP-RS) -is the focus, with the core clinical features, varying cognitive/motor/neuropsychiatric/sleep manifestations, neuropsychological findings, and typical neuroimaging findings all reviewed. Management strategies are also discussed. Of particular interest are the recently commenced clinical trials involving agents which affect key steps in the presumed pathogenesis of the tauopathies. The distinctive and recognizable characteristics of PSP-RS and advent of clinical trials involving potential disease modifying agents underscore the importance of identifying patients with this disorder and encouraging their involvement in trials. PMID:22166432

  19. Monocular Elevation Deficiency - Double Elevator Palsy

    MedlinePlus

    ... Condiciones Chinese Conditions Monocular Elevation Deficiency/ Double Elevator Palsy En Español Read in Chinese What is monocular elevation deficiency (Double Elevator Palsy)? Monocular Elevation Deficiency, also known by the older ...

  20. Neuroevolutional Approach to Cerebral Palsy and Speech.

    ERIC Educational Resources Information Center

    Mysak, Edward D.

    Intended for cerebral palsy specialists, the book emphasizes the contribution that a neuroevolutional approach to therapy can make to habilitation goals of the child with cerebral palsy and applies the basic principles of the Bobath approach to therapy. The first section discusses cerebral palsy as a reflection of disturbed neuro-ontogenisis and…

  1. Facial nerve palsy due to birth trauma

    MedlinePlus

    Seventh cranial nerve palsy due to birth trauma ... these factors do not lead to facial nerve palsy or birth trauma. ... The most common form of facial nerve palsy due to birth trauma ... This part controls the muscles around the lips. The muscle ...

  2. Injection nerve palsy

    PubMed Central

    Kakati, Arindhom; Bhat, Dhananjaya; Devi, Bhagavathula Indira; Shukla, Dhaval

    2013-01-01

    Objective: To study the clinical profile and outcome of surgery for injection nerve palsies. Materials and Methods: This is a retrospective study of patients with INP who were treated at our institute during May 2000 to May 2009. Clinical, electroneuromyography (ENMG), and operative findings were noted. Intraoperative nerve action potential monitoring was not used in any case. Outcome of patients who were followed was reviewed. Results: INP comprised 92 (11%) of 837 nerve injury patients. Seventy one patients were children less than 16 years. The nerves involved were sciatic in 80 patients, radial in 8, and others in four. Fifty seven patients had power, grade 0/5. ENMG studies revealed absent compound muscle action potential in 64 and absent sensory nerve action potential in 67 patients. Thirty nine (42.3%) of 92 patients underwent surgery. The mean duration since injury in these patients was 5.2 months (3 months to 11 months). All underwent neurolysis. Only 18 patients who underwent surgery had a follow up of more than 3 months. Ten (55.5%) patients had good or fair outcome after surgery. Except for grade of motor deficit prior to surgery, none of the variables were found to significantly affect the outcome. Conclusion: The outcome of INP is generally good and many patients recover spontaneously. The outcome of surgery is dependent on preoperative motor power. PMID:23546341

  3. Delayed facial palsy in Miller Fisher syndrome.

    PubMed

    Tan, Cheng-Yin; Yuki, Nobuhiro; Shahrizaila, Nortina

    2015-11-15

    Miller Fisher syndrome is characterised by the triad of ophthalmoplegia, ataxia and areflexia. However, facial palsy can occur during the course of the illness although development of facial palsy when other cardinal signs of Miller Fisher syndrome have reached nadir or improving, is unusual. This delayed appearance of facial palsy can be easily overlooked by the treating clinician. Here, we report four patients with Miller Fisher syndrome and delayed-onset facial palsy. We discuss the possible underlying reasons behind the delay in facial palsy. PMID:26277343

  4. [Facial palsy in Equatorial Africa].

    PubMed

    Pietruski, J

    1993-01-01

    The author presents the analysis of 29 cases of facial palsy collected in Shaba, Zaire (former Katanga, Belgian Congo) between 1984-1988 in the region called Copperbelt. Because to date there has been no work done on these problems in this part of Africa, it would be interesting to present a short report. Patients with facial palsy came to the ENT Department mostly for other reasons, and very late. Only 5 patients came before 3 mos after the onset. The different etiologies were as follows: 2--post traumatic, 7--otogenic, 9--tumors, 4--iatrogenic, 6--Bells palsy, 1--other. The assessment, comparison and the treatment of such cases in an underdeveloped country is very difficult or almost impossible. The small number of cases is insufficient for any conclusions, but the author tried to get some data, which would be a starting point in the future researches. PMID:8255587

  5. Cerebral Palsy: A Dental Update

    PubMed Central

    Sehrawat, Nidhi; Bansal, Kalpana; Chopra, Radhika

    2014-01-01

    ABSTRACT Special and medically compromised patients present a unique population that challenges the dentist’s skill and knowledge. Providing oral care to people with cerebral palsy (CP) requires adaptation of the skills we use everyday. In fact, most people with mild or moderate forms of CP can be treated successfully in the general practice setting. This article is to review various dental considerations and management of a CP patient. How to cite this article: Sehrawat N, Marwaha M, Bansal K, Chopra R. Cerebral Palsy: A Dental Update. Int J Clin Pediatr Dent 2014;7(2):109-118. PMID:25356010

  6. [Management of peripheral facial nerve palsy in children].

    PubMed

    Tabarki, B

    2014-10-01

    Peripheral facial nerve palsy may (secondary) or may not have a detectable cause (idiopathic facial palsy or Bell's palsy). Idiopathic facial palsy is the common form of facial palsy. It remains diagnosis by exclusion. The prognosis is more favourable in children than in adults. We present current diagnostic procedures and recommendations regarding treatment in children. PMID:25048647

  7. Cerebral palsy and aging

    PubMed Central

    Haak, Peterson; Lenski, Madeleine; Hidecker, Mary Jo Cooley; Li, Min; Paneth, Nigel

    2014-01-01

    Cerebral palsy (CP), the most common major disabling motor disorder of childhood, is frequently thought of as a condition that affects only children. Deaths in children with CP, never common, have in recent years become very rare, unless the child is very severely and multiply disabled. Thus, virtually all children assigned the diagnosis of CP will survive into adulthood. Attention to the adult with CP has been sparse, and the evolution of the motor disorder as the individual moves through adolescence, young adulthood, middle age, and old age is not well understood. Nor do we know what happens to other functional domains, such as communication and eating behavior, in adults with CP. Although the brain injury that initially causes CP by definition does not progressively worsen through the lifetime, the effects of CP manifest differently throughout the life span. The aging process must inevitably interact with the motor disorder, but we lack systematic, large-scale follow-up studies of children with CP into adulthood and through adulthood with thorough assessments performed over time. In this paper we summarize what is known of the epidemiology of CP throughout the life span, beginning with mortality and life expectancy, then survey what is known of functioning, ability, and quality of life of adults with CP. We conclude by describing a framework for future research on CP and aging that is built around the World Health Organization's International Classification of Functioning, Disability, and Health (ICF) and suggest specific tools and approaches for conducting that research in a sound manner. PMID:19740206

  8. Japanese neuropathy patients with peripheral myelin protein-22 gene aneuploidy

    SciTech Connect

    Lebo, R.V.; Li, L.Y.; Flandermeyer, R.R.

    1994-09-01

    Peripheral myelin protein (PMP-22) gene aneuploidy results in Charcot-Marie-Tooth disease Type 1A (CMT1A) and the Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) in Japanese patients as well as Caucasian Americans. Charcot-Marie-Tooth disease (CMT), the most common genetic neuropathy, results when expression of one of at least seven genes is defective. CMT1A, about half of all CMT mutations, is usually associated with a duplication spanning the peripheral myelin protein-22 gene on distal chromosome band 17p11.2. Autosomal dominant HNPP (hereditary pressure and sensory neuropathy, HPSN) results from a deletion of the CMT1A gene region. Multicolor in situ hybridization with PMP-22 gene region probe characterized HNPP deletion reliably and detected all different size duplications reported previously. In summary, 72% of 28 Japanese CMT1 (HMSNI) patients tested had the CMT1A duplication, while none of the CMT2 (HMSNII) or CMT3 (HMSNIII) patients had a duplication. Three cases of HNPP were identified by deletion of the CMT1A gene region on chromosome 17p. HNPP and CMT1A have been reported to result simultaneously from the same unequal recombination event. The lower frequency of HNPP compared to CMT1A suggests that HNPP patients have a lower reproductive fitness than CMT1A patients. This result, along with a CMT1A duplication found in an Asian Indian family, demonstrates the broad geographic distribution and high frequency of PMP-22 gene aneuploidy.

  9. Common questions about Bell palsy.

    PubMed

    Albers, Janet R; Tamang, Stephen

    2014-02-01

    Bell palsy is an acute affliction of the facial nerve, resulting in sudden paralysis or weakness of the muscles on one side of the face. Testing patients with unilateral facial paralysis for diabetes mellitus or Lyme disease is not routinely recommended. Patients with Lyme disease typically present with additional manifestations, such as arthritis, rash, or facial swelling. Diabetes may be a comorbidity of Bell palsy, but testing is not needed in the absence of other indications, such as hypertension. In patients with atypical symptoms, magnetic resonance imaging with contrast enhancement can be used to rule out cranial mass effect and to add prognostic value. Steroids improve resolution of symptoms in patients with Bell palsy and remain the preferred treatment. Antiviral agents have a limited role, and may improve outcomes when combined with steroids in patients with severe symptoms. When facial paralysis is prolonged, surgery may be indicated to prevent ocular desiccation secondary to incomplete eyelid closure. Facial nerve decompression is rarely indicated or performed. Physical therapy modalities, including electrostimulation, exercise, and massage, are neither beneficial nor harmful. PMID:24506123

  10. Bone age in cerebral palsy

    PubMed Central

    Miranda, Eduardo Régis de Alencar Bona; Palmieri, Maurício D'arc; de Assumpção, Rodrigo Montezuma César; Yamada, Helder Henzo; Rancan, Daniela Regina; Fucs, Patrícia Maria de Moraes Barros

    2013-01-01

    Objective To compare the chronological age and bone age among cerebral palsy patients in the outpatient clinic and its correlation with the type of neurological involvement, gender and functional status. Methods 401 patients with spastic cerebral palsy, and ages ranging from three months to 20 years old, submitted to radiological examination for bone age and analyzed by two independent observers according Greulich & Pyle. Results In the topographic distribution, there was a significant delay (p<0.005) in tetraparetic (17.7 months), hemiparetic (10.1 months), and diparetic patients (7.9 months). In the hemiparetic group, the mean bone age in the affected side was 96.88 months and the uncompromised side was 101.13 months (p<0.005). Regarding functional status, the ambulatory group showed a delay of 18.73 months in bone age (p<0.005). Comparing bone age between genders, it was observed a greater delay in males (13.59 months) than in females (9.63 months), but not statistically significant (p = 0.54). Conclusion There is a delay in bone age compared to chronological age influenced by the topography of spasticity, functional level and gender in patients with cerebral palsy. Level of Evidence IV, Case Series. PMID:24453693

  11. Mobility Experiences of Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Palisano, Robert J.; Shimmell, Lorie J.; Stewart, Debra; Lawless, John J.; Rosenbaum, Peter L.; Russell, Dianne J.

    2009-01-01

    The purpose of this study was to describe how youth with cerebral palsy experience mobility in their daily lives using a phenomenological approach. The participants were 10 youth with cerebral palsy, 17 to 20 years of age, selected using purposeful sampling with maximum variation strategies. A total of 14 interviews were completed. Transcripts…

  12. Mobility Experiences of Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Palisano, Robert J.; Shimmell, Lorie J.; Stewart, Debra; Lawless, John J.; Rosenbaum, Peter L.; Russell, Dianne J.

    2009-01-01

    The purpose of this study was to describe how youth with cerebral palsy experience mobility in their daily lives using a phenomenological approach. The participants were 10 youth with cerebral palsy, 17 to 20 years of age, selected using purposeful sampling with maximum variation strategies. A total of 14 interviews were completed. Transcripts

  13. Clinical practice guideline: Bell's Palsy executive summary.

    PubMed

    Baugh, Reginald F; Basura, Gregory J; Ishii, Lisa E; Schwartz, Seth R; Drumheller, Caitlin Murray; Burkholder, Rebecca; Deckard, Nathan A; Dawson, Cindy; Driscoll, Colin; Gillespie, M Boyd; Gurgel, Richard K; Halperin, John; Khalid, Ayesha N; Kumar, Kaparaboyna Ashok; Micco, Alan; Munsell, Debra; Rosenbaum, Steven; Vaughan, William

    2013-11-01

    The American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) has published a supplement to this issue featuring the new Clinical Practice Guideline: Bell's Palsy. To assist in implementing the guideline recommendations, this article summarizes the rationale, purpose, and key action statements. The 11 recommendations developed encourage accurate and efficient diagnosis and treatment and, when applicable, facilitate patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell's palsy. There are myriad treatment options for Bell's palsy; some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, there are numerous diagnostic tests available that are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have an unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy. PMID:24190889

  14. [Neurorehabilitation of Patients with Cerebral Palsy].

    PubMed

    Solopova, I A; Moshonkina, T R; Umnov, V V; Vissarionov, S V; Baindurashvili, A G; Gerasimenko, Yu P

    2015-01-01

    Cerebral palsy is one of the common diseases that cause significant motor impairments. This review deals with new methods of motor rehabilitation of children with cerebral palsy in terms of modern physiology, as well as with summarized and analyzed results of experimental studies on the effectiveness of these methods. PMID:26485797

  15. New Hope for Children with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Obringer, S. John

    This paper explains the use of a unique experimental therapy for students with a type of cerebral palsy specifically called Botox. Botulinum Toxin Type A has been tried on a sizable number of students with cerebral palsy in clinical settings to reduce spastic and dystonic movements. By injecting Botox into overly tight heel cords, a normal or near…

  16. UNDERSTANDING CEREBRAL PALSY, A HANDBOOK FOR PARENTS.

    ERIC Educational Resources Information Center

    HATTON, DANIEL A.

    THIS MANUAL, WRITTEN BY A PSYCHOLOGIST, PROVIDES BASIC INFORMATION ABOUT THE NATURE OF CEREBRAL PALSY AND DISCUSSES FEELINGS AND REACTIONS OF PARENTS OF CEREBRAL PALSIED CHILDREN. INFORMATION ABOUT THE BRAIN AND BRAIN DAMAGE IN RELATION TO MOTOR AND EMOTIONAL FUNCTION, PERCEPTION AND INTELLIGENCE IS PRESENTED. PART TWO GIVES INSIGHT INTO PROBLEMS…

  17. Caring for Children with Cerebral Palsy: A Team Approach.

    ERIC Educational Resources Information Center

    Dormans, John P., Ed.; Pellegrino, Louis, Ed.

    Twenty-one papers on caring for children with cerebral palsy are organized into four sections, including: (1) cerebral palsy and the interdisciplinary team approach; (2) management of impairments related to cerebral palsy; (3) preventing disability by optimizing function of the child with cerebral palsy; and (4) preventing handicap by creating

  18. Caring for Children with Cerebral Palsy: A Team Approach.

    ERIC Educational Resources Information Center

    Dormans, John P., Ed.; Pellegrino, Louis, Ed.

    Twenty-one papers on caring for children with cerebral palsy are organized into four sections, including: (1) cerebral palsy and the interdisciplinary team approach; (2) management of impairments related to cerebral palsy; (3) preventing disability by optimizing function of the child with cerebral palsy; and (4) preventing handicap by creating…

  19. CEREBRAL PALSY. PRENTICE-HALL FOUNDATIONS OF SPEECH PATHOLOGY SERIES.

    ERIC Educational Resources Information Center

    CHANCE, BURTON, JR.; MCDONALD, EUGENE T.

    THIS INTRODUCTORY TEXT ON CEREBRAL PALSY IS DIVIDED INTO TWO SECTIONS. THE FIRST SECTION OF THE BOOK CONTAINS INFORMATION ABOUT UNDERSTANDING THE MEANING OF CEREBRAL PALSY, PROGRAMS FOR THOSE WITH CEREBRAL PALSY, THE NEUROLOGICAL BASES, ETIOLOGY, AND DIAGNOSIS, AND THE CLASSIFICATION OF CEREBRAL PALSY. PROBLEMS OFTEN ASSOCIATED WITH CEREBRAL PALSY…

  20. Valacyclovir for the treatment of Bell's palsy.

    PubMed

    Hato, Naohito; Sawai, Naoki; Teraoka, Masato; Wakisaka, Hiroyuki; Takahashi, Hirotaka; Hinohira, Yasuyuki; Gyo, Kiyofumi

    2008-10-01

    Despite recent evidence suggesting that Bell's palsy is associated with reactivation of alfa-herpes viruses, the disease has been treated empirically, and the use of valacyclovir has not been definitively established. In 2007, two prospective, randomised, placebo-controlled trials evaluating valacyclovir were reported in patients with Bell's palsy. One demonstrated that valacyclovir/prednisolone therapy was statistically more effective than placebo/prednisolone therapy in improving the recovery of patients with Bell's palsy, excluding zoster sine herpete. However, considering the cost-benefit ratio of this treatment and the limitations of virological diagnoses, we recommend that valacyclovir should be used in cases of severe palsy within 3 days after the onset of Bell's palsy. PMID:18778190

  1. Clinical Correlations in Cerebral Palsy

    PubMed Central

    MINCIU, Ioana

    2012-01-01

    ABSTRACT Background: Cerebral palsy (CP) is a group of persistent (but not necessarily unchanged), movement, posture, muscle tone and motor skills disorders non-progressive, with early onset, due to non-progressive impairments, occurring on an immature brain or a brain under development (prenatal, perinatal, postnatal during the first 3-4 years of life). It is associated to a variable extent with: cognitive disorders, epilepsy, sensory deficits, behaviour disorders. Aim: The study of the correlations between the clinical forms/subtypes of CP, comorbidities, and severity of functional impairment. Material and method: It is a retrospective trial aimed only at patients with the diagnosis of cerebral palsy admitted at Paediatric Neurology Clinic of the "Alexandru Obregia" Clinical Hospital in 2010. Results, discussions and conclusions: Patients with cerebral palsy corresponding with the criteria for inclusion: 379. The spastic CP type has prevailed. Comorbidities like mental retardation, epilepsy, and ophthalmic disorders were found with greater frequency than in the studies in the literature. The unilateral spastic form was statistically correlated with slight functional impairment (GMFCS I), with the absence of comorbidities or mild mental retardation, or with focal epilepsy when there is epilepsy. The bilateral spastic, tetraparetic and dyskinetic forms were correlated significantly with severe functional impairment (GMFCS IV, V), with profound or severe retardation, microcephaly, swallowing disorders, statural, ponderal hypotrophy, blindness and epilepsy. The bilateral spastic paraparetic form, which in the literature is mentioned as having fewer associated disorders (for example strabismus, slight retardation), when there is severe functional impairment, it may have the same comorbidities as the tetraparetic form (similar to the cases studied in the hospital). Comorbidities are the main admission cause and it correlates with the severity and prognosis. PMID:23483832

  2. Early Observations on Facial Palsy.

    PubMed

    Pearce, J M S

    2015-01-01

    Before Charles Bell's eponymous account of facial palsy, physicians of the Graeco-Roman era had chronicled the condition. The later neglected accounts of the Persian physicians Abu al-Hasan Ali ibn Sahl Rabban al-Tabari and Abu Bakr Muhammad ibn Zakarīya Rāzi ("Rhazes") and Avicenna in the first millennium are presented here as major descriptive works preceding the later description by Stalpart van der Wiel in the seventeenth century and those of Friedreich and Bell at the end of the eighteenth and the beginning of the nineteenth centuries. PMID:25513852

  3. Bilateral peroneal palsy after weightlifting.

    PubMed

    Kyavar, Leila; Heckmann, Josef G

    2013-09-01

    In a 48-year-old otherwise healthy man, a bilateral common peroneal palsy was diagnosed clinically and neurophysiologically. He reported on strength training with weights in both arms, lifting the weights and his upper body from a deep squatting position with broadly positioned legs akimbo in a hitherto unusual intensity. Regarding the pathophysiological mechanisms, 2 options are considered: first, stretching of the nerve at the fascia of the peroneal longus muscle and along the fibula neck, and second, compression of the nerve during squatting with weights loaded and with strongly activated anterior tibial and peroneal muscles. PMID:23615488

  4. Radiation-induced long thoracic nerve palsy

    SciTech Connect

    Pugliese, G.N.; Green, R.F.; Antonacci, A.

    1987-09-15

    The incidence of long thoracic nerve palsy after radical mastectomy has been documented to be approximately 10%. No cases have been reported after the more recent treatment for breast cancer, lumpectomy with axillary dissection. This more recent surgical procedure is customarily followed by aggressive radiation therapy to the remaining breast tissue. This is the first case report of a patient with radiation-induced long thoracic nerve palsy. The patient was a young woman who underwent left breast quadrantectomy and axillary dissection for breast cancer. After radiation therapy, she had isolated left long thoracic nerve palsy. The diagnosis was confirmed by electrodiagnostic studies. Almost full recovery occurred after 5 months.

  5. Temperament of premature infants with cerebral palsy

    PubMed Central

    Ryu, Hyo Jeong; Don Kim, Kyoung

    2015-01-01

    [Purpose] The purpose of this study was to examine the infant temperaments of children with cerebral palsy due to premature birth. [Subjects and Methods] Data were collected through questionnaires sent to 118 mothers of infants diagnosed with cerebral palsy due to premature birth. [Results] Different infant temperament scores were obtained according to the degrees of disability, type of palsy, birth weights, gestational age, and periods of hospitalization in an NICU; however, the differences were not statistically significant. [Conclusion] Additional comprehensive studies are necessary in order to understand the infant temperaments of newborns with cerebral palsy due to premature birth, as a prerequisite to providing efficient intervention programs supporting the childrens development and growth, and to verify statistical significance. PMID:25931751

  6. Central conduction time in progressive supranuclear palsy.

    PubMed

    Pakalnis, A; Drake, M E; Huber, S; Paulson, G; Phillips, B

    1992-01-01

    Progressive nuclear palsy (PSP) is a parkinson-like extrapyramidal disorder with pathological evidence of brain stem demyelination. We studied brain stem auditory (BAEP) and somatosensory (SSEP) evoked potentials in 8 patients with progressive supra-nuclear palsy to look for evidence of such central demyelination. We found minor alterations in BAEPs and frequently abnormal SSEPs, suggestive of brain stem white matter involvement in this disorder. Evoked potentials may assist in the differentiation of PSP from other parkinsonian conditions. PMID:1541246

  7. Joubert syndrome labeled as hypotonic cerebral palsy

    PubMed Central

    Dekair, Lubna H.; Kamel, Hussein; El-Bashir, Haitham O.

    2014-01-01

    Joubert syndrome (JS) is a rare autosomal recessive disorder with cerebellar vermis hypoplasia and complex brainstem malformation. The diagnosis of cases can be difficult as the presentation can be similar to cases of cerebral palsy. We present a case of JS in an 18-month-old girl who presented to pediatric rehabilitation with a diagnosis of hypotonic cerebral palsy and abnormal eye movements. The brain MRI confirmed the typical brain malformations. PMID:24983287

  8. Cerebral Palsy Gait, Clinical Importance

    PubMed Central

    TUGUI, Raluca Dana; ANTONESCU, Dinu

    2013-01-01

    ABSTRACT Cerebral palsy refers to a lesion on an immature brain, that determines permanent neurological disorders. Knowing the exact cause of the disease does not alter the treatment management. The etiology is 2-2.5/1000 births and the rate is constant in the last 40-50 years because advances in medical technologies have permitted the survival of smaller and premature new born children. Gait analysis has four directions: kinematics (represents body movements analysis without calculating the forces), kinetics (represents body moments and forces), energy consumption (measured by oximetry), and neuromuscular activity (measured by EMG). Gait analysis can observe specific deviations in a patient, allowing us to be more accurate in motor diagnoses and treatment solutions: surgery intervention, botulinum toxin injection, use of orthosis, physical kinetic therapy, oral medications, baclofen pump. PMID:24790675

  9. Genetics of Progressive Supranuclear Palsy

    PubMed Central

    Im, Sun Young; Kim, Young Eun; Kim, Yun Joong

    2015-01-01

    Progressive supranuclear palsy (PSP) is a neurodegenerative syndrome that is clinically characterized by progressive postural instability, supranuclear gaze palsy, parkinsonism and cognitive decline. Pathologically, diagnosis of PSP is based on characteristic features, such as neurofibrillary tangles, neutrophil threads, tau-positive astrocytes and their processes in basal ganglia and brainstem, and the accumulation of 4 repeat tau protein. PSP is generally recognized as a sporadic disorder; however, understanding of genetic background of PSP has been expanding rapidly. Here we review relevant publications to outline the genetics of PSP. Although only small number of familial PSP cases have been reported, the recognition of familial PSP has been increasing. In some familial cases of clinically probable PSP, PSP pathologies were confirmed based on NINDS neuropathological diagnostic criteria. Several mutations in MAPT, the gene that causes a form of familial frontotemporal lobar degeneration with tauopathy, have been identified in both sporadic and familial PSP cases. The H1 haplotype of MAPT is a risk haplotype for PSP, and within H1, a sub-haplotype (H1c) is associated with PSP. A recent genome-wide association study on autopsyproven PSP revealed additional PSP risk alleles in STX6 and EIF2AK3. Several heredodegenerative parkinsonian disorders are referred to as PSP-look-alikes because their clinical phenotype, but not their pathology, mimics PSP. Due to the fast development of genomics and bioinformatics, more genetic factors related to PSP are expected to be discovered. Undoubtedly, these studies will provide a better understanding of the pathogenesis of PSP and clues for developing therapeutic strategies. PMID:26413239

  10. Hypoglossal nerve palsy complicating a case of infectious mononucleosis

    PubMed Central

    Sibert, J. R.

    1972-01-01

    A case of infectious mononucleosis complicated by the rare neurological complication of left isolated hypoglossal nerve palsy is described. The literature on cranial nerve palsies in infectious mononucleosis is briefly reviewed. PMID:4650785

  11. Attitudes and Needs of Parents of Cerebral Palsied Children.

    ERIC Educational Resources Information Center

    Knott, Gladys P.

    1979-01-01

    The article reviews the literature in the following five areas of concern for parents of cerebral palsied children: causes and effects of cerebral palsy, family dynamics, counseling, educational and vocational programs, and employment. (PHR)

  12. Bell's Palsy: Treatment with Steroids and Antiviral Drugs

    MedlinePlus

    ... based Guideline for PATIENTS and their FAMILIES BELL’S PALSY: TREATMENT WITH STEROIDS AND ANTIVIRAL DRUGS This information ... of steroids and antiviral drugs for treating Bell’s palsy. Neurologists from the AAN are doctors who identify ...

  13. Genetic factors for nerve susceptibility to injuries – lessons from PMP22 deficiency

    PubMed Central

    Li, Jun

    2014-01-01

    Genetic factors may be learnt from families with gene mutations that render nerve-injury susceptibility even to ordinary physical activities. A typical example is hereditary neuropathy with liability to pressure palsies (HNPP). HNPP is caused by a heterozygous deletion of PMP22 gene. PMP22 deficiency disrupts myelin junctions (such as tight junction and adherens junctions), leading to abnormally increased myelin permeability that explains the nerve susceptibility to injury. This finding should motivate investigators to identify additional genetic factors contributing to nerve vulnerability of injury. PMID:25374586

  14. Genetic factors for nerve susceptibility to injuries - lessons from PMP22 deficiency.

    PubMed

    Li, Jun

    2014-09-15

    Genetic factors may be learnt from families with gene mutations that render nerve-injury susceptibility even to ordinary physical activities. A typical example is hereditary neuropathy with liability to pressure palsies (HNPP). HNPP is caused by a heterozygous deletion of PMP22 gene. PMP22 deficiency disrupts myelin junctions (such as tight junction and adherens junctions), leading to abnormally increased myelin permeability that explains the nerve susceptibility to injury. This finding should motivate investigators to identify additional genetic factors contributing to nerve vulnerability of injury. PMID:25374586

  15. Mental Imagery Abilities in Adolescents with Spastic Diplegic Cerebral Palsy

    ERIC Educational Resources Information Center

    Courbois, Yanick; Coello, Yann; Bouchart, Isabelle

    2004-01-01

    Four visual imagery tasks were presented to three groups of adolescents with or without spastic diplegic cerebral palsy. The first group was composed of six adolescents with cerebral palsy who had associated visual-perceptual deficits (CP-PD), the second group was composed of five adolescents with cerebral palsy and no associated visual-perceptual…

  16. Pathophysiological mechanisms in cerebral palsy.

    PubMed Central

    Milner-Brown, H S; Penn, R D

    1979-01-01

    To investigate some of the pathophysiological mechanisms in cerebral palsy, surface electromyograms (EMG) were recorded from pairs of flexor/extensor muscles during both voluntary and passive flexion/extension of upper and lower limbs of 20 patients. Elbow, knee, or ankle joint angles were measured simultaneously, as well as the force required to flex/extend the limbs passively at frequencies of 0.1--1.0 Hz. In addition, single motor units were recorded from the first dorsal interosseous muscles of six of the patients. Almost all patients showed resistance to passive movements (hypertonia). This hypertonia did not necessarily impair voluntary flexion/extension movements if alternating EMG activity was maintained in at least one of the pairs of flexor/extensor muscles involved in the movement. In six severly involved patients, there was a complete breakdown in the reciprocal relationship between reciprocally acting pairs of flexor/extensor motoneurones, which resulted in synchronous activation (co-contractions) of flexor/extensor muscles during both voluntary and passive movements. In these patients the hyperactive segmental reflex added to the disabling effects of co-contractions during voluntary movements. Single motor units recorded from patients with dystonic movements were recruited with variable delays (2--10 s) and usually discharged intermittently at high frequencies (60--120/s). This abnormla motor unit discharge pattern may relate to pathology of the basal ganglia. Images PMID:479900

  17. Bone Density in Cerebral Palsy

    PubMed Central

    Houlihan, Christine Murray; Stevenson, Richard D.

    2010-01-01

    Osteoporosis is a skeletal disorder characterized by compromised bone strength predisposing a person to an increased risk of fracture.1 Osteoporosis remains a major health problem worldwide, costing an estimated $13.8 billion in health care each year in the United States. Despite advances in treating osteoporosis in the elderly, no cure exists. Osteoporosis has its roots in childhood. Accrual of bone mass occurs throughout childhood and early adulthood, and peak bone mass is a key determinant of the lifetime risk of osteoporosis. Because the foundation for skeletal health is established so early in life, osteoporosis prevention begins by optimizing gains in bone mineral throughout childhood and adolescence.2,3 Osteoporosis evaluation and prevention is relevant to children with cerebral palsy (CP). CP is the most prevalent childhood condition associated with osteoporosis. Bone density is significantly decreased, and children with CP often sustain painful fractures with minimal trauma that impair their function and quality of life. Preventing or improving osteoporosis and maximizing bone accrual during critical stages of growth will minimize the future lifelong risks of fractures in children with CP. This article addresses the anatomy and structure of bone and bone metabolism, the clinical assessment of bone mass, the causes of osteoporosis and its evaluation and treatment in children with CP. PMID:19643349

  18. [Orthopedic surgery in children with cerebral palsy].

    PubMed

    Smetana, V; Schejbalová, A

    1993-11-01

    The authors present their experience with orthopaedic surgical treatment of children with cerebral palsy. The paper is based on a group of 630 patients where they performed 1882 operations of the lower extremities in 1970-1992. The authors evaluate the long-term results in regularly checked 361 patients (205 boys and 156 girls), mostly with the spastic form of cerebral palsy, with regard to different types of affections which markedly influence the prognosis of surgery (monoparesis, hemiparesis, diparesis, triparesis, quadruparesis). The authors emphasize the primary importance of rehabilitation and team work where the orthopaedist's work is guided by efforts to make further rehabilitation training possible and to facilitate it. They consider surgery an integral part of comprehensive treatment of cerebral palsy which must be followed immediately by further rehabilitation. The authors emphasize the part played be the orthopaedic surgeon to prevent the genesis and development of deformities of the locomotor apparatus. PMID:8299197

  19. Cerebral palsy: A reconceptualization of the spectrum.

    PubMed

    Shapiro, Bruce K

    2004-08-01

    Approximately 50 years ago, interest in cerebral palsy increased, and the current definitions and classification were developed. The interplay among the dimensions of significant impairment, nonprogressive lesions, and persistence defines a group of children who were of interest to the researchers who developed the definition. Cerebral palsy as a definition does not attend to the broader issues of neurodevelopmental dysfunction. It isolates a portion of the spectrum of motor dysfunction and creates a category whose bounds are defined by a range of motor capability. The classifications of cerebral palsy that require revision are discussed. Some classifications should be discarded. Others should be brought in line with current knowledge and approaches. Still others should be modified to encompass the broader views of function and therapy that reflect the current expectations for persons with disabilities. PMID:15292880

  20. The history of facial palsy and spasm

    PubMed Central

    Sajadi, Mohamad-Reza M.; Tabatabaie, Seyed Mahmoud

    2011-01-01

    Although Sir Charles Bell was the first to provide the anatomic basis for the condition that bears his name, in recent years researchers have shown that other European physicians provided earlier clinical descriptions of peripheral cranial nerve 7 palsy. In this article, we describe the history of facial distortion by Greek, Roman, and Persian physicians, culminating in Razi's detailed description in al-Hawi. Razi distinguished facial muscle spasm from paralysis, distinguished central from peripheral lesions, gave the earliest description of loss of forehead wrinkling, and gave the earliest known description of bilateral facial palsy. In doing so, he accurately described the clinical hallmarks of a condition that we recognize as Bell palsy. PMID:21747074

  1. Extensive dural sinus thrombosis and bilateral lateral rectus palsy as an uncommon complication of chronic suppurative otitis media

    PubMed Central

    Balasubramanian, Anusha; Mohamad, Irfan; Sidek, Dinsuhaimi

    2013-01-01

    Dural venous sinus thrombosis, especially of the sigmoid sinus, is a known but uncommon intracranial extradural complication of chronic suppurative otitis media. Even rarer is the simultaneous occurrence of bilateral abducens palsy in the same patient. We report the case of an adolescent male who presented with signs of raised intracranial pressure, diplopia and bilateral lateral rectus palsy associated with a history of left ear discharge and neck swelling. Extensive dural sinus thrombosis extending right up to the left internal jugular vein was confirmed on CT imaging. The patient was successfully treated with thrombolytic agents and antibiotic therapy. The pathophysiology of the concurrent complications is discussed. PMID:23355565

  2. Bilateral Abducens Nerve Palsy due to Idiopathic Intracranial Hypertension as an Initial Manifestation of Systemic Lupus Erythematosus.

    PubMed

    Katsuyama, Eri; Sada, Ken-Ei; Tatebe, Noriko; Watanabe, Haruki; Katsuyama, Takayuki; Narazaki, Mariko; Sugiyama, Koichi; Watanabe, Katsue S; Wakabayashi, Hiroshi; Kawabata, Tomoko; Wada, Jun; Makino, Hirofumi

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure and presents as an intractable headache, vomiting, and ophthalmologic manifestations. We herein report the case of a young girl who presented with bilateral abducens nerve palsy due to IIH as the onset of systemic lupus erythematosus (SLE). The patient was successfully treated with corticosteroid therapy. Our case lacked the typical symptoms of IIH, such as headache or nausea; therefore, it is necessary to carefully determine the cause of bilateral abducens nerve palsies. The development of IIH in SLE patients is a rare occurrence, but this manifestation should not be overlooked. PMID:27086818

  3. Pretend Play of Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Pfeifer, Luzia Iara; Pacciulio, Amanda Mota; dos Santos, Camila Abrao; dos Santos, Jair Licio; Stagnitti, Karen Ellen

    2011-01-01

    Background and Purpose: Evaluate self-initiated pretend play of children with cerebral palsy. Method: Twenty preschool children participated in the study. Pretend play ability was measured by using the child-initiated pretend play assessment culturally adapted to Brazil. Results: There were significant negative correlations between the children's…

  4. Complementary and Alternative Therapies for Cerebral Palsy

    ERIC Educational Resources Information Center

    Liptak, Gregory S.

    2005-01-01

    The optimal practice of medicine includes integrating individual clinical expertise with the best available clinical evidence from systematic research. This article reviews nine treatment modalities used for children who have cerebral palsy (CP), including hyperbaric oxygen, the Adeli Suit, patterning, electrical stimulation, conductive education,

  5. Complementary and Alternative Therapies for Cerebral Palsy

    ERIC Educational Resources Information Center

    Liptak, Gregory S.

    2005-01-01

    The optimal practice of medicine includes integrating individual clinical expertise with the best available clinical evidence from systematic research. This article reviews nine treatment modalities used for children who have cerebral palsy (CP), including hyperbaric oxygen, the Adeli Suit, patterning, electrical stimulation, conductive education,…

  6. Narrative Ability in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Holck, Pernille; Sandberg, Annika Dahlgren; Nettelbladt, Ulrika

    2011-01-01

    In a previous study a group of children with cerebral palsy (CP) were found to have considerable difficulties with narratives, performing several standard deviations below the criteria for the Information score of the Bus Story Test (BST). To examine in depth the performance of children with CP and a control group with typically developing (TD)

  7. Narrative Ability in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Holck, Pernille; Sandberg, Annika Dahlgren; Nettelbladt, Ulrika

    2011-01-01

    In a previous study a group of children with cerebral palsy (CP) were found to have considerable difficulties with narratives, performing several standard deviations below the criteria for the Information score of the Bus Story Test (BST). To examine in depth the performance of children with CP and a control group with typically developing (TD)…

  8. Gait Stability in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Bruijn, Sjoerd M.; Millard, Matthew; van Gestel, Leen; Meyns, Pieter; Jonkers, Ilse; Desloovere, Kaat

    2013-01-01

    Children with unilateral Cerebral Palsy (CP) have several gait impairments, amongst which impaired gait stability may be one. We tested whether a newly developed stability measure (the foot placement estimator, FPE) which does not require long data series, can be used to asses gait stability in typically developing (TD) children as well as…

  9. Gait Stability in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Bruijn, Sjoerd M.; Millard, Matthew; van Gestel, Leen; Meyns, Pieter; Jonkers, Ilse; Desloovere, Kaat

    2013-01-01

    Children with unilateral Cerebral Palsy (CP) have several gait impairments, amongst which impaired gait stability may be one. We tested whether a newly developed stability measure (the foot placement estimator, FPE) which does not require long data series, can be used to asses gait stability in typically developing (TD) children as well as

  10. Cerebral Palsy: Exceptional Child Bibliography Series.

    ERIC Educational Resources Information Center

    Council for Exceptional Children, Reston, VA. Information Center on Exceptional Children.

    Eighty-one references selected from Exceptional Child Education Abstracts are included in the annotated bibliography on cerebral palsy, one in a series of over 50 similar listings dealing with handicapped and gifted children. For each listing, bibliographic and availability information, indexing and retrieval descriptors, and an abstract are…

  11. Cerebral Palsy--A Continuing Battle.

    ERIC Educational Resources Information Center

    Gullerud, Ruth A.

    1979-01-01

    A review of the changes in the treatment of and attitudes toward cerebral palsy in the last 30 years is presented. The author stresses the need for early diagnosis and evaluation, day care (especially respite care), counseling, transportation, special living arrangements, and integration of this population. (PHR)

  12. CEREBRAL PALSY, ITS INDIVIDUAL AND COMMUNITY PROBLEMS.

    ERIC Educational Resources Information Center

    CRUICKSHANK, WILLIAM M.; AND OTHERS

    IN THIS REVISED EDITION, ILLUSTRATED WITH 98 FIGURES AND TABLES, SOME ORIGINAL CHAPTERS HAVE BEEN EXPANDED, AND NEW CHAPTERS HAVE BEEN ADDED. CONTRIBUTING AUTHORS ARE LISTED, AND INCLUDE EDUCATORS, CLINICAL WORKERS, AND ADMINISTRATORS IN THE FIELD OF CEREBRAL PALSY. REFERENCES AND NOTES CONCLUDE EACH CHAPTER, AND SEVERAL CHAPTERS HAVE SECTIONS ON…

  13. The use of nerve conduction studies in determining the short-term outcome of Bell's palsy.

    PubMed

    Prakash, K M; Raymond, A A

    2003-03-01

    Bell's palsy is a common neurological problem causing considerable loss of self-esteem among patients. A prospective observational study was conducted to determine the short-term outcome of Bell's palsy at 1 month and 2 months after the onset and the relationship between these outcomes with facial nerve degeneration. We also determined if gender, age, diabetes, systolic and diastolic blood pressure influence the severity of facial nerve degeneration and the clinical outcome at 2 months after the onset. After clinically grading the newly diagnosed unilateral Bell's palsy patients using the House-Brackmann facial nerve grading system, nerve conduction studies of the facial nerve were done to determine the severity of facial nerve degeneration. The recovery of the facial paralysis was clinically graded again at the end of 1 month and 2 months from the onset. A total of 37 patients were recruited. There was a strong positive correlation between facial nerve degeneration and the clinical outcome of Bell's palsy at 1 month (r = 0.794; p < 0.0005) and 2 months (r = 0.732; p < 0.0005) after the onset. There was no significant correlation between either the facial nerve degeneration or the clinical outcome at 2 months with the patients' age (p = 0.288 and p = 0.799 respectively), systolic blood pressure (p = 0.425 and p = 0.933 respectively) or diastolic blood pressure (p = 0.243 and p = 0.579 respectively). Neither the severity of facial nerve degeneration nor the clinical outcome at 2 months were significantly different between male and female patients (p = 0.460 and p = 0.725 respectively) or diabetic and non-diabetic patients (p = 0.655 and p = 0.655 respectively). PMID:14556328

  14. Arachnoid cyst masquerading as obstetric brachial plexus palsy.

    PubMed

    Muthukumar, Natarajan; Santhanakrishnan, Alwar Govindan; Sivakumar, Krishnaswamy

    2012-07-01

    Obstetric brachial plexus palsy is not uncommon. However, lesions masquerading as obstetric brachial plexus palsy are rare. A child with a cervicothoracic arachnoid cyst masquerading as obstetric brachial plexus palsy is presented, and the relevant literature is reviewed. A girl born by vaginal delivery at full term without any antecedent risk factors for obstetric brachial plexus palsy was noted to have decreased movements of the right upper extremity. After 7 months, there was no improvement. An MRI scan was obtained, which revealed a cervicothoracic spinal extradural arachnoid cyst. During surgery, the cyst was found to communicate with the dura at the axilla of the C-7 nerve root. The cyst was excised in toto. Six months later, there was improvement in the infant's neurological status. This case illustrates that spinal arachnoid cysts should be entertained in the differential diagnosis when a child presents with obstetric brachial plexus palsy without known antecedent risk factors for obstetric palsy. PMID:22681321

  15. Adrenomyeloneuropathy with bulbar palsy: A rare association.

    PubMed

    Chafale, Vishal Annaji; Lahoti, Satish Arunkumar; Biswas, Atanu; Roy, Arijit; Senapati, Asit Kumar

    2014-07-01

    Adrenomyeloneuropathy (AMN) is a variant of adrenoleukodystrophy (ALD), an X-linked recessive peroxisomal disorder associated with accumulation of very long chain fatty acids (VLCFA). Mutations of this gene lead to abnormal peroxisomal β-oxidation, which results in the harmful accumulation of VLCFAs in affected cells. Neurological symptoms occur due to progressive demyelination and destruction of cerebral white matter and primary adrenal insufficiency. Bulbar palsy in a case of AMN is very unusual. We report a case of a 22-year-old male with AMN who developed adrenal insufficiency at the age of 4 years successfully treated by gluco- and mineralocorticoids followed by features of myeloneuropathy with bulbar palsy. AMN with prominent bulbar symptoms emphasizes the diverse clinical manifestation of this disease. PMID:25221415

  16. Lagophthalmos after facial palsy: current therapeutic options.

    PubMed

    Vásquez, Luz María; Medel, Ramón

    2014-01-01

    As the facial nerve carries sensory, motor and parasympathetic fibres involved in facial muscle innervation, facial palsy results in functional and cosmetic impairment. It can result from a wide variety of causes like infectious processes, trauma, neoplasms, autoimmune diseases, and most commonly Bell's palsy, but it can also be of iatrogenic origin. The main ophthalmic sequel is lagophthalmos. The increased surface exposure increases the risk of keratitis, corneal ulceration, and potentially loss of vision. Treatment options are wide; some are temporary, some permanent. In addition to gold standard and traditional therapies and procedures, new options are being proposed aiming to improve not only lagophthalmos but also the quality of life of these patients. PMID:25342248

  17. [Cytomegalovirus mononucleosis complicated with peripheral facial palsy].

    PubMed

    Hirano, Taichi; Tsuji, Takahiro; Yamasaki, Hiroshi; Tsuda, Hiroyuki

    2014-03-01

    A 36-year-old woman was admitted to our hospital for further examination of an acute febrile illness with liver dysfunction. A peripheral blood smear displayed atypical lymphocytes. Cytomegalovirus (CMV) mononucleosis was diagnosed based on the detection of CMV-specific IgM and conventional CMV pp65 antigen. The physical examination on admission revealed signs of lower motor neuron right facial palsy. There were no significant cerebrospinal fluid findings, nor were there other neurological abnormalities. After receiving a short-course of oral corticosteroids, the patient gradually recovered from the facial paralysis. A one-month follow-up examination indicated that she had fully recovered neurologically, showing disappearance of CMV-DNA and a significant increase in the anti-CMV IgG titer. To our knowledge, there has been only one previous report describing CMV as the cause of an isolated facial palsy combined with CMV mononucleosis. PMID:24681941

  18. Tendon Transfers Part II: Transfers for Ulnar Nerve Palsy and Median Nerve Palsy

    PubMed Central

    Sammer, Douglas M.; Chung, Kevin C.

    2009-01-01

    Objectives After reading this article (part II of II), the participant should be able to: 1. Describe the anatomy and function of the median and ulnar nerves in the forearm and hand. 2. Describe the clinical deficits associated with injury to each nerve. 3. Describe the indications, benefits, and drawbacks for various tendon transfer procedures used to treat median and ulnar nerve palsy.4. Describe the treatment of combined nerve injuries. 5. Describe postoperative care and possible complications associated with these tendon transfer procedures. Summary This article discusses the use of tendon transfer procedures for treatment of median and ulnar nerve palsy as well as combined nerve palsies. Postoperative management and potential complications are also discussed. PMID:19730287

  19. Facial nerve palsy after mandibular fracture.

    PubMed

    Weinberg, M J; Merx, P; Antonyshyn, O; Farb, R

    1995-05-01

    A 19-year-old man sustained a right parasymphyseal fracture and bilateral condylar neck fractures in a motor vehicle accident. The parasymphyseal fracture was treated by open reduction and internal fixation, and the subcondylar fractures were treated with closed reduction and maxillomandibular fixation. Three days postoperatively, a near-complete left facial nerve palsy developed. Facial nerve recovery was not full. The literature is reviewed, and possible mechanisms of this rare and devastating complication are discussed. PMID:7639495

  20. [Third cranial nerve palsy in sphenoid sinusitis].

    PubMed

    Dores, Luís Almeida; Simão, Marco Alveirinho; Marques, Marta Canas; Dias, Éscar

    2014-01-01

    Sphenoid sinus disease is particular not only for its clinical presentation, as well as their complications. Although rare, these may present as cranial nerve deficits, so it is important to have a high index of suspicion and be familiar with its diagnosis and management. Symptoms are often nonspecific, but the most common are headache, changes in visual acuity and diplopia due to dysfunction of one or more ocular motor nerves. The authors report a case of a 59 years-old male, who was referred to the ENT emergency department with frontal headaches for one week which had progressively worsened and were associated, since the last 12 hours, with diplopia caused by left third cranial nerve palsy. Neurologic examination was normal aside from the left third cranial nerve palsy. Anterior and posterior rhinoscopy excluded the presence of nasal masses and purulent rhinorrhea. The CT scan revealed a soft tissue component and erosion of the roof of the left sphenoid sinus. Patient was admitted for intravenous antibiotics and steroids treatment without any benefit after 48 hours. He was submitted to endoscopic sinus surgery with resolution of the symptoms 10 days after surgery. The authors present this case for its rarity focusing on the importance of differential diagnosis in patients with headaches and cranial nerves palsies. PMID:25641296

  1. Manual muscle test at C5 palsy onset predicts the likelihood of and time to C5 palsy resolution.

    PubMed

    Macki, Mohamed; Alam, Ridwan; Kerezoudis, Panagiotis; Gokaslan, Ziya; Bydon, Ali; Bydon, Mohamad

    2016-02-01

    The primary objective of this study was to identify time to and prognostic factors of C5 palsy resolution. All patients over a 7year period who experienced C5 palsy following a posterior decompression and instrumented fusion surgery were retrospectively reviewed. C5 palsy resolution was defined as a recovery of deltoid muscle function equal to or greater than the preoperative condition as defined by the manual muscle test (MMT). Of the 511 patients who met the selection criteria, 8.6% (n=44) experienced C5 palsy. MMT information was available for 43 patients; 81.4% (n=35) had full resolution from their condition. Of the 35 patients who resolved, the median MMT score at onset was 3-. Following a discrete-time proportional hazards model, the hazards of C5 palsy resolution increased by 19% for every one-grade increase in MMT score at symptom onset (hazard ratio [HR]=1.19, p=0.005). Moreover, males displayed a 71% lower hazard of resolution than females (HR=0.29, p=0.003). Following an adjusted Kaplan-Meier analysis, the median time to C5 palsy resolution was between 6months and 1year. In a multiple linear regression, a lower MMT score at the onset of C5 palsy predicted a longer time to C5 palsy resolution (coefficient=-0.19, p=0.003). Time to C5 palsy onset was not statistically associated with hazards of palsy resolution (p=0.381) or time to resolution (p=0.121). A higher MMT score at the onset of C5 palsy statistically significantly predicted a higher chance of resolution and a shorter recovery time. Female sex was also associated with a higher hazard of resolution. PMID:26602799

  2. Chemo-port insertion: A cause of vocal cord palsy.

    PubMed

    Alazzawi, Sarmad; Hindi, Khalid; Malik, Ausama; Wee, Chong Aun; Prepageran, Narayanan

    2015-11-01

    We describe extremely rare cases of vocal cord palsy following surgical insertion of a chemo port. Our cohort consisted of patients with cancer who developed hoarseness immediately after central venous line placement for the administration of chemotherapy, with vocal cord palsy confirmed with flexible laryngoscopy. Given the timing, central venous line placement appears to be the most likely cause. PMID:26108861

  3. The Child With Cerebral Palsy in the Regular Class.

    ERIC Educational Resources Information Center

    Aiello, Barbara

    This handbook was developed to help the teacher deal successfully with physical problems involved in having a child with cerebral palsy mainstreamed into the regular classroom. Characteristics of cerebral palsy are described, and it is pointed out that most children with this affliction have average or above average intelligence. Classroom…

  4. Quality of Arithmetic Education for Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Jenks, Kathleen M.; de Moor, Jan; van Lieshout, Ernest C. D. M.; Withagen, Floortje

    2010-01-01

    The aim of this exploratory study was to investigate the quality of arithmetic education for children with cerebral palsy. The use of individual educational plans, amount of arithmetic instruction time, arithmetic instructional grouping, and type of arithmetic teaching method were explored in three groups: children with cerebral palsy (CP) in

  5. The School Experience for Gifted Students with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Willard-Holt, Colleen

    This paper presents a study of two intellectually gifted students (ages 6 and 14) who have cerebral palsy and are unable to communicate orally. Results of participant observation, interviews, and document analysis revealed that: (1) gifted students who have cerebral palsy and do not speak exhibit indicators of cognitive ability that are similar to…

  6. Quality of Arithmetic Education for Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Jenks, Kathleen M.; de Moor, Jan; van Lieshout, Ernest C. D. M.; Withagen, Floortje

    2010-01-01

    The aim of this exploratory study was to investigate the quality of arithmetic education for children with cerebral palsy. The use of individual educational plans, amount of arithmetic instruction time, arithmetic instructional grouping, and type of arithmetic teaching method were explored in three groups: children with cerebral palsy (CP) in…

  7. Erb-Duchenne and Dejerine-Klumpke Palsies

    MedlinePlus

    ... About NINDS NINDS Erb-Duchenne and Dejerine-Klumpke Palsies Information Page Synonym(s): Brachial Plexus Birth Injuries, Dejerine- ... Español What are Erb-Duchenne and Dejerine-Klumpke Palsies? The brachial plexus is a network of nerves ...

  8. Do oral steroids aid recovery in children with Bell's palsy?

    PubMed

    Ismail, Abdul Qader; Alake, Oluwaseyi; Kallappa, Chetana

    2014-10-01

    There is growing evidence that steroids are not beneficial for treatment of paediatric patients with Bell's palsy. To investigate, we conducted a retrospective longitudinal study examining notes of 100 children, over 12 years coded for facial nerve palsy. Of the 79 diagnosed with Bell's palsy, all recovered, and for 46 patients we had data on interval from onset of symptoms to resolution (median duration in treated group = 5 weeks, range = 39; median duration in untreated group = 6 weeks, range = 11; P = .86). From our results, we conclude that all children with Bell's palsy recovered, with or without steroid treatment, with no statistically significant difference in symptoms duration. Complications of unresolved Bell's palsy can have important long-term functional and psychosocial consequences. Therefore, we need further research on use of steroids in children with complete/severe cases; it would be a shame to omit treatment due to "absence of evidence" rather than "evidence of absence." PMID:24141272

  9. Peripheral myelin protein 22 gene duplication with atypical presentations: a new example of the wide spectrum of Charcot-Marie-Tooth 1A disease.

    PubMed

    Mathis, Stéphane; Corcia, Philippe; Tazir, Meriem; Camu, William; Magdelaine, Corinne; Latour, Philippe; Biberon, Julien; Guennoc, Anne-Marie; Richard, Laurence; Magy, Laurent; Funalot, Benoît; Vallat, Jean-Michel

    2014-06-01

    Charcot-Marie-Tooth type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are both autosomal-dominant disorders linked to peripheral myelin anomalies. CMT1A is associated with a Peripheral Myelin Protein 22 (PMP22) duplication, whereas HNPP is due to a PMP22 deletion on chromosome 17. In spite of this crucial difference, we report three observations of patients with the 1.4 megabase CMT1A duplication and atypical presentation (electrophysiological, clinical or pathological): a 10 year-old girl with tomaculous lesions on nerve biopsy; a 26 year-old woman with recurrent paresthesiae and block conduction on the electrophysiological study; a 46 year-old woman with transient recurrent nerve palsies mimicking HNPP. These observations highlight the wide spectrum of CMT1A and the overlap between CMT1A and HNPP (both linked to the PMP22 gene), and finally illustrate the complexity of the genotype-phenotype correlations in Charcot-Marie-Tooth diseases. PMID:24792522

  10. The Effect of Long-Term Training Program on Balance in Children with Cerebral Palsy: Results of a Pilot Study for Individually Based Functional Exercises

    ERIC Educational Resources Information Center

    Uzun, Selda

    2013-01-01

    This study examines the effects of long-term training program on balance and center of pressure (COP) for four male children (13 years of age) with cerebral palsy (CP). These children were classified into one hemiplegic (level II), one diplegic (level II) and two quadriplegic children (levels III and II) using the Gross Motor Function…

  11. The Effect of Long-Term Training Program on Balance in Children with Cerebral Palsy: Results of a Pilot Study for Individually Based Functional Exercises

    ERIC Educational Resources Information Center

    Uzun, Selda

    2013-01-01

    This study examines the effects of long-term training program on balance and center of pressure (COP) for four male children (13 years of age) with cerebral palsy (CP). These children were classified into one hemiplegic (level II), one diplegic (level II) and two quadriplegic children (levels III and II) using the Gross Motor Function

  12. [Advantage of facial rehabilitation after facial palsy].

    PubMed

    Gatignol, Peggy; Lannadre, Elodie; Bernat, Isabelle; Tankr, Frdric; Lamas, Georges

    2011-10-01

    Visible and immediate trauma, facial palsy (FP) covers functional but also psychological damage and it is essential to evaluate before a comprehensive therapeutic care tailored. Few patients, however, are emerging with a prescription for rehabilitation after a consultation. Why? This rehabilitation is it ignored? Is it absolutely necessary? It is evident in the extension of medical care to minimize the effects. Yet the foundation of rehabilitation is sadly little known and often poorly enforced. In addition to its specificity, this therapy preceded by a report called "pretreatments offers a prognosis for recovery to patient" regardless of the origin and degree of involvement of the PF. PMID:22046678

  13. Evidence based management of Bell's palsy.

    PubMed

    McCaul, James A; Cascarini, Luke; Godden, Daryl; Coombes, Darryl; Brennan, Peter A; Kerawala, Cyrus J

    2014-05-01

    Bell's palsy (idiopathic facial paralysis) is caused by the acute onset of lower motor neurone weakness of the facial nerve with no detectable cause. With a lifetime risk of 1 in 60 and an annual incidence of 11-40/100,000 population, the condition resolves completely in around 71% of untreated cases. In the remainder facial nerve function will be impaired in the long term. We summarise current published articles regarding early management strategies to maximise recovery of facial nerve function and minimise long-term sequelae in the condition. PMID:24685475

  14. Improving care for children with cerebral palsy.

    PubMed

    Newey, Colin

    2008-09-01

    A report about the health care of people with learning disability published by a UK charity concluded that this group was discriminated against and that healthcare professionals had a poor understanding of their needs. A case report of a young person with cerebral palsy is used here to demonstrate good practice in the care of children with learning disabilities. The careful development over time of individualised solutions makes a difference to the quality of life for children and families. Improved understanding through education as well as collaborative working and family participation will help ensure that children and young people receive the range of services they require. PMID:18808052

  15. Isolated third nerve palsy: A rare presentation of high grade glioma.

    PubMed

    Singh, Deepak Kumar; Singh, Neha; Singh, Ragini

    2016-01-01

    High grade gliomas account for almost one-third of primary central nervous system neoplasm, mainly in adults with a mean age of 41 years. They usually present with symptoms of raised intracranial pressure such as headache, vomiting, and seizures. We report a case of 55-year-old male presenting with right side complete third nerve palsy. Magnetic resonance imaging revealed an intraaxial tumor of the right medial temporal lobe. The tumor was removed grossly, and the histological diagnosis was anaplastic astrocytoma (WHO grade 3). We discuss clinical presentation of this case along with pertinent literature. PMID:27057228

  16. Isolated third nerve palsy: A rare presentation of high grade glioma

    PubMed Central

    Singh, Deepak Kumar; Singh, Neha; Singh, Ragini

    2016-01-01

    High grade gliomas account for almost one-third of primary central nervous system neoplasm, mainly in adults with a mean age of 41 years. They usually present with symptoms of raised intracranial pressure such as headache, vomiting, and seizures. We report a case of 55-year-old male presenting with right side complete third nerve palsy. Magnetic resonance imaging revealed an intraaxial tumor of the right medial temporal lobe. The tumor was removed grossly, and the histological diagnosis was anaplastic astrocytoma (WHO grade 3). We discuss clinical presentation of this case along with pertinent literature. PMID:27057228

  17. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

    PubMed Central

    Sethi, Prahlad K.; Batra, Anuradha; Sethi, Nitin K.; Torgovnick, Josh; Arsura, Edward

    2011-01-01

    Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome. PMID:21655204

  18. Neuralgic Amyotrophy Manifesting as Mimicking Posterior Interosseous Nerve Palsy

    PubMed Central

    Yang, Jin Seo; Kang, Suk Hyung; Choi, Eun Hi

    2015-01-01

    The upper trunk of the brachial plexus is the most common area affected by neuralgic amyotrophy (NA), and paresis of the shoulder girdle muscle is the most prevalent manifestation. Posterior interosseous nerve palsy is a rare presentation in patients with NA. It results in dropped finger on the affected side and may be misdiagnosed as entrapment syndrome or compressive neuropathy. We report an unusual case of NA manifested as PIN palsy and suggest that knowledge of clinical NA phenotypes is crucial for early diagnosis of peripheral nerve palsies. PMID:26713154

  19. Contralateral diaphragmatic palsy in acute stroke: an interesting observation.

    PubMed

    Kumar, Sudhir; Reddy, Rajesh; Prabhakar, Subhashini

    2009-01-01

    Diaphragmatic palsy in hemiparetic stroke is not well recognized. Further, its implications on stroke outcome have not been studied. Here, we report a patient with left-sided diaphragmatic palsy due to an acute right middle cerebral artery territory infarction. The diagnosis was suspected on finding an elevated dome of the diaphragm on the left side in a routine chest radiograph and was confirmed by finding decreased movements of the left hemidiaphragm on fluoroscopic examination. We hypothesize that this condition is probably under-recognized in clinical practice and its clinical importance not well known. The pathophysiological basis of diaphragmatic palsy in acute stroke and its possible clinical implications are discussed. PMID:19881177

  20. Pediatric Cerebral Palsy in Africa: Where Are We?

    PubMed

    Donald, Kirsten A; Kakooza, Angelina M; Wammanda, Robinson D; Mallewa, Macpherson; Samia, Pauline; Babakir, Haydar; Bearden, David; Majnemer, Annette; Fehlings, Darcy; Shevell, Michael; Chugani, Harry; Wilmshurst, Jo M

    2015-07-01

    Cerebral palsy is the most common cause of physical disability in children worldwide. However, little is reported on this condition in the African context. Doctors from 22 countries in Africa, and representatives from a further 5 countries outside Africa, met to discuss the challenges in the evaluation and management of children with cerebral palsy in Africa and to propose service needs and further research. Basic care is limited by the poor availability of diagnostic facilities or medical personnel with experience and expertise in managing cerebral palsy, exacerbated by lack of available interventions such as medications, surgical procedures, or even regular therapy input. Relevant guidelines are lacking. In order to guide services for children with existing disabilities, to effectively target the main etiologies and to develop preventive strategies for the continent, research priorities must include multicenter collaborative studies looking at the prevalence, risk factors, and treatment of cerebral palsy. PMID:25296926

  1. Health-related physical fitness for children with cerebral palsy.

    PubMed

    Maltais, Désirée B; Wiart, Lesley; Fowler, Eileen; Verschuren, Olaf; Damiano, Diane L

    2014-08-01

    Low levels of physical activity are a global health concern for all children. Children with cerebral palsy have even lower physical activity levels than their typically developing peers. Low levels of physical activity, and thus an increased risk for related chronic diseases, are associated with deficits in health-related physical fitness. Recent research has provided therapists with the resources to effectively perform physical fitness testing and physical activity training in clinical settings with children who have cerebral palsy, although most testing and training data to date pertains to those who walk. Nevertheless, on the basis of the present evidence, all children with cerebral palsy should engage, to the extent they are able, in aerobic, anaerobic, and muscle-strengthening activities. Future research is required to determine the best ways to evaluate health-related physical fitness in nonambulatory children with cerebral palsy and foster long-term changes in physical activity behavior in all children with this condition. PMID:24820339

  2. Health-related physical fitness for children with cerebral palsy

    PubMed Central

    Maltais, Désirée B.; Wiart, Lesley; Fowler, Eileen; Verschuren, Olaf; Damiano, Diane L.

    2014-01-01

    Low levels of physical activity are a global health concern for all children. Children with cerebral palsy have even lower physical activity levels than their typically developing peers. Low levels of physical activity, and thus an increased risk for related chronic diseases, are associated with deficits in health-related physical fitness. Recent research has provided therapists with the resources to effectively perform physical fitness testing and physical activity training in clinical settings with children who have cerebral palsy, although most testing and training data to date pertains to those who walk. Nevertheless, based on the present evidence, all children with cerebral palsy should engage, to the extent they are able, in aerobic, anaerobic and muscle strengthening activities. Future research is required to determine the best ways to evaluate health-related physical fitness in non-ambulatory children with cerebral palsy and foster long-term changes in physical activity behavior in all children with this condition. PMID:24820339

  3. Facial nerve palsy, Kawasaki disease, and coronary artery aneurysm.

    PubMed

    Stowe, Robert C

    2015-09-01

    Kawasaki disease is rarely complicated by cranial nerve VII palsy. This report describes a 15-month-old female presenting with 3 days of fever, irritability, and rash who was subsequently diagnosed with Kawasaki disease and treated with intravenous immunoglobulin. She was found to have mild coronary artery ectasia and developed an acute, transient, left-sided facial palsy on the sixth day of illness. Repeat echocardiography demonstrated worsening aneurysm and intravenous methylprednisolone was added to her treatment regimen. At 1 and 3 months post-discharge, echocardiography demonstrated resolution of her coronary aneurysm. This case makes 41 total described in the literature. Patients tend to be under 12-months-old and there is a higher association with coronary artery aneurysm in such patients compared to those without facial palsy who never even received treatment. Kawasaki disease associated with facial palsy may indicate increased inflammatory burden and patients may require additional anti-inflammatory agents and more vigilant echocardiography. PMID:26101056

  4. New Criteria of Adjustment for the Cerebral Palsied

    ERIC Educational Resources Information Center

    Floor, Lucretia; Rosen, Marvin

    1976-01-01

    Examined were the effects on selected adjustment criterion measures of 1 year of maximum, moderate, or minimum rehabilitation treatment with 113 cerebral palsied adults (mean age =28) having a wide range of physical and intellectual abilities. (IM)

  5. Lame from birth: early concepts of cerebral palsy.

    PubMed

    Obladen, Michael

    2011-02-01

    Deformations have been attributed to supernatural causes since antiquity. Cerebral palsy was associated with God's wrath, witchcraft, the evil eye, or maternal imagination. Greek scholars recommended prevention by tight swaddling, a custom that persisted into modern times. In the Middle Ages, the midwife's negligence was held responsible as was difficult teething. Morgagni described in 1769 that the neonatal brain can liquefy, and Bednar described leukomalacia in 1850 as a distinct disorder of the newborn. In 1861, Little associated cerebral palsies with difficult or protracted labor and neonatal asphyxia, but he was challenged by Freud, who in 1897 declared that most cases are prenatal in origin. In 1868, Virchow demonstrated inflammatory changes, a view recently confirmed by Leviton and Nelson. Although a causal relationship of cerebral palsy to the birth never has been established, the habit to put the blame for cerebral palsy on someone remained a frequent attitude. PMID:21193777

  6. Stability and Harmony of Gait in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Iosa, Marco; Marro, Tiziana; Paolucci, Stefano; Morelli, Daniela

    2012-01-01

    The aim of this study was to quantitatively assess the stability and harmony of gait in children with cerebral palsy. Seventeen children with spastic hemiplegia due to cerebral palsy (5.0 [plus or minus] 2.3 years old) who were able to walk autonomously and seventeen age-matched children with typical development (5.7 [plus or minus] 2.5 years old,…

  7. Stability and Harmony of Gait in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Iosa, Marco; Marro, Tiziana; Paolucci, Stefano; Morelli, Daniela

    2012-01-01

    The aim of this study was to quantitatively assess the stability and harmony of gait in children with cerebral palsy. Seventeen children with spastic hemiplegia due to cerebral palsy (5.0 [plus or minus] 2.3 years old) who were able to walk autonomously and seventeen age-matched children with typical development (5.7 [plus or minus] 2.5 years old,

  8. Management of spasticity in children with cerebral palsy.

    PubMed

    Shamsoddini, Alireza; Amirsalari, Susan; Hollisaz, Mohammad-Taghi; Rahimnia, Alireza; Khatibi-Aghda, Amideddin

    2014-08-01

    Cerebral palsy is the most common cause of spasticity and physical disability in children and spasticity is one of the commonest problems in those with neurological disease. The management of spasticity in children with cerebral palsy requires a multidisciplinary effort and should be started as early as possible. There are a number of treatments available for the management of spasticity. This article reviews the variety of options available for the clinical management of spasticity. PMID:25755853

  9. Delayed Facial Nerve Palsy after Seat Belt Induced Injury.

    PubMed

    Osuagwu, Ferdnand C; Bradley, Ronald; Pasupuleti, Devakinanda; Bobeda, Erik

    2015-12-01

    Blunt trauma causing facial nerve palsy and facial nerve trauma due to seat belt injury is rarely reported. We describe a 63 year old female who was involved in a road traffic accident without any imaging evidence of temporal bone fracture developing left facial nerve palsy on the second day. We discuss the pathophysiology of this condition and the need to be aware of this unusual complication when evaluating patient with blunt trauma to the face. PMID:26662661

  10. Clinical Approach to Progressive Supranuclear Palsy

    PubMed Central

    Ling, Helen

    2016-01-01

    Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson’s syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients’ complex needs is the current core treatment strategy for this devastating disorder. PMID:26828211

  11. Complementary and alternative therapies for cerebral palsy.

    PubMed

    Liptak, Gregory S

    2005-01-01

    The optimal practice of medicine includes integrating individual clinical expertise with the best available clinical evidence from systematic research. This article reviews nine treatment modalities used for children who have cerebral palsy (CP), including hyperbaric oxygen, the Adeli Suit, patterning, electrical stimulation, conductive education, equine-assisted therapy, craniosacral therapy, Feldenkrais therapy, and acupuncture. Unfortunately, these modalities have different degrees of published evidence to support or refute their effectiveness. Uncontrolled and controlled trials of hippotherapy have shown beneficial effects on body structures and functioning. Studies of acupuncture are promising, but more studies are required before specific recommendations can be made. Most studies of patterning have been negative and its use cannot be recommended. However, for the other interventions, such as hyperbaric oxygen, more evidence is required before recommendations can be made. The individual with CP and his or her family have a right to full disclosure of all possible treatment options and whatever knowledge currently is available regarding these therapies. PMID:15977320

  12. Cerebral palsy: the whys and hows.

    PubMed

    Fairhurst, Charlie

    2012-08-01

    The descriptive term of cerebral palsy encompasses the largest group of childhood movement disorders. Severity and pattern of clinical involvement varies widely dependent on the area of the central nervous system compromised. A multidisciplinary team approach is vital for all the aspects of management to improve function and minimise disability. From a medical viewpoint, there are two pronged approaches. First a focus on developmental and clinical comorbidities such as communication, behaviour, epilepsy, feeding problems, gastro-oesophageal reflux and infections; and second on specifics of muscle tone, motor control and posture. With regards to the latter, there is an increasing number of available treatments including oral antispasticity and antidystonic medications, injectable botulinum toxin, multilevel orthopaedic and neurosurgical options and a variety of complementary and alternative therapies. PMID:22868578

  13. Early identification and intervention in cerebral palsy.

    PubMed

    Herskind, Anna; Greisen, Gorm; Nielsen, Jens Bo

    2015-01-01

    Infants with possible cerebral palsy (CP) are commonly assumed to benefit from early diagnosis and early intervention, but substantial evidence for this is lacking. There is no consensus in the literature on a definition of 'early', but this review focuses on interventions initiated within the first 6 months after term age. We cover basic neuroscience, arguing for a beneficial effect of early intervention, and discuss why clinical research to support this convincingly is lacking. We argue that infants offered early intervention in future clinical studies must be identified carefully, and that the intervention should be focused on infants showing early signs of CP to determine an effect of treatment. Such signs may be efficiently detected by a combination of neuroimaging and the General Movements Assessment. We propose a research agenda directed at large-scale identification of infants showing early signs of CP and testing of high-intensity, early interventions. PMID:25041565

  14. Cerebral palsy after maternal trauma in pregnancy.

    PubMed

    Hayes, B; Ryan, S; Stephenson, J B P; King, M D

    2007-09-01

    Ten children (six males, four females) with spastic (n=9) and mixed spastic-dyskinetic (n=1) cerebral palsy were born at term to mothers who earlier in the pregnancy had been involved in accidents without suffering overt abdominal injury, placental abruption, or premature onset of labour. At follow-up (at ages 2-24y), Gross Motor Function Classification System levels were II (n=7) and V (n=3). Cognitive level was normal in five patients, while learning disability was mild to moderate in two and severe in three. Magnetic resonance imaging of the brain in all children, assessed blind to the dates of maternal trauma in pregnancy, showed lesions consistent with prenatal vascular insult at the time of the trauma. Feasible mechanisms of brain injury include reduced placental blood flow and/or placental embolization. PMID:17718828

  15. Bilateral Facial Nerve Palsy: A Diagnostic Dilemma

    PubMed Central

    Pothiawala, Sohil; Lateef, Fatimah

    2012-01-01

    Introduction. Bilateral facial nerve palsy (FNP) is a rare condition, representing less than 2% of all cases of FNP. Majority of these patients have underlying medical conditions, ranging from neurologic, infectious, neoplastic, traumatic, or metabolic disorders. Objective. The differential diagnosis of its causes is extensive and hence can present as a diagnostic challenge. Emergency physicians should be aware of these various diagnostic possibilities, some of which are potentially fatal. Case Report. We report a case of a 43-year-old female who presented to the emergency department with sequential bilateral facial nerve paralysis which could not be attributed to any particular etiology and, hence, presented a diagnostic dilemma. Conclusion. We reinforce the importance of considering the range of differential diagnosis in all cases presenting with bilateral FNP. These patients warrant admission and prompt laboratory and radiological investigation for evaluation of the underlying cause and specific further management as relevant. PMID:23326715

  16. Prognostic Factors for Postsurgical Recovery of Deltoid Palsy due to Cervical Disc Herniations

    PubMed Central

    Chung, Jae-Yoon; Chang, Han; Song, Kyung-Jin; Kim, Jin-Hyok; Hong, Chang-Hwa; Lee, Jung Sub; Lee, Sang-Hun; Song, Kwang-Sup; Yang, Jae Jun; Uh, Jae-Hyung; Kim, Young-Tae; Lee, Jae Min

    2015-01-01

    Study Design Retrospective multicenter study. Purpose We aimed to investigate prognostic factors affecting postsurgical recovery of deltoid palsy due to cervical disc herniation (CDH). Overview of Literature Little information is available about prognostic factors affecting postsurgical recovery of deltoid palsy due to CDH. Methods Sixty-one patients with CDH causing deltoid palsy (less than grade 3) were included in this study: 35 soft discs and 26 hard discs. Average duration of preoperative deltoid palsy was 11.9 weeks. Thirty-two patients underwent single-level surgery, 22 two-level, four three-level, and three four-level. Patients with accompanying myelopathy, shoulder diseases, or peripheral neuropathy were excluded from the study. Results Deltoid palsy (2.4 grades vs. 4.5 grades, p<0.001) and radiculopathy (6.4 points vs. 2.1 points, p<0.001) significantly improved after surgery. Thirty-six of 61 patients (59%) achieved full recovery (grade 5) of deltoid palsy, with an average time of 8.4 weeks. Longer duration of preoperative deltoid palsy and more severe radiculopathy negatively affected the degree of improvement in deltoid palsy. Age, gender, number of surgery level, and disc type did not affect the degree of improvement of deltoid palsy. Contrary to our expectations, severity of preoperative deltoid palsy did not affect the degree of improvement. Due to the shorter duration of preoperative deltoid palsy, in the context of rapid referral, early surgical decompression resulted in significant recovery of more severe grades (grade 0 or 1) of deltoid palsy compared to grade 2 or 3 deltoid palsy. Conclusions Early surgical decompression significantly improved deltoid palsy caused by CDH, irrespective of age, gender, number of surgery level, and disc type. However, longer duration of deltoid palsy and more severe intensity of preoperative radiating pain were associated with less improvement of deltoid palsy postoperatively. PMID:26435786

  17. [Accuracy of the prognostic diagnosis in acute peripheral facial palsy].

    PubMed

    Aoyagi, Masaru

    2005-01-01

    The important factors in the prognostic diagnosis of acute peripheral facial palsy are (1) the causal disease, (2) the site of injury and (3) the degree of injury, although the age of the patient, complication, treatment method and initial day of treatment are also important. Among these 3 factors, the degree of injury is most strongly related to the prognosis. However, the diagnosis of etiology is the most important for the selection of the treatment method. Above all, the differential diagnosis between Bell's palsy and zoster sine herpete (Ramsay Hunt syndrome), is the most significant. However, it is impossible to diagnose all patients with complete accuracy within 3 days after the onset of palsy, even using molecular biological examination including polymerase chain reaction analysis. The diagnosis of the site of injury does not contribute to the prediction of prognosis or the selection of treatment method, except for the determination of the approaching route of the facial nerve decompression for traumatic facial palsy. The scoring system of facial movement (40-point method), nerve excitability test (NET), electroneurography (ENoG), transcranial magnetic stimulation (TMS) and stapedial reflex (SR) are commonly used to estimate the degree of injury. To estimate the accuracy of these examinations, sensitivity and specificity of the tests were calculated according to the findings within 3 days after the onset of palsy and the outcome of 116 patients with Bell's palsy and 31 with Ramsay Hunt syndrome. According to the results, none of these tests seem to be a perfect diagnostic examination for the completely precise prediction of prognosis. However, a patient is predicted to have a good prognosis, if the following 3 findings are observed: (1) more than 10 points in the 40-point scoring system of facial movement, (2) a positive response to TMS and (3) a positive response to SR. An antidromic facial nerve response probably contributes to a precise prediction of prognosis within 3 days after the onset of facial palsy. PMID:15712490

  18. Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia.

    PubMed

    Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda, Naoya; Oshima, Etsuko; Sakane, Katsuaki; Terada, Seishi; Ihara, Yuetsu; Uchitomi, Yosuke

    2012-12-15

    We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis. PMID:23026537

  19. Cerebral Palsy. Fact Sheet = La Paralisis Cerebral. Hojas Informativas Sobre Discapacidades.

    ERIC Educational Resources Information Center

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This fact sheet on cerebral palsy is written in both English and Spanish. First, it provides a definition of cerebral palsy and considers various causes (e.g., an insufficient amount of oxygen reaching the fetal or newborn brain). The fact sheet then offers incidence figures and explains characteristics of the three main types of cerebral palsy:…

  20. Gait training facilitates central drive to ankle dorsiflexors in children with cerebral palsy

    PubMed Central

    Petersen, Tue Hvass; Farmer, Simon Francis; Nielsen, Jens Bo

    2015-01-01

    Foot drop and toe walking are frequent concerns in children with cerebral palsy. The main underlying cause of these problems is early damage and lack of maturation of the corticospinal tract. In the present study we investigated whether 4 weeks of daily treadmill training with an incline may facilitate corticospinal transmission and improve the control of the ankle joint in children with cerebral palsy. Sixteen children with cerebral palsy (Gross Motor Classification System I:6, II:6, III:4) aged 5–14 years old, were recruited for the study. Evaluation of gait ability and intramuscular coherence was made twice before and twice after training with an interval of 1 month. Gait kinematics were recorded by 3D video analysis during treadmill walking with a velocity chosen by the child at the first evaluation. Foot pressure was measured by force sensitive foot soles during treadmill and over ground walking. EMG-EMG coherence was calculated from two separate electrode recordings placed over the tibialis anterior muscle. Training involved 30 min of walking daily on a treadmill with an incline for 30 days. Gait training was accompanied by significant increases in gait speed, incline on the treadmill, the maximal voluntary dorsiflexion torque, the number and amplitude of toe lifts late in the swing phase during gait and the weight exerted on the heel during the early stance phase of the gait cycle. EMG-EMG coherence in the beta and gamma frequency bands recorded from tibialis anterior muscle increased significantly when compared to coherence before training. The largest changes in coherence with training were observed for children <10 years of age. Importantly, in contrast to training-induced EMG increases, the increase in coherence was maintained at the follow-up measurement 1 month after training. Changes in the strength of coherence in the beta and gamma band were positively correlated with improvements in the subjects’ ability to lift the toes in the swing phase. These data show that daily intensive gait training increases beta and gamma oscillatory drive to ankle dorsiflexor motor neurons and that it improves toe lift and heel strike in children with cerebral palsy. We propose that intensive gait training may produce plastic changes in the corticospinal tract, which are responsible for improvements in gait function. PMID:25623137

  1. Bell's palsy: aetiology, clinical features and multidisciplinary care.

    PubMed

    Eviston, Timothy J; Croxson, Glen R; Kennedy, Peter G E; Hadlock, Tessa; Krishnan, Arun V

    2015-12-01

    Bell's palsy is a common cranial neuropathy causing acute unilateral lower motor neuron facial paralysis. Immune, infective and ischaemic mechanisms are all potential contributors to the development of Bell's palsy, but the precise cause remains unclear. Advancements in the understanding of intra-axonal signal molecules and the molecular mechanisms underpinning Wallerian degeneration may further delineate its pathogenesis along with in vitro studies of virus-axon interactions. Recently published guidelines for the acute treatment of Bell's palsy advocate for steroid monotherapy, although controversy exists over whether combined corticosteroids and antivirals may possibly have a beneficial role in select cases of severe Bell's palsy. For those with longstanding sequaelae from incomplete recovery, aesthetic, functional (nasal patency, eye closure, speech and swallowing) and psychological considerations need to be addressed by the treating team. Increasingly, multidisciplinary collaboration between interested clinicians from a wide variety of subspecialties has proven effective. A patient centred approach utilising physiotherapy, targeted botulinum toxin injection and selective surgical intervention has reduced the burden of long-term disability in facial palsy. PMID:25857657

  2. Whole body vibration and cerebral palsy: a systematic review

    PubMed Central

    Duquette, Sean A.; Guiliano, Anthony M.; Starmer, David J.

    2015-01-01

    Purpose: The goal of this review is to evaluate the effects of whole body vibration on outcomes in patients with cerebral palsy. The findings in this review may help clinicians make evidence informed decisions on the use of whole body vibration for cerebral palsy. Methods: A systematic search was conducted on April 29, 2014.The following search terms were used to search of several databases: (whole body vibration OR whole-body vibration OR whole body-vibration OR WBV) AND (cerebral palsy). Articles that met the inclusion criteria were assessed using the Scottish intercollegiate guidelines network (SIGN) rating system to assess the methodology and bias of the articles for randomized control trials. Results: The search produced 25 articles, of which 12 duplicates were identified and removed. Another seven articles were not considered since they did not fit the inclusion criteria, leaving a total of five studies for review. Four of the articles analyzed the effects of WBV in children while the other study focused on adults with cerebral palsy. There was one low quality article, four acceptable quality articles and one high quality article when assessed using the SIGN criteria. Conclusions: It appears that whole body vibration has the potential to provide symptomatic relief for patients with cerebral palsy. Whole body vibration may improve spasticity, muscle strength and coordination. There is a lack of research to conclusively determine whether it does alter bone mineral density. PMID:26500358

  3. Dynamic touch is affected in children with cerebral palsy.

    PubMed

    Ocarino, Juliana M; Fonseca, Sergio T; Silva, Paula L P; Gonçalves, Gabriela G P; Souza, Thales R; Mancini, Marisa C

    2014-02-01

    Children with developmental disorders such as cerebral palsy have limited opportunities for effortful interactions with objects and tools. The goal of the study was to investigate whether children with cerebral palsy have deficits in their ability to perceive object length by dynamic touch when compared to typically developing children. Fourteen children with typical development and 12 children with cerebral palsy were asked to report the length of hand-held rods after wielding them out of sight. Multilevel regression models indicated that I1 (maximum principal moment of inertia) was a significant predictor of perceived length - LP (p<.0001). The effect of I1 on LP was significantly different among children (p=.001) and the presence of cerebral palsy (group factor) partially explained such variance (p=.002). In addition, accuracy and reliability of the length judgments made by children with cerebral palsy were significantly lower than the typically developing children (p<.05). Theoretical and clinical implications of these results were identified and discussed. PMID:24054355

  4. Frontal deficits differentiate progressive supranuclear palsy from Parkinson's disease.

    PubMed

    Lee, Young-Eun C; Williams, David R; Anderson, Jacqueline F I

    2016-03-01

    The clinical differentiation of progressive supranuclear palsy from Parkinson's disease can be challenging, due to overlapping clinical features and a lack of diagnostic markers. Abnormalities in cognitive function form part of the clinical spectrums of these diseases and distinctive cognitive profiles may be helpful in differentiating these diseases in the diagnostic period. A comprehensive neuropsychological test battery was administered to 12 patients with clinically diagnosed progressive supranuclear palsy and 12 patients with Parkinson's disease matched for age and disease duration. Effect size (Cohen's d) was calculated for cognitive tests that were significantly different between groups. Patients with progressive supranuclear palsy performed significantly worse than those with Parkinson's disease on measures of processing speed, verbal fluency, planning, verbal abstract reasoning, verbal memory, and made more perseverative responses on a set shifting task. Measures of executive function, manual dexterity and processing speed were most diagnostically useful (Cohen's d > 2.0) in differentiating between progressive supranuclear palsy and Parkinson's disease. These findings suggest that more severe and prominent 'frontal' cognitive deficits in patients with progressive parkinsonism would be helpful in predicting progressive supranuclear palsy rather than Parkinson's disease and these findings may contribute to the development of diagnostic criteria. PMID:25223526

  5. Laser Phototherapy As Modality of Clinical Treatment in Bell's Palsy

    NASA Astrophysics Data System (ADS)

    Marques, A. M. C.; Soares, L. G. P.; Marques, R. C.; Pinheiro, A. L. B.; Dent, M.

    2011-08-01

    Bell's palsy is defined as a peripheral facial nerve palsy, idiophatic, and sudden onset and is considered the most common cause of this pathology. It is caused by damage to cranial nerves VII, resulting in complete or partial paralysis of the facial mimic. May be associated with taste disturbances, salivation, tearing and hyperacusis. It is diagnosed after ruling out all possible etiologies, because its cause is not fully understood.Some researches shows that herpes virus may cause this type of palsy due to reactivation of the virus or by imunnomediated post-viral nerve demielinization. Physical therapy, corticosteroids and antiviral therapy have become the most widely accepted treatments for Bell's palsy. Therapy with low-level laser (LLLT) may induce the metabolism of injured nerve tissue for the production of proteins associated with its growth and to improve nerve regeneration. The success of the treatment of Bell's palsy by using laser phototherapy isolated or in association with other therapeutic approach has been reported on the literature. In most cases, the recovery occurs without uneventfully (complications), the acute illness is not associated with serious disorders. We will present a clinical approach for treating this condition.

  6. The Role of Information Systems to Manage Cerebral Palsy

    PubMed Central

    AJAMI, Sima; MAGHSOUDLORAD, Ali Akbar

    2016-01-01

    Objective In healthcare system, it is necessary to have exact and accurate information in order to address health care needs and requirements of society members as well as expectations of policy makers, planners and decision makers. The aim of this narrative review article was to explain the role of information systems in cerebral palsy management and identify the advantages and barriers to the development of cerebral palsy registry system. Data were collected using databases such as of Science Direct, PubMed, Proquest, Springer, and SID (Scientific Information Database). Overall, 65 sources were selected. One of the biggest challenges for children with physical and motor disabilities in rehabilitation center is access to a system, which provides a comprehensive data set reflecting all information on a patient’s care. Thus, data and information management in children with physical and motor disability such as cerebral palsy facilitates access to data and cerebral palsy data comparison as well as the monitoring incidence rate of cerebral palsy, enhancing health care quality; however, there are always numerous barriers to establish the system. One of the ways to overcome these problems is the establishment of a standard framework of minimum data sets and exact definition of its data components. Reliable standards in the use of applications as well as user-friendly software will ensure patients’ data extraction and registration.

  7. [Delayed facial nerve palsy after otologic surgery].

    PubMed

    Kitahara, Tadashi; Kubo, Takeshi; Doi, Katsumi; Mishiro, Yasuo; Kondoh, Kazumasa; Horii, Arata; Okumura, Shin-ichi; Miyahara, Hiroshi

    2006-07-01

    Delayed facial nerve palsy (DFP) is rarely experienced after otologic surgeries that do not directly touch the facial nerves, such as tympano-mastoidectomy, cochlear implants, and stapes surgery, and is troublesome to both surgeons and patients if it happens. Here, we report 7 cases of DFP, including one case that developed DFP after endolymphatic sac surgery. The ratios of occurrence were as follows: 0.7% (2/305) for tympano-mastoidectomy, 0.8% (3/354) for cochlear implant, 0.4% (1/260) for stapes surgery and 1.0% (1/98) for endolymphatic sac surgery. All otologic surgeries, except for endolymphatic sac surgery, exposed the chorda tympani, and all surgeries, except for stapes surgery, underwent drilling for a mastoidectomy. Furthermore, DFP was always observed ipsilaterally to the operated ear after otologic surgeries and was never seen after benign parotid tumor surgery or total laryngectomy. Therefore, there may be a strong relationship between DFP and the procedures, used during otologic surgeries. PMID:16910581

  8. Considerations and complications after Bells' palsy.

    PubMed

    Nemet, Arie Y; Vinker, Shlomo

    2015-12-01

    We present a retrospective, observational study of all patients diagnosed with Bells' palsy (BP) at the Central District of Clalit Health Services from 2003 through to 2012. BP is associated with several complications. We evaluated clinical characteristics including the number of patient visits to general physicians (GP), otolaryngologists, ophthalmologists and neurologists, medications prescribed in the acute phase of BP (steroids and antiviral agents), and the ophthalmic diagnoses. A total of 4463 patients with the diagnosis of BP were included. The incidence per 100,000/year was 87.0, and it increased with age. Patients had significantly more visits to all specialists at 6 months after the BP event. Steroid treatment was prescribed to 50.4% of the patients and antiviral agents to 65.5%. Both treatments were associated with older patient age and female sex. The rate of post BP lagophthalmos and keratitis was 3.45% and 0.63% at 1-3 months, respectively, and both were more likely to develop in older patients. Steroid and/or antiviral treatments were not associated with a decrease in ophthalmic complications. BP may cause ophthalmic complications at a low rate, which are associated with older age. Steroids and antiviral agents appeared to have no effect on ophthalmic complications. PMID:26314659

  9. Rehabilitation Outcomes of Children with Cerebral Palsy

    PubMed Central

    Yalcinkaya, Ebru Yilmaz; Caglar, Nil Sayıner; Tugcu, Betul; Tonbaklar, Aysegul

    2014-01-01

    [Purpose] To evaluate the results of Bobath-based rehabilitation performed at a pediatric cerebral palsy (CP) inpatient clinic. [Subjects and Methods] The study subjects were 28 children with CP who were inpatients at a pediatric service. Inclusion criteria were: being an inpatient of our hospital aged 2–12 with a diagnosis of CP; having one permanent primary caregiver; and the caregiver having no medical or psychotic problems. All of the patients received Bobath treatment for 1 hour per day, 5 days a week. The locomotor system, neurologic and orthopedic examination, Gross Motor Function Measure (GMFM) of the patients, and Short Form-36 (SF-36) of permanent caregivers were evaluated at the time of admission to hospital, discharge from hospital, and at 1 and 3 months after discharge. [Results] Post-admission scores of GMFM at discharge, and 1 and 3 months later showed significant increase. Social function and emotional role subscores of SF-36 had increased significantly at discharge. [Conclusion] Bobath treatment is promising and randomized controlled further studies are needed for rehabilitation technics. PMID:24648650

  10. Lip segmentation and tracking for facial palsy

    NASA Astrophysics Data System (ADS)

    Park, MinJae; Seo, JongMo; Park, KwangSuk

    2006-02-01

    We developed the asymmetry analyzing system for facial palsy patient's rehabilitation progress study. Using PC standard imaging device, captured 640*480 RGB image is converted into HSV space. A Lip-shape mask is extracted by thresholding. By taking 5 regions consisted in one region on lip and four regions on face skin, reasonable thresholds are determined by Fuzzy C-Means clustering. The extreme points on the lip shape mask are extracted to get the seeds for tracking. Segmented seed points are tracking by Iterative Lucas-Kanade tracking method in pyramids at 30 fps and recording simultaneously. To reduce the disk writing load on computer, we use asynchronous mode file writing, which is going to transfer to and review by clinician. Tracking shows quite reliable results, but sometimes the tracked points are following along the lip line because of the similar contrasts. Therefore, the first strategy to improve the reliability of tracking is using the high contrast points, such as left and right maximal point of lip shape. The second is clustering some points near the maximal points and eliminating outlying tracking points. The third is rechecking the lip shape using lip segmentation when the operator confirms that subject's maximal lip moving. Left and right tracking points are compared in forms of trajectory plot.

  11. Gait stability in children with cerebral palsy

    PubMed Central

    Bruijn, Sjoerd M.; Millard, Matthew; van Gestel, Leen; Meyns, Pieter; Jonkers, Ilse; Desloovere, Kaat

    2013-01-01

    Children with unilateral Cerebral Palsy (CP) have several gait impairments, amongst which impaired gait stability may be one. We tested whether a newly developed stability measure (the foot placement estimator, FPE) which does not require long data series, can be used to asses gait stability in typically developing (TD) children as well as children with CP. In doing so, we tested the FPE’s sensitivity to the assumptions needed to calculate this measure, as well as the ability of the FPE to detect differences in stability between children with CP and TD children, and differences in walking speed. Participants were asked to walk at two different speeds, while gait kinematics were recorded. From these data, the FPE, as well as the error that violations of assumptions of the FPE could have caused were calculated. The results showed that children with CP walked with marked instabilities in anterior-posterior and mediolateral directions. Furthermore, errors caused by violations of assumptions in calculation of FPE were only small (~1.5 cm), while effects of walking speed (~20 cm per m/s increase in walking speed) and group (~5cm) were much larger. These results suggest that the FPE may be used to quantify gait stability in TD children and children with CP. PMID:23500163

  12. Malignant lymphoma presented as recurrent multiple cranial nerve palsy after spontaneous regression of oculomotor nerve palsy: A case report.

    PubMed

    Hirose, Takahiko; Nakajima, Hideto; Shigekiyo, Tarou; Yokote, Taiji; Ishida, Shimon; Kimura, Fumiharu

    2016-01-29

    We report the case of a 62-year-old man who presented with malignant lymphoma as recurrent multiple cranial nerve palsy after spontaneous regression of oculomotor nerve palsy. He developed ptosis and diplopia due to right oculomotor nerve palsy. Brain MRI/MRA showed no abnormality, and he recovered with conservative medical management. Three months later, he showed diplopia due to right abducens nerve palsy and facial pain and trigeminal sensory loss. Neurological examination revealed multiple cranial nerve palsy involved cranial nerve III, V, IX, and X of the right side. Serum soluble interleukin-2 receptor levels were normal, and cerebrospinal fluid examination was unremarkable. Steroid and subsequent intravenous immunoglobulin therapy didn't improve his symptoms. Six weeks after his admission, he showed rapid enlargement of the cervical lymph node and the right tonsil, and post-contrast T1-weighted MRI showed enlargement and enhancement of the left infraorbital nerve, the bilateral cavernous sinus, the bilateral facial nerves, and the left trigeminal nerve. The histopathologic examination of the tonsil biopsy revealed diffuse large B cell lymphoma. The cause of these symptoms was thought to be infiltrating the cavernous sinus, and adjacent nerves. Spontaneous regression of malignant lymphoma is an exceptional event, but this possibility should be considered so as to the correct diagnosis and proper treatment. PMID:26616489

  13. Recurrent Isolated Oculomotor Nerve Palsy after Radiation of a Mesencephalic Metastasis. Case Report and Mini Review

    PubMed Central

    Grabau, Olga; Leonhardi, Jochen; Reimers, Carl D.

    2014-01-01

    Introduction: Recurrent oculomotor nerve palsies are extremely rare clinical conditions. Case report: Here, we report on a unique case of a short-lasting recurrent unilateral incomplete external and complete internal oculomotor nerve palsy. The episodic palsies were probably caused by an ipsilateral mesencephalic metastasis of a breast carcinoma and occurred after successful brain radiation therapy. Discussion: While the pathogenic mechanism remains unclear, the recurrent sudden onset and disappearance of the palsies and their decreasing frequency after antiepileptic treatment suggest the occurrence of epilepsy-like brainstem seizures. A review of case reports of spontaneous reversible oculomotor nerve palsies is presented. PMID:25104947

  14. Biomechanical bases of rehabilitation of children with cerebral palsy

    NASA Astrophysics Data System (ADS)

    Davlet'yarova, K. V.; Korshunov, S. D.; Kapilevich, L. V.

    2015-11-01

    Biomechanical analysis and the study results of children's with cerebral palsy (CP) muscles bioelectrical activity while walking on a flat surface are represented. Increased flexion in the hip and shoulder joints and extension in the elbow joint in children with cerebral palsy were observed, with the movement of the lower limbs had less smooth character in comparison with the control group. Herewith, the oscillation amplitude was significantly increased, and the frequency in the m. gastrocnemius and m. lateralis was decreased. It was shown, that the dynamic stereotype of walking in children with cerebral palsy was characterized by excessive involvement of m. gastrocnemius and m.latissimus dorsi in locomotion. Thus, resulting biomechanical and bioelectrical parameters of walking should be considered in the rehabilitation programs development.

  15. Partial third nerve palsy after Measles Mumps Rubella vaccination

    PubMed Central

    2010-01-01

    Background Measles Mumps Rubella (MMR) vaccination is known to cause some serious adverse events, such as fever, rash, gland inflammation and neurologic disorders. These include third and sixth cranial nerve palsies. Results The case reported describes a partial recurrent oculomotor palsy associated with systemic symptoms following MMR vaccination in a healthy young child. The oculomotor palsy did not recover completely during the follow-up. Conclusions Most of the times, measles, mumps and rubella cause mild illness and discomfort; but can also have serious or fatal sequelae. MMR vaccination has been proved to be safe and to reduce significantly the number of reported infections due to these viruses. However, significant adverse events can occur and paediatricians and public health operators should be aware of this aspect. PMID:20831779

  16. Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

    PubMed

    Potulska-Chromik, Anna; Ryniewicz, Barbara; Aragon-Gawinska, Karolina; Kabzinska, Dagmara; Seroka, Andrzej; Lipowska, Marta; Kaminska, Anna M; Kostera-Pruszczyk, Anna

    2016-03-01

    Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) needs to be differentiated from hereditary neuropathy. We aimed to validate existing CIDP nerve conduction study (NCS) criteria in a group of children with demyelinating neuropathies of chronic or subacute onset. Retrospective analysis of clinical and NCS results in 18 children with CIDP, 7 with hereditary neuropathy with pressure palsy (HNPP), and 24 with Charcot-Marie-Tooth 1a (CMT1a). AAN and EFNS electrodiagnostic CIDP criteria were fulfilled in 17 of 18 CIDP, 3 of 7 HNPP, and 23 of 24 CMT1a patients. A distal compound muscle action potential (dCMAP) of >9 ms was observed in 14 of 18 CIDP patients but not in any patients with HNPP. Abnormal median/normal sural SNAP (AMNS) and a 10 m/s difference between conduction velocities (CV) of two corresponding nerves were not observed in any CMT1a patients. NCS in CMT1a, HNPP, and CIDP reflect demyelination. dCMAP duration, sensory AMNS, and a 10 m/s CV difference parameter are most useful in the differential diagnosis of pediatric CIDP. PMID:26663344

  17. Entrapment in anti myelin-associated glycoprotein neuropathy.

    PubMed

    Faber, Catharina G; Notermans, Nicolette C; Wokke, John H J; Franssen, Hessel

    2009-04-01

    Anti-myelin associated glycoprotein (MAG) neuropathy is a chronic disorder in which IgM antibodies react with Schwann cell glycoproteins, including MAG and peripheral myelin protein 22 (PMP22). Nerve conduction studies show features of axon loss and predominantly distal slowing consistent with demyelination. Because a genetic loss of PMP22 function yields hereditary neuropathy with liability to pressure palsies (HNPP), loss of PMP22 function due to anti- MAG antibodies may result in increased sensitivity to entrapment. We investigated this by performing standardized electrophysiological studies in 16 patients with anti-MAG neuropathy and 16 disease controls with genetically confirmed HNPP. Disproportionate slowing relative to adjacent segments occurred in similar proportions of patients with anti-MAG neuropathy and HNPP, and was of the same magnitude in each group. Affected were the elbow, carpal tunnel and the wrist-hand segments of the median and ulnar nerves. However, in anti-MAG neuropathy as compared to HNPP, absolute values of distal motor latencies and conduction velocities outside entrapment sites were slower and amplitudes were lower. In conclusion, increased sensitivity for entrapment may occur in anti-MAG neuropathy and contribute to part of the nerve damage. PMID:19306083

  18. Workplace cluster of Bell’s palsy in Lima, Peru

    PubMed Central

    2014-01-01

    Background We report on a workplace cluster of Bell’s palsy that occurred within a four-month period in 2011 among employees of a three-story office building in Lima, Peru and our investigation to determine the etiology and associated risk factors. Findings An outbreak investigation was conducted to identify possible common infectious or environmental exposures and included patient interviews, reviews of medical records, an epidemiologic survey, serological analysis for IgM and IgG antibodies to putative Bell’s palsy-inducing pathogens, and an environmental exposure assessment of the office building. Three cases of Bell’s palsy were reported among 65 at-risk employees, attack rate 4.6%. Although two patients had underlying risk factors, there was no clear association or common identifiable risk factor among all cases. Serologic analysis showed no evidence of recent infections, and air and water sample measures of all known chemical or neurotoxins were below maximum allowable concentrations for exposure. Conclusions An infection spread among workplace employees could not be excluded as a potential cause of this cluster; however, it was unlikely a pathogen commonly associated with individual cases of Bell’s palsy. Although a specific etiology was not identified among all cases, we believe this methodology will aid future outbreak investigations of Bell’s palsy and a better understanding of its etiology. While environmental assessments may be useful in their ability to ascertain the cause of clusters of Bell’s palsy, future investigations should prioritize focus on common infectious etiology. PMID:24885256

  19. Multiple cranial nerve palsies associated with type 2 diabetes mellitus.

    PubMed

    Singh, N P; Garg, S; Kumar, S; Gulati, S

    2006-08-01

    Although isolated cranial nerve palsies are common in patients with diabetes mellitus, multiple simultaneous cranial neuropathies are rare. We report a 48-year-old man, a known case of diabetes mellitus, who presented with facial palsy, foot drop and painful ophthalmoplegia of the left eye. The initial differential diagnosis included diabetic polyneuropathy, septic cavernous sinus thrombosis, mucormycosis and the Tolosa Hunt syndrome. Magnetic resonance (MR) imaging findings were consistent with those of the Tolosa Hunt syndrome. The patient had a remarkable complete resolution of his ophthalmoplegia after four weeks of steroid treatment, with repeat MR imaging showing resolution of the initial changes. PMID:16865214

  20. Stepwise surgical approach to equinocavovarus in patients with cerebral palsy.

    PubMed

    Won, Sung Hun; Kwon, Soon Sun; Chung, Chin Youb; Lee, Kyoung Min; Lee, In Hyeok; Jung, Ki Jin; Moon, Sang Young; Chung, Myung Ki; Park, Moon Seok

    2016-03-01

    This study investigated the radiologic results of a stepwise surgical approach to equinocavovarus in 24 patients with cerebral palsy and determined the extent to which each procedure affected radiographic parameters using a linear mixed model. The anteroposterior talus-first metatarsal and anteroposterior talonavicular coverage angles were improved. The calcaneal pitch angle, tibiocalcaneal angle, lateral talus-first metatarsal angle, and naviculocuboid overlap were also improved. The Dwyer sliding osteotomy affected the tibiocalcaneal angle, whereas first metatarsal dorsal wedge osteotomy improved the calcaneal pitch angle and lateral first metatarsal angle. The stepwise surgical approach is effective for correction of equinocavovarus in cerebral palsy patients. PMID:26529433

  1. Early postnatal hypotension is not associated with indicators of white matter damage or cerebral palsy in extremely low gestational age newborns

    PubMed Central

    Logan, J. Wells; O’Shea, T. Michael; Allred, Elizabeth N.; Laughon, Matthew M.; Bose, Carl L.; Dammann, Olaf; Batton, Daniel G.; Kuban, Karl C.; Paneth, Nigel; Leviton, Alan

    2011-01-01

    Objectives To evaluate, in extremely low gestational age newborns (ELGANs), relationships between indicators of early postnatal hypotension and cranial ultrasound indicators of cerebral white matter damage imaged in the nursery and cerebral palsy diagnoses at 24 month follow-up. Methods The 1041 infants in this prospective study were born at < 28 weeks gestation, were assessed for 3 indicators of hypotension in the first 24 postnatal hours, had at least one set of protocol cranial ultrasound scans, and were evaluated with a structured neurologic exam at 24 months corrected age. Indicators of hypotension included: 1) lowest mean arterial pressure (MAP) in the lowest quartile for gestational age; 2) treatment with a vasopressor; and 3) blood pressure lability, defined as the upper quartile of the difference between each infant’s lowest and highest MAP. Outcomes included indicators of cerebral white matter damage, i.e. moderate/severe ventriculomegaly or an echolucent lesion on cranial ultrasound, and cerebral palsy diagnoses at 24 months gestation. Logistic regression was used to evaluate relationships among hypotension indicators and outcomes, adjusting for potential confounders. Results Twenty-one percent of surviving infants had a lowest blood pressure in the lowest quartile for gestational age, 24% were treated with vasopressors, and 24% had labile blood pressure. Among infants with these hypotension indicators, 10% percent developed ventriculomegaly and 7% developed an echolucent lesion. At 24-months follow-up, 6% had developed quadriparesis, 4% diparesis, and 2% hemiparesis. After adjusting for confounders, we found no association between indicators of hypotension, and indicators of cerebral white matter damage or a cerebral palsy diagnosis. Conclusions The absence of an association between indicators of hypotension and cerebral white matter damage and or cerebral palsy suggests that early hypotension may not be important in the pathogenesis of brain injury in ELGANs. PMID:21273984

  2. Cerebral Palsy Among Asian Ethnic Subgroups

    PubMed Central

    Lang, Tess C.; Fuentes-Afflick, Elena; Gilbert, William M.; Newman, Thomas B.; Xing, Guibo

    2012-01-01

    OBJECTIVE: Asians have a reduced risk for cerebral palsy (CP) compared with whites. We examined whether individual Asian subgroups have a reduced risk of CP and whether differences in sociodemographic factors explain disparities in CP prevalence. METHODS: In a retrospective cohort of 629 542 Asian and 2 109 550 white births in California from 1991 to 2001, we identified all children who qualified for services from the California Department of Health Services on the basis of CP. Asians were categorized as East Asian (Chinese, Japanese, Koreans), Filipino, Indian, Pacific Islander (Guamanians, Hawaiians, and Pacific Islanders), Samoan, or Southeast Asian (Cambodian, Laotian, Thai, Vietnamese). RESULTS: Overall, CP prevalence was lower in Asians than whites (1.09 vs 1.36 per 1000; relative risk = 0.80, 95% confidence interval [CI] = 0.74–0.87) and ranged from 0.61/1000 in Thai children to 2.08/1000 in Samoan children. Several Asian subgroups had low risk profiles with respect to maternal age, educational attainment, and birth weight. However, after we adjusted for maternal age and education, infant gender, and birth weight, the adjusted risk of CP remained lower in East Asians (odds ratio [OR] = 0.75, 95% CI = 0.65–0.87), Filipinos (OR = 0.87, 95% CI = 0.75–0.99), Indians (OR = 0.59, 95% CI = 0.44–0.80), Pacific Islanders (OR = 0.62, 95% CI = 0.40–0.97), and Southeast Asians (OR = 0.68, 95% CI = 0.57–0.82) compared with whites. CONCLUSIONS: Most Asian national origin subgroups have a lower rate of CP than whites, and this disparity is unexplained. Additional studies that focus on the cause of ethnic disparities in CP may provide new insights into pathogenesis and prevention. PMID:22430449

  3. Dietary Practices in Saudi Cerebral Palsy Children

    PubMed Central

    Al-Hammad, Nouf S.

    2015-01-01

    Objectives: To determine the dietary practices of Saudi cerebral palsy (CP) children. Methods: A self-administered questionnaire was used to collect the following information from parents of CP children: demographics, main source of dietary information, frequency of main meals, foods/drinks used for main meals and in-between-meals. Results: Parents of 157 CP children participated. Parents were divided into three, while children were divided into two age groups. The main sources of dietary information included popular media (46.5%) and dentist (36.3%). Most of the children had three meals (71.3%) or two meals (24.8%) daily. Choices for main meals included meats (68.8%), vegetables (65.6%), fruits (28.4%) and puddings (38.9%). The main three drinks choices with main meals included packed juices (59.9%), bottled water (58.8%) and fresh fruit juices (33.1%). The choices for in-between meals snacks included biscuits (61.1%), potato chips (51.6%), fruits (43.9%) and chocolates (41.4%). The choice of drinks with snacks was similar to that used with main meals. In cross-tabulation, older parents used meat (p=.03) and soft drinks (p=.04) more often for their children’s main meals. Older children were given meat (p=.004) and soft drinks (p=.04) more often with main meals. Older children were given potato chips as snacks more often than younger children (p=.02), and there was a trend towards use of chocolates as snacks in older children (p=.08). Conclusion: Parents of CP children need to be educated about dietary practices of their children especially in areas such as the use of packed juices, dairy products, soft drinks and chocolates. PMID:26430418

  4. Parental infertility and cerebral palsy in children

    PubMed Central

    Zhu, Jin Liang; Hvidtjørn, Dorte; Basso, Olga; Obel, Carsten; Thorsen, Poul; Uldall, Peter; Olsen, Jørn

    2010-01-01

    BACKGROUND Children born after in vitro fertilization (IVF) or intracytoplasmic sperm injection (ICSI) have been reported to have a higher risk of cerebral palsy (CP), perhaps due to the higher frequency of preterm birth, multiple births or vanishing embryo in the pregnancies. However, it has been suggested that the underlying infertility may be part of the pathway. In this study, we examined whether untreated subfecundity (measured by time to pregnancy) or infertility treatment was associated with an increased risk of CP in the offspring. METHODS Using the Danish National Birth Cohort (1997–2003), we compared children born after 0–2 months of waiting time to pregnancy (n = 35 848) with those born after a time to pregnancy of 3–5 months (n = 15 361), 6–12 months (n = 11 528) and >12 months (n = 7387), as well as those born after IVF/ICSI (n = 3617), ovulation induction with or without intrauterine insemination (n = 3000), and unplanned pregnancies (n = 13 462). CP cases were identified through the Danish CP Register. RESULTS In total, 165 (0.18%) children were diagnosed with CP in the entire cohort. We found no significant association between time to pregnancy and the risk of CP in children conceived spontaneously. Children born after IVF/ICSI had an increased risk of CP, even after adjustment for preterm birth and multiplicity (hazard ratio 2.30, 95% confidence interval 1.12–4.73). CONCLUSIONS Subfecundity per se did not appear to be associated with the risk of CP in children, whereas being born after IVF/ICSI conferred an increased risk. PMID:21045245

  5. Narrative ability in children with cerebral palsy.

    PubMed

    Holck, Pernille; Dahlgren Sandberg, Annika; Nettelbladt, Ulrika

    2011-01-01

    In a previous study a group of children with cerebral palsy (CP) were found to have considerable difficulties with narratives, performing several standard deviations below the criteria for the Information score of the Bus Story Test (BST). To examine in depth the performance of children with CP and a control group with typically developing (TD) children on a narrative task, in order to search for possible underlying causes to the problems in the CP group. The results of the BST for 10 children with CP, mean age 7;11 years, were investigated. The analysis of the BST was supplemented with the use of the Narrative Assessment Profile (NAP) and quantitative analyses of number of words, mazes, propositions, types of conjunctions and story elements. A significant relationship between the explicitness dimension on the Narrative Assessment Profile and the BST Information score in the CP group suggested that the problems could be derived to a limited use of cohesion and a scarcity of essential information. Compared to the CP group, the TD group used significantly more causal conjunctions. The results indicate a general problem with cohesion at the textual level in the CP group. A further finding was the occurrence of a positive correlation between the use of mazes and the BST Information score in the CP group. These results have implications for the design of a more specific intervention for children, where the NAP was found to be a valuable tool in combination with the BST or other assessment materials. Further, it is shown that mazes, mostly regarded as a behaviour that not enhances speech production, for some children can be used as a means to find necessary words and pieces of information. PMID:21041064

  6. Treating cerebral palsy with aculaser therapy

    NASA Astrophysics Data System (ADS)

    Anwar, Shahzad; Nazir Khan, Malik M.; Nadeem Khan, Malik M.; Qazi, Faiza M.; Awan, Abid H.; Dar, Irfan

    2008-03-01

    A single, open and non comparative study was conducted at Anwar Shah Trust for C.P. & Paralysis in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (C.P.) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, diplegia, quadriplegia, monoplegia, sensory-neural deafness and speech disorders. In all 250 childern were treated and the data was gathered during a period of 3 years from December 2003 till December 2006. These children were further classified according to the type of C.P. (spastic, athetoid, mixed) they suffered from and associated Neurological Disorders. This article shows results in C.P. childern who were treated with ACULASER THERAPY for minimum 6 weeks and more or had minimum of 15 treatment sessions and more. This article also shows that those childern who were given a break in the treatment for 1 month to 1 year did not show any reversal of the signs and symptoms. Analysis of the data showed that out of 171 children with Spasticity and Stiffness 147 showed marked improvement showing 87% success rate, out of 126 children with Epileptic fits, there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 91 children showing 72% success rate, out of 48 children with Cortical Blindness 30 children showed improvement accounting for 63% efficacy rate, out of 105 children with Hearing Difficulties, 63 showed marked improvement accounting for 60% improvement rate, out of 190 children with Speech Disorders 122 showed improvement reflecting 64% improvement rate, out of 96 children with Hemiplegia 71 showed improvement in movement, tone and power accounting for 74% improvement rate, out of 76 children with Quadriplegia 52 showed improvement in gross and fine motor functions showing 69% success rate and out of 58 children with Paraplegia of lower limbs 44 showed improvement in weight bearing, standing and movement accounting for 76% improvement rate.

  7. Rare copy number variation in cerebral palsy

    PubMed Central

    McMichael, Gai; Girirajan, Santhosh; Moreno-De-Luca, Andres; Gecz, Jozef; Shard, Chloe; Nguyen, Lam Son; Nicholl, Jillian; Gibson, Catherine; Haan, Eric; Eichler, Evan; Martin, Christa Lese; MacLennan, Alastair

    2014-01-01

    Recent studies have established the role of rare copy number variants (CNVs) in several neurological disorders but the contribution of rare CNVs to cerebral palsy (CP) is not known. Fifty Caucasian families having children with CP were studied using two microarray designs. Potentially pathogenic, rare (<1% population frequency) CNVs were identified, and their frequency determined, by comparing the CNVs found in cases with 8329 adult controls with no known neurological disorders. Ten of the 50 cases (20%) had rare CNVs of potential relevance to CP; there were a total of 14 CNVs, which were observed in <0.1% (<8/8329) of the control population. Eight inherited from an unaffected mother: a 751-kb deletion including FSCB, a 1.5-Mb duplication of 7q21.13, a 534-kb duplication of 15q11.2, a 446-kb duplication including CTNND2, a 219-kb duplication including MCPH1, a 169-kb duplication of 22q13.33, a 64-kb duplication of MC2R, and a 135-bp exonic deletion of SLC06A1. Three inherited from an unaffected father: a 386-kb deletion of 12p12.2-p12.1, a 234-kb duplication of 10q26.13, and a 4-kb exonic deletion of COPS3. The inheritance was unknown for three CNVs: a 157-bp exonic deletion of ACOX1, a 693-kb duplication of 17q25.3, and a 265-kb duplication of DAAM1. This is the first systematic study of CNVs in CP, and although it did not identify de novo mutations, has shown inherited, rare CNVs involving potentially pathogenic genes and pathways requiring further investigation. PMID:23695280

  8. Quantity Concept Development in Individuals with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Lister, Caroline; Juniper, Kirsten

    1995-01-01

    Investigated order of quantity concept development on a range of quantity conservation tasks. Subjects were 15 adults and children having spastic cerebral palsy from birth and attending the same special school or day unit. Found a sequence of development similar to that in children without the handicap, and scalogram analysis supported similarity…

  9. Portrayals of People with Cerebral Palsy in Homicide News

    ERIC Educational Resources Information Center

    Lucardie, Richard; Sobsey, Dick

    2005-01-01

    Through content analysis, employing qualitative and quantitative methods, Canadian media representation of people with cerebral palsy (PWCP) in public life was examined. Canadian NewsDisc, an online biographic database service, was used to examine the use of stigmatizing language such as afflicted by, afflicted with, suffered from, suffers from,…

  10. Childhood Educational Experiences of Women with Cerebral Palsy

    ERIC Educational Resources Information Center

    Freeborn, Donna; Mandleco, Barbara

    2010-01-01

    The purpose of this study was to examine the childhood experiences of women with cerebral palsy (CP), from the perspectives of these women. Using the feminist biographical method, eight women with CP participated in two in-depth interviews. Participants ranged in age from 22 to 55 years and had moderate to severe athetoid or spastic CP. Four…

  11. Home Literacy Environment: Characteristics of Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Peeters, Marieke; Verhoeven, Ludo; van Balkom, Hans; de Moor, Jan

    2009-01-01

    Background: Various aspects of the home literacy environment are considered to stimulate the emergent literacy development in children without disabilities. It is important to gain insight into the home literacy environment of children with cerebral palsy given that they have been shown to have difficulty acquiring literacy skills. Aims: The aims…

  12. Understanding Participation of Preschool-Age Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Chiarello, Lisa Ann; Palisano, Robert J.; Orlin, Margo N.; Chang, Hui-Ju; Begnoche, Denise; An, Mihee

    2012-01-01

    Participation in home, school, and community activities is a primary outcome of early intervention services for children with disabilities and their families. The objectives of this study were to (a) describe participation of preschool-age children with cerebral palsy (CP); (b) determine effects of sex, age, and gross motor function on intensity…

  13. Functional Electrical Stimulation in Children and Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    van der Linden, Marietta

    2012-01-01

    In this article, the author talks about functional electrical stimulation in children and adolescents with cerebral palsy. Functional electrical stimulation (FES) is defined as the electrical stimulation of muscles that have impaired motor control, in order to produce a contraction to obtain functionally useful movement. It was first proposed in…

  14. Robot-Assisted Task-Specific Training in Cerebral Palsy

    ERIC Educational Resources Information Center

    Krebs, Hermano I.; Ladenheim, Barbara; Hippolyte, Christopher; Monterroso, Linda; Mast, Joelle

    2009-01-01

    Our goal was to examine the feasibility of applying therapeutic robotics to children and adults with severe to moderate impairment due to cerebral palsy (CP). Pilot results demonstrated significant gains for both groups. These results suggest that robot-mediated therapy may be an effective tool to ameliorate the debilitating effects of CP and…

  15. Predictors of Verbal Working Memory in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Peeters, Marieke; Verhoeven, Ludo; de Moor, Jan

    2009-01-01

    The goal of the present study was to examine the precursors of verbal working memory in 52 children with cerebral palsy with varying degrees of speech impairments in the first grade of special education. Following Baddeley's model of working memory, children's verbal working memory was measured by means of a forced-recognition task. As precursors…

  16. Language and Motor Speech Skills in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Pirila, Silja; van der Meere, Jaap; Pentikainen, Taina; Ruusu-Niemi, Pirjo; Korpela, Raija; Kilpinen, Jenni; Nieminen, Pirkko

    2007-01-01

    The aim of the study was to investigate associations between the severity of motor limitations, cognitive difficulties, language and motor speech problems in children with cerebral palsy. Also, the predictive power of neonatal cranial ultrasound findings on later outcome was investigated. For this purpose, 36 children (age range 1 year 10 months…

  17. Intermittent versus Continuous Physiotherapy in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Christiansen, Annette Sandahl; Lange, Christa

    2008-01-01

    The aim of this study was to compare the effect of the delivery of the same amount of intermittent versus continuous physiotherapy given to children with cerebral palsy (CP). This was organized either in an intermittent regime four times a week for 4 weeks alternating with a 6-week treatment pause, or a continuous once or twice a week regime, both…

  18. Early Numeracy in Cerebral Palsy: Review and Future Research

    ERIC Educational Resources Information Center

    van Rooijen, Maaike; Verhoeven, Ludo; Steenbergen, Bert

    2011-01-01

    Children with cerebral palsy (CP) often have problems with arithmetic, but the development of numerical abilities in these children has received only minor attention. In comparison, detailed accounts have been written on the arithmetic abilities of typically developing children, but a theoretical framework is still lacking. A promising perspective…

  19. Growth and Nutrition Disorders in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Kuperminc, Michelle N.; Stevenson, Richard D.

    2008-01-01

    Growth and nutrition disorders are common secondary health conditions in children with cerebral palsy (CP). Poor growth and malnutrition in CP merit study because of their impact on health, including psychological and physiological function, healthcare utilization, societal participation, motor function, and survival. Understanding the etiology of

  20. Diversity of Participation in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Imms, Christine; Reilly, Sheena; Carlin, John; Dodd, Karen

    2008-01-01

    The aim of this study was to investigate the participation of children with cerebral palsy (CP) in activities outside school and to compare their participation with a large representative sample of children. A population-based survey was conducted of children with CP born in Victoria, Australia in 1994 and 1995. Of 219 living children identified,…

  1. Bimanual Force Coordination in Children with Spastic Unilateral Cerebral Palsy

    ERIC Educational Resources Information Center

    Smits-Engelsman, B. C. M.; Klingels, K.; Feys, H.

    2011-01-01

    In this study bimanual grip-force coordination was quantified using a novel "Gripper" system that records grip forces produced while holding a lower and upper unit, in combination with the lift force necessary to separate these units. Children with unilateral cerebral palsy (CP) (aged 5-14 years, n = 12) were compared to age matched typically…

  2. MRI and Motor Outcomes in Children with Cerebral Palsy.

    PubMed

    Gaebler-Spira, Deborah; McCormick, Kristen

    2015-08-01

    Investigators from University of Melbourne, Monash Children's Hospital, Royal Children's Hospital & Murdoch Children's Research Institute sought to identify correlation between magnetic resonance imaging (MRI) characteristics including white matter injury (WMI) in children with cerebral palsy (CP) and severity in motor outcomes later in life, irrespective of CP subtype. PMID:26933597

  3. Understanding Participation of Preschool-Age Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Chiarello, Lisa Ann; Palisano, Robert J.; Orlin, Margo N.; Chang, Hui-Ju; Begnoche, Denise; An, Mihee

    2012-01-01

    Participation in home, school, and community activities is a primary outcome of early intervention services for children with disabilities and their families. The objectives of this study were to (a) describe participation of preschool-age children with cerebral palsy (CP); (b) determine effects of sex, age, and gross motor function on intensity

  4. Training Guide to Cerebral Palsy Sports. Third Edition.

    ERIC Educational Resources Information Center

    Jones, Jeffery A., Ed.

    This official training manual of the United States Cerebral Palsy Athletic Association includes the latest coaching and training techniques specific to all sports in the national program. The book features guidelines for coaching over a dozen sports, including soccer, swimming, cycling, and track and field. It contains everything coaches,…

  5. Cerebral palsy litigation: change course or abandon ship.

    PubMed

    Sartwelle, Thomas P; Johnston, James C

    2015-06-01

    The cardinal driver of cerebral palsy litigation is electronic fetal monitoring, which has continued unabated for 40 years. Electronic fetal monitoring, however, is based on 19th-century childbirth myths, a virtually nonexistent scientific foundation, and has a false positive rate exceeding 99%. It has not affected the incidence of cerebral palsy. Electronic fetal monitoring has, however, increased the cesarian section rate, with the expected increase in mortality and morbidity risks to mothers and babies alike. This article explains why electronic fetal monitoring remains endorsed as efficacious in the worlds' labor rooms and courtrooms despite being such a feeble medical modality. It also reviews the reasons professional organizations have failed to condemn the use of electronic fetal monitoring in courtrooms. The failures of tort reform, special cerebral palsy courts, and damage limits to stem the escalating litigation are discussed. Finally, the authors propose using a currently available evidence rule-the Daubert doctrine that excludes "junk science" from the courtroom-as the beginning of the end to cerebral palsy litigation and electronic fetal monitoring's 40-year masquerade as science. PMID:25183322

  6. Bathing Techniques for Children Who Have Cerebral Palsy.

    ERIC Educational Resources Information Center

    Dunaway, Avtar; Klein, Marsha Dunn

    Helpful techniques are offered for making bathtime easier, safer, and more fun for children who have cerebral palsy. Safety in the bathtub is stressed, both for the child who needs protection from slippery surfaces and extreme water temperature, and for the caregiver who must lift and carry the child without causing injury to the lower back.

  7. Comparing Scanning Modes for Youths with Cerebral Palsy. Final Report.

    ERIC Educational Resources Information Center

    Ottenbacher, Kenneth J.; Angelo, Jennifer

    This study of 22 individuals (ages 13-20) with cerebral palsy investigated the use of scanning, an interface technique that allows access to assistive devices such as communication boards, electronic augmentative communication devices, and computers by using a pointer, either a finger or a cursor. This packet of information includes the findings…

  8. Tactile Assessment in Children with Cerebral Palsy: A Clinimetric Review

    ERIC Educational Resources Information Center

    Auld, Megan Louise; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

    2011-01-01

    This review evaluates the clinimetric properties of tactile assessments for children with cerebral palsy. Assessment of registration was reported using Semmes Weinstein Monofilaments (SWMs) or exteroception. Assessment of two-point discrimination was reported using the Disk-Criminator[R] or paperclip methods; Single point localization and double…

  9. Degree of Involvement and Young Children with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Parette, H. P., Jr.; And Others

    1996-01-01

    Studies of the degree of involvement (DOI) and its relationship to therapeutic intervention effectiveness and related services for young children with cerebral palsy were reviewed. Three dimensions of DOI: (1) brain damage and mental retardation, (2) functional motor ability, and (3) emotional disturbance and behavior problems were reviewed. The…

  10. Progressive supranuclear palsy: what do we know about it?

    PubMed

    Long, Ling; Cai, Xiao-Dong; Wei, Xiao-Bo; Liao, Jin-Chi; Xu, Yun-Qi; Gao, Hui-Min; Chen, Xiao-Hong; Wang, Qing

    2015-01-01

    Progressive supranuclear palsy (PSP) is a progressive tauopathy characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, axial rigidity, frontal lobe dysfunction, and dementia. The typical pathology includes neuronal loss, gliosis and microtubule-associated protein tau (MAPT)-positive inclusions in neurons and glial cells, primarily in basal ganglia, brainstem and cerebellum. The pathogenesis of PSP is not yet completely understood; however, there are several hypotheses. This article reviews the present knowledge about PSP, and the concepts underlying mitochondrial dysfunction, lipoperoxidation, and gene mutations. The clinical features of PSP are also discussed; these include vertical gaze palsy, pseudobulbar palsy, aphasia, dysarthria, axial rigidity, and neuropsychiatric symptoms, such as amnesia, irritability, loss of interest, and dementia. In terms of diagnosis, there is considerable interest in neuroimaging for detecting PSP; therefore, neuroimaging techniques such as magnetic resonance imaging (MRI) and [18F]- fluorodeoxyglucose positron-emission tomography (FDG-PET) are reviewed. A definitive diagnosis of PSP depends on pathology, and the introduction of new clinical subtypes challenges presents the widely adopted diagnosis criteria. PSP treatments such as serotonin antagonists, α2 receptor antagonists, and coenzyme Q10 are also discussed. There is no curative therapy for PSP; all of the available treatments are palliative. PMID:25732053

  11. Hansen's disease and HIV coinfection with facial nerve palsy

    PubMed Central

    Yadav, Nidhi; Kar, Sumit; Madke, Bhushan; Gangane, Nitin

    2015-01-01

    There are very few published reports of HIV leprosy co infection in India in spite of having a large burden of both leprosy and HIV. Herein we are reporting a case of co-infection of Hansen's disease and HIV with facial nerve palsy. PMID:25883486

  12. Cerebral Palsy Symptoms in Children Decreased Following Massage Therapy

    ERIC Educational Resources Information Center

    Hernandez-Reif, Maria; Field, Tiffany; Largie, Shay; Diego, Miguel; Manigat, Natasha; Seoanes, Jacqueline; Bornstein, Joan

    2005-01-01

    Twenty young children (mean age = 32 months) with cerebral palsy (CP) recruited from early intervention programs received 30 minutes of massage or reading twice weekly for 12 weeks. The children receiving massage therapy showed fewer physical symptoms including reduced spasticity, less rigid muscle tone overall and in the arms, and improved fine…

  13. Behaviour in Children with Cerebral Palsy with and without Epilepsy

    ERIC Educational Resources Information Center

    Carlsson, Malin; Olsson, Ingrid; Hagberg, Gudrun; Beckung, Eva

    2008-01-01

    The aim of the study was to describe behavioural problems in children with cerebral palsy (CP) with and without epilepsy. The children were sampled from the Western Sweden CP register and were part of a European Union project. The Strength and Difficulties Questionnaire and questions on epilepsy were answered by one parent of each child. Medical

  14. [Treatment of Bell's palsy with acyclovir and prednisolone].

    PubMed

    Hato, N; Honda, N; Gyo, K; Aono, H; Murakami, S; Yanagihara, N

    2000-02-01

    Many current studies have suggested that herpes simplex virus is a probable cause of Bell's palsy, and that treatment with antiviral agents such as acyclovir might benefit the patients. In the present study, 69 patients with Bell's palsy were treated with oral administration of acyclovir (2000 mg/day) and prednisolone (60-40 mg/day) at Ehime University Hospital between Oct. 1995 and Dec. 1998. Patients enrolled in this study met the following criteria: 1) severe or complete paralysis with a score lower than 20 by the 40-point Japanese grading system, and 2) treatment started within 7 days of onset. The overall recovery rate was 95.7% (66/69). The rate in patients who started this treatment within 3 days after disease onset was 100%, and this early treatment was highly efficacious in the prevention of nerve degeneration and resulted in a significantly better recovery. By comparison, the recovery rate in patients whose treatment was started 4 days or more after onset was only 84.2%. All patients who were given a diagnosis of zoster sine herpete and treated with acyclovir-prednisolone had a good outcome. These results suggest that early treatment, within 3 days after palsy onset, is necessary for effective acyclovir-prednisolone therapy of Bell's palsy. PMID:10737002

  15. Bathing Techniques for Children Who Have Cerebral Palsy.

    ERIC Educational Resources Information Center

    Dunaway, Avtar; Klein, Marsha Dunn

    Helpful techniques are offered for making bathtime easier, safer, and more fun for children who have cerebral palsy. Safety in the bathtub is stressed, both for the child who needs protection from slippery surfaces and extreme water temperature, and for the caregiver who must lift and carry the child without causing injury to the lower back.…

  16. Monocular elevation deficiency ("double elevator" palsy): a cautionary note.

    PubMed

    Brodsky, Michael C; Karlsson, Virginia

    2011-03-01

    Monocular elevation deficiency (or "double elevator" palsy) is a descriptive term denoting a congenital deficiency of monocular elevation that is equal in abduction and adduction. We describe a child with monocular elevation deficiency who displayed tethering and buckling of the central lower eyelid in downgaze. We caution that this manifestation of inferior rectus contracture can simulate impaired infraduction in the involved eye. PMID:21131851

  17. Reproducibility of Tactile Assessments for Children with Unilateral Cerebral Palsy

    ERIC Educational Resources Information Center

    Auld, Megan Louise; Ware, Robert S.; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

    2012-01-01

    A systematic review identified tactile assessments used in children with cerebral palsy (CP), but their reproducibility is unknown. Sixteen children with unilateral CP and 31 typically developing children (TDC) were assessed 2-4 weeks apart. Test-retest percent agreements within one point for children with unilateral CP (and TDC) were

  18. Aerobic Capacity in Children and Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Verschuren, Olaf; Takken, Tim

    2010-01-01

    This study described the aerobic capacity [VO[subscript 2peak] (ml/kg/min)] in contemporary children and adolescents with cerebral palsy (CP) using a maximal exercise test protocol. Twenty-four children and adolescents with CP classified at Gross Motor Functional Classification Scale (GMFCS) level I or level II and 336 typically developing

  19. Physical and Sedentary Activity in Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Maher, Carol A.; Williams, Marie T.; Olds, Tim; Lane, Alison E.

    2007-01-01

    Participation in regular physical activity (PA) provides health, psychological, and physiological benefits for people with and without a physical disability. This study investigated the physical and sedentary activity patterns of adolescents with cerebral palsy (CP). A cross-sectional, descriptive, postal survey was used, consisting of the…

  20. Handling the Young Cerebral Palsied Child at Home.

    ERIC Educational Resources Information Center

    Finnie, Nancie R.

    Written primarily for parents of cerebral palsied children, the text discusses and illustrates methods for handling the child in daily activities. Introduced with a questionnaire concerning developmental stages and activity levels, the manual describes the most common difficulties of the spastic, athetoid, ataxic, or flaccid child. Drawings and…

  1. Tactile Assessment in Children with Cerebral Palsy: A Clinimetric Review

    ERIC Educational Resources Information Center

    Auld, Megan Louise; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

    2011-01-01

    This review evaluates the clinimetric properties of tactile assessments for children with cerebral palsy. Assessment of registration was reported using Semmes Weinstein Monofilaments (SWMs) or exteroception. Assessment of two-point discrimination was reported using the Disk-Criminator[R] or paperclip methods; Single point localization and double

  2. Behaviour in Children with Cerebral Palsy with and without Epilepsy

    ERIC Educational Resources Information Center

    Carlsson, Malin; Olsson, Ingrid; Hagberg, Gudrun; Beckung, Eva

    2008-01-01

    The aim of the study was to describe behavioural problems in children with cerebral palsy (CP) with and without epilepsy. The children were sampled from the Western Sweden CP register and were part of a European Union project. The Strength and Difficulties Questionnaire and questions on epilepsy were answered by one parent of each child. Medical…

  3. A case of Todd's Palsy following unilateral electroconvulsive therapy.

    PubMed

    Bell, Christine; Lepping, Peter; Clifford, John; Gardner-Thorpe, Catherine

    2012-04-01

    This case describes a woman undergoing unilateral electroconvulsive therapy (ECT) who developed a Todd's Palsy following the treatment, and which resolved when converted to bilateral ECT. We go on to hypothesize that this rare side effect may be an indication of the need to switch laterality during a course of ECT. PMID:22988330

  4. Degree of Involvement and Young Children with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Parette, H. P., Jr.; And Others

    1996-01-01

    Studies of the degree of involvement (DOI) and its relationship to therapeutic intervention effectiveness and related services for young children with cerebral palsy were reviewed. Three dimensions of DOI: (1) brain damage and mental retardation, (2) functional motor ability, and (3) emotional disturbance and behavior problems were reviewed. The

  5. [Sixth nerve palsies in children. Presentation of four cases].

    PubMed

    Zimmermann-Paiz, Martin A; Fang-Sung, Jen Wen

    2008-10-01

    The etiology of the sixth nerve palsy in children includes multiple causes, being the acquired ones the most frequent. Due to the importance of the adequate management and possible implications of this pathology, four patients are presented for analysis and discussion. PMID:19030647

  6. Robot-Assisted Task-Specific Training in Cerebral Palsy

    ERIC Educational Resources Information Center

    Krebs, Hermano I.; Ladenheim, Barbara; Hippolyte, Christopher; Monterroso, Linda; Mast, Joelle

    2009-01-01

    Our goal was to examine the feasibility of applying therapeutic robotics to children and adults with severe to moderate impairment due to cerebral palsy (CP). Pilot results demonstrated significant gains for both groups. These results suggest that robot-mediated therapy may be an effective tool to ameliorate the debilitating effects of CP and

  7. Language and Motor Speech Skills in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Pirila, Silja; van der Meere, Jaap; Pentikainen, Taina; Ruusu-Niemi, Pirjo; Korpela, Raija; Kilpinen, Jenni; Nieminen, Pirkko

    2007-01-01

    The aim of the study was to investigate associations between the severity of motor limitations, cognitive difficulties, language and motor speech problems in children with cerebral palsy. Also, the predictive power of neonatal cranial ultrasound findings on later outcome was investigated. For this purpose, 36 children (age range 1 year 10 months

  8. Systemic inflammation and cerebral palsy risk in extremely preterm infants.

    PubMed

    Kuban, Karl C K; O'Shea, T Michael; Allred, Elizabeth N; Paneth, Nigel; Hirtz, Deborah; Fichorova, Raina N; Leviton, Alan

    2014-12-01

    The authors hypothesized that among extremely preterm infants, elevated concentrations of inflammation-related proteins in neonatal blood are associated with cerebral palsy at 24 months. In 939 infants born before 28 weeks gestation, the authors measured blood concentrations of 25 proteins on postnatal days 1, 7, and 14 and evaluated associations between elevated protein concentrations and cerebral palsy diagnosis. Protein elevations within 3 days of birth were not associated with cerebral palsy. Elevations of tumor necrosis factor-?, tumor necrosis factor-?-receptor-1, interleukin-8, and intercellular adhesion molecule-1 on at least 2 days were associated with diparesis. Recurrent-persistent elevations of interleukin-6, E-selectin, or insulin-like growth factor binding protein-1 were associated with hemiparesis. Diparesis and hemiparesis were more likely among infants who had at least 4 of 9 protein elevations that previously have been associated with cognitive impairment and microcephaly. Repeated elevations of inflammation-related proteins during the first 2 postnatal weeks are associated with increased risk of cerebral palsy. PMID:24646503

  9. Cerebral Palsy and Communication--What Parents Can Do.

    ERIC Educational Resources Information Center

    Golbin, Arlene, Ed.

    Intended for parents of cerebral palsied children, the manual discusses special communication problems that often accompany the condition, and describes various strategies for helping such children communicate. A chapter on positioning for speech diagrams 14 different positions to help facilitate better functioning in many areas, including speech.…

  10. Reproducibility of Tactile Assessments for Children with Unilateral Cerebral Palsy

    ERIC Educational Resources Information Center

    Auld, Megan Louise; Ware, Robert S.; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

    2012-01-01

    A systematic review identified tactile assessments used in children with cerebral palsy (CP), but their reproducibility is unknown. Sixteen children with unilateral CP and 31 typically developing children (TDC) were assessed 2-4 weeks apart. Test-retest percent agreements within one point for children with unilateral CP (and TDC) were…

  11. Aerobic Capacity in Children and Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Verschuren, Olaf; Takken, Tim

    2010-01-01

    This study described the aerobic capacity [VO[subscript 2peak] (ml/kg/min)] in contemporary children and adolescents with cerebral palsy (CP) using a maximal exercise test protocol. Twenty-four children and adolescents with CP classified at Gross Motor Functional Classification Scale (GMFCS) level I or level II and 336 typically developing…

  12. Multiple concomitant cranial nerve palsies secondary to preeclampsia.

    PubMed

    Gilca, Marina; Luneau, Katie

    2015-06-01

    A 32-year-old primigravid woman developed pre-eclampsia after delivery of twins along with left fifth, sixth, and seventh cranial neuropathies. She also had evidence of hepatic and renal involvement. Results of patient evaluation were otherwise unremarkable, and the palsies completely resolved over 3 months after treatment with valacyclovir and systemic corticosteroids. PMID:25768245

  13. Rating Scales for Dystonia in Cerebral Palsy: Reliability and Validity

    ERIC Educational Resources Information Center

    Monbaliu, E.; Ortibus, E.; Roelens, F.; Desloovere, K.; Deklerck, J.; Prinzie, P.; De Cock, P.; Feys, H.

    2010-01-01

    Aim: This study investigated the reliability and validity of the Barry-Albright Dystonia Scale (BADS), the Burke-Fahn-Marsden Movement Scale (BFMMS), and the Unified Dystonia Rating Scale (UDRS) in patients with bilateral dystonic cerebral palsy (CP). Method: Three raters independently scored videotapes of 10 patients (five males, five females;…

  14. Maternal Compensatory Factors for Infants with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Hrncir, Elizabeth J.; And Others

    Viewed from an ecological perspective, maternal sense of competence and social support would be expected to influence a mother's spontaneous interactions with her infant. In this study 29 mother-infant dyads were visited in their homes when the infants with cerebral palsy were 18 months old. Infants' solitary spontaneous play and mother-infant

  15. Operant Control of Pathological Tongue Thrust in Spastic Cerebral Palsy.

    ERIC Educational Resources Information Center

    Thompson, George A., Jr.

    1979-01-01

    The behavior modification procedure, carried out at mealtime with a ten-year-old retarded boy who had spastic cerebral palsy, consisted of differential reinforcement and punishment, and resulted in substantial decreases in tongue thrust (reverse swallowing) and food expulsion, and a large increase in observed chewing. (Author/DLS)

  16. Cerebral Palsy Symptoms in Children Decreased Following Massage Therapy

    ERIC Educational Resources Information Center

    Hernandez-Reif, Maria; Field, Tiffany; Largie, Shay; Diego, Miguel; Manigat, Natasha; Seoanes, Jacqueline; Bornstein, Joan

    2005-01-01

    Twenty young children (mean age = 32 months) with cerebral palsy (CP) recruited from early intervention programs received 30 minutes of massage or reading twice weekly for 12 weeks. The children receiving massage therapy showed fewer physical symptoms including reduced spasticity, less rigid muscle tone overall and in the arms, and improved fine

  17. Growth and Nutrition Disorders in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Kuperminc, Michelle N.; Stevenson, Richard D.

    2008-01-01

    Growth and nutrition disorders are common secondary health conditions in children with cerebral palsy (CP). Poor growth and malnutrition in CP merit study because of their impact on health, including psychological and physiological function, healthcare utilization, societal participation, motor function, and survival. Understanding the etiology of…

  18. Rehabilitation of Bells' palsy from a multi-team perspective.

    PubMed

    Hultcrantz, Malou

    2016-04-01

    Conclusions Defectively healed facial paralysis causes difficulties to talk and eat, involuntary spasms (synkinesis), and cosmetic deformities which can give rise both to severe psychological and physical trauma. A team consisting of Ear-Nose-Throat specialists, Plastic surgeons and Physiotherapists can offer better care, treatment and outcome for patients suffering from Bells' palsy. Objectives Patients suffering from Bells' palsy from all ENT hospitals in Sweden and the University Hospital in Helsinki has been included. Methods Results have been drawn and statistically processed for different outcomes from a prospective, double blind cross over study. Results from a pilot surgical study and therapeutic results from physiotherapy studies have been included. Ideas concerning different kinds of surgery will be reviewed and the role of physiotherapy discussed. Results According to common results, treatment with Prednisolone enhances the recovery rate and should, if possible, be used early in the course. Sunnybrook grading at 1 month after onset most accurately predicts non-recovery at 12 months in Bells' palsy and a risk factor curve will be presented in order to predict outcome and selection of patients for undergoing facial surgery. This report is focusing on how to handle patients with Bells' palsy from a multi-rehabilitation team point of view, and what will be recommended to provide these patients with the best clinical and surgical help. PMID:26634395

  19. The Determinants of Daily Function in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Tseng, Mei-Hui; Chen, Kuan-Lin; Shieh, Jeng-Yi; Lu, Lu; Huang, Chien-Yu

    2011-01-01

    The aim of this study was to identify determinants of daily function in a population-based sample of children with cerebral palsy (CP). The study took into consideration factors from the entire scope of the International Classification of Functioning, Disability, and Health (ICF). Furthermore, the determinants of daily function were examined from…

  20. Bilateral Abducent Nerve Palsy After Neck Trauma: A Case Report

    PubMed Central

    Aminiahidashti, Hamed; Shafiee, Sajad; Sazegar, Mohammad; Nosrati, Nazanin

    2016-01-01

    Introduction The abducent nucleus is located in the upper part of the rhomboid fossa beneath the fourth ventricle in the caudal portion of the pons. The abducent nerve courses from its nucleus, to innervate the lateral rectus muscle. This nerve has the longest subarachnoid course of all the cranial nerves, it is the cranial nerve most vulnerable to trauma. It has been reported that 1% to 2.7% of all head injuries are followed by unilateral abducent palsy, but bilateral abducent nerve palsy is extremely rare. Case Presentation A 65-year-old woman presented to the emergency department following a motor vehicle accident. A neurological assessment showed the patient’s Glascow coma scale (GCS) to be 15. She complained of double vision, and we found lateral gaze palsy in both eyes. A hangman fracture type IIA (C2 fracture with posterior ligamentous C1 - C2 distraction) was found on the cervical CT scan. A three-month follow-up of the patient showed complete recovery of the abducent nerve. Conclusions Conservative treatment is usually recommended for traumatic bilateral abducent nerve palsy. Our patient recovered from this condition after three months without any remaining neurological deficit, a very rare outcome in a rare case. PMID:27218062

  1. Nutritional Assessment of the Young Child with Cerebral Palsy.

    ERIC Educational Resources Information Center

    Fee, Maureen A.; And Others

    1988-01-01

    Children with cerebral palsy sometimes display nutritional inadequacy, as evaluated through anthropometric measurements and laboratory values. Causes of poor nutritional status include inadequate calories offered or adequate calories offered but not consumed. Inadequate caloric retention may be due to vomiting, rumination, or gastroesophageal…

  2. Stability of Motor Impairment in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Shevell, Michael I.; Majnemer, Annette; Poulin, Chantal; Law, Mary

    2008-01-01

    Classification of the quality and topographical pattern of motor impairment is used to describe cerebral palsy (CP). As an adjunct to a study characterizing the quality of life and participation of school-age children with CP, initial and follow-up classification of CP were compared. A cohort of 93 children (58 males, 35 females) were initially…

  3. Childhood Educational Experiences of Women with Cerebral Palsy

    ERIC Educational Resources Information Center

    Freeborn, Donna; Mandleco, Barbara

    2010-01-01

    The purpose of this study was to examine the childhood experiences of women with cerebral palsy (CP), from the perspectives of these women. Using the feminist biographical method, eight women with CP participated in two in-depth interviews. Participants ranged in age from 22 to 55 years and had moderate to severe athetoid or spastic CP. Four

  4. Lyme borreliosis as a cause of facial palsy during pregnancy.

    PubMed

    Grandsaerd, M J; Meulenbroeks, A A

    2000-07-01

    The medical history of a pregnant woman in whom the initial pattern of complaints suggested hyperemesis gravidarum is described. After about 18 days the patient developed left facial palsy. Repeated tests eventually confirmed the diagnosis of neuroborreliosis. The problems concerning diagnostics, therapy and the possible complications of Lyme borreliosis during gestation are described. PMID:10817889

  5. Double Elevator Palsy, Subtypes and Outcomes of Surgery

    PubMed Central

    Bagheri, Abbas; Sahebghalam, Ramin; Abrishami, Mohammad

    2008-01-01

    Purpose To describe the clinical manifestations of subtypes of double elevator palsy and to report the outcomes of surgery in these patients. Methods This retrospective study was conducted on hospital records of patients with double elevator palsy at Labbafinejad Medical Center over a ten-year period from 1994 to 2004. Patients were classified into three subgroups of primary elevator muscle palsy (9 subjects), primary supranuclear palsy with secondary inferior rectus restriction (4 subjects) and pure inferior rectus restriction (7 subjects) according to forced duction test (FDT), force generation test (FGT) and Bell’s reflex. Patients in the first group underwent Knapp procedure, the second group received Knapp procedure and inferior rectus recession simultaneously and in the third group vertical recess-resect or mere inferior rectus recess operation was performed. Success was defined as final residual deviation ≤5 PD and ≥25% improvement in restriction after all operations. Results Overall 20 subjects including 10 male and 10 female patients with mean age of 12.6±9.3 (range 1.5–32) years were operated during the mentioned period which included 9 cases of primary elevator muscle palsy, 4 patients with primary supranuclear palsy and secondary inferior rectus restriction, and 7 subjects with pure inferior rectus restriction. Mean follow-up was 22.0±20.0 (range 3–63.5) months. Mean pre and postoperative deviation was 32.0±8.0 PD and 3.8±8.0 PD (P<0.001) respectively, and mean restriction before and after the operation(s) was −3.5±0.7 and −2.3±1.2 (P<0.001), respectively. Success rate was 77% for correction of deviation and 80% for improvement in muscle restriction. Conclusion Surgery for double elevator palsy must be individualized according to FDT, FGT and Bell’s reflex. The outcomes are favorable with appropriate surgical planning. PMID:23479532

  6. Hallmarks in the history of cerebral palsy: from antiquity to mid-20th century.

    PubMed

    Panteliadis, Christos; Panteliadis, Panos; Vassilyadi, Frank

    2013-04-01

    Cerebral palsy (CP) is a term that has been applied over the years to a group of children with motor disability and related service requirements. The first conceptions of cerebral palsy and our knowledge about aetiology and pathogeny allow us to assume that cerebral palsy existed in the Ancient World. Although there is lack of detailed medical descriptions from before the 19th century, mentions to cerebral palsy can be found in representational art, literary sources and paleopathology; however, because of the poor medical documentation, the diagnosis of cerebral palsy must remain a more or less well-justified supposition. In the Ancient World, the first medical description of cerebral palsy was made by Hippocrates in his work "Corpus Hippocraticum". Concrete examples and definitions of cerebral palsy, however, did not emerge until the early 19th century with observations by William John Little; thus, Little was the first personality to intensely engage cerebral palsy. Towards the end of the 19th century, two more personalities emerged, adding to the historical hallmarks of cerebral palsy: William Osler and Sigmund Freud. The significant developments that have followed since then are all due to the contributions of these three personalities in the field of cerebral palsy. PMID:22658818

  7. Neuro-ophthalmological approach to facial nerve palsy

    PubMed Central

    Portelinha, Joana; Passarinho, Maria Picoto; Costa, João Marques

    2014-01-01

    Facial nerve palsy is associated with significant morbidity and can have different etiologies. The most common causes are Bell’s palsy, Ramsay–Hunt syndrome and trauma, including surgical trauma. Incidence varies between 17 and 35 cases per 100,000. Initial evaluation should include accurate clinical history, followed by a comprehensive investigation of the head and neck, including ophthalmological, otological, oral and neurological examination, to exclude secondary causes. Routine laboratory testing and diagnostic imaging is not indicated in patients with new-onset Bell’s palsy, but should be performed in patients with risk factors, atypical cases or in any case without resolution within 4 months. Many factors are involved in determining the appropriate treatment of these patients: the underlying cause, expected duration of nerve dysfunction, anatomical manifestations, severity of symptoms and objective clinical findings. Systemic steroids should be offered to patients with new-onset Bell’s palsy to increase the chance of facial nerve recovery and reduce synkinesis. Ophthalmologists play a pivotal role in the multidisciplinary team involved in the evaluation and rehabilitation of these patients. In the acute phase, the main priority should be to ensure adequate corneal protection. Treatment depends on the degree of nerve lesion and on the risk of the corneal damage based on the amount of lagophthalmos, the quality of Bell’s phenomenon, the presence or absence of corneal sensitivity and the degree of lid retraction. The main therapy is intensive lubrication. Other treatments include: taping the eyelid overnight, botulinum toxin injection, tarsorrhaphy, eyelid weight implants, scleral contact lenses and palpebral spring. Once the cornea is protected, longer term planning for eyelid and facial rehabilitation may take place. Spontaneous complete recovery of Bell’s palsy occurs in up to 70% of cases. Long-term complications include aberrant regeneration with synkinesis. FNP after acoustic neuroma surgery remains the most common indication for FN rehabilitation. PMID:25859138

  8. Use of Sensorimotor Functions for Early Identification and Neurohabilitation of Infants with Cerebral Palsy and/or Cerebral Palsy Precursors.

    ERIC Educational Resources Information Center

    Covey, Thomas J.

    1997-01-01

    Presents the use of six sensorimotor functions (SMF) as a screening test for cerebral palsy in neonates. Functions include sitting in air, self-pulling to sit, self-propelling Katona slide crawl, assisted crawling, and elementary walking. Nine case examples are provided in an appendix. (Author/CR)

  9. Neuropathic scapuloperoneal syndrome (Davidenkow's syndrome) with chromosome 17p11.2 deletion.

    PubMed

    Verma, Ashok

    2005-11-01

    The nosologic boundary of neuropathic scapuloperoneal syndrome (Davidenkow's syndrome) remains ill defined and its genetic basis is unknown. A case of Davidenkow's syndrome with the monochromosomic 17p11.2 deletion that often is associated with hereditary neuropathy with liability to pressure palsies (HNPP) is described. The other allele at chromosome 17p11.2 locus was of normal length, and direct sequencing of the coding region of the peripheral nerve protein-22 gene in this allele revealed no additional mutation. The deleted allele in the proband was inherited from the paternal line in which the affected members had a late onset Charcot-Marie-Tooth type 1 clinical phenotype. This observation suggests that the rare Davidenkow's syndrome is clinically related to HNPP and its genotype could be a chromosome 17p11.2 deletion. PMID:16007675

  10. Operative treatment for spinal deformities in cerebral palsy.

    PubMed

    Hasler, Carol C

    2013-11-01

    The higher the functional impairment, the more likely patients with cerebral palsy (cP) are to develop a scoliotic deformity. This is usually long-sweeping, C-shaped, and progressive in nature, since the causes of the deformity, such as muscular weakness, imbalance, and osteoporosis, persist through adulthood. In contrast to idiopathic scoliosis, not only is the spine deformed, the patient is also sick. This multimorbidity warrants a multidisciplinary approach with close involvement of the caregivers from the beginning. Brace treatment is usually ineffective or intolerable in light of the mostly stiff and severe deformities and the poor nutritional status. The pros and cons of surgical correction need to weighed up when pelvic obliquity, subsequent loss of sitting balance, pressure sores, and pain due to impingement of the rib cage on the ileum become issues. General risks of, for example, pulmonary or urogenital infections, pulmonary failure, the need for a tracheostoma, permanent home ventilation, and death add to the particular surgery-related hazards, such as excessive bleeding, surgical site infections, pseudarthrosis, implant failure, and dural tears with leakage of cerebrospinal fluid. The overall complication rate averages around 25 %. From an orthopedic perspective, stiffness, marked deformities including sagittal profile disturbances and pelvic obliquity, as well as osteoporosis are the main challenges. In nonambulatory patients, long fusions from T2/T3 with forces distributed over all segments, low-profile anchors in areas of poor soft tissue coverage (sublaminar bands, wires), and strong lumbosacropelvic modern screw fixation in combination with meticulous fusion techniques (facetectomies, laminar decortication, use of local autologous bone) and hemostasis can be employed to keep the rate of surgical and implant-related complications at an acceptably low level. Excessive posterior release techniques, osteotomies, or even vertebrectomies in cases of very severe short-angled deformity mostly prevent anterior one- or two-stage releases. Despite improved operative techniques and implants with predictable and satisfactory deformity corrections, the comorbidities and quality-of-life related issues demand a thorough preoperative, multidisciplinary decision-making process that takes ethical and economic aspects into consideration. PMID:24432105

  11. The Cerebral Palsy Research Registry: Development and Progress Toward National Collaboration in the United States

    PubMed Central

    Hurley, Donna S.; Sukal-Moulton, Theresa; Msall, Michael E.; Gaebler-Spira, Deborah; Krosschell, Kristin J.; Dewald, Julius P.

    2011-01-01

    Cerebral palsy is the most common neurodevelopmental motor disability in children. The condition requires medical, educational, social, and rehabilitative resources throughout the life span. Several countries have developed population-based registries that serve the purpose of prospective longitudinal collection of etiologic, demographic, and functional severity. The United States has not created a comprehensive program to develop such a registry. Barriers have been large population size, poor interinstitution collaboration, and decentralized medical and social systems. The Cerebral Palsy Research Registry was created to fill the gap between population and clinical-based cerebral palsy registries and promote research in the field. This is accomplished by connecting persons with cerebral palsy, as well as their families, to a network of regional researchers. This article describes the development of an expandable cerebral palsy research registry, its current status, and the potential it has to affect families and persons with cerebral palsy in the United States and abroad. PMID:21677201

  12. Pupil-sparing third nerve palsies and hemiataxia: Claude's and reverse Claude's syndrome.

    PubMed

    Bateman, James R; Murty, Pavan; Forbes, Michael; Collier, Kisha Young; Tememe, Danoushka; Marchena, Octavio de; Powers, William J

    2016-06-01

    We report two patients with midbrain infarction with pupil-sparing third nerve palsies and hemiataxia: one with contralateral ataxia (Claude's syndrome) and one with ipsilateral ataxia (which we refer to as reverse Claude's syndrome). We highlight the importance of a thorough neurologic evaluation with partial oculomotor palsies and describe, to our knowledge, the fourth account in the literature of a pupil-sparing third nerve palsy with ipsilateral cerebellar ataxia. PMID:26883351

  13. Delayed Bilateral C5 Palsy following Circumferential Decompression and Fusion in Patient with Cervical Spondylotic Myelopathy

    PubMed Central

    Jeon, Hyeong-Seok

    2015-01-01

    C5 palsy is a common complication after cervical decompressive surgery, which have 0 to 30% complication rate. A 61-year-old female patient with cervical spondylotic myelopathy showed bilateral C5 palsy following circumferential decompression and fusion. Unexpectedly, bilateral C5 palsy was noted in different time points on postoperative day 2 and 8, respectively. Steroid injection and physical therapy were performed, and her motor function is recovering. Surgeons should make an effort to prevent possible C5 palsy when performing cervical decompression surgery. PMID:26512284

  14. Digital capture, design, and manufacturing of an extraoral device for a clarinet player with Bell's palsy.

    PubMed

    Aita-Holmes, Cynthia; Liacouras, Peter; Wilson, William O; Grant, Gerald T

    2015-08-01

    An extraoral device was fabricated to assist a clarinet player with Bell's palsy. The device was fabricated by using stereophotogrammetry, digital design, and additive manufacturing technologies. PMID:25985740

  15. Sixth cranial nerve palsy caused by compression from a dolichoectatic vertebral artery.

    PubMed

    Zhu, Ying; Thulborn, Keith; Curnyn, Kimberlee; Goodwin, James

    2005-06-01

    A 68-year-old man had an unremitting left sixth cranial nerve palsy immediately after completing a long bicycle trip. High-resolution (3 Tesla) magnetic resonance imaging disclosed a dolichoectatic vertebral artery that compressed the left sixth cranial nerve against the belly of the pons at its root exit zone. It was postulated that increased blood flow in the vessel during the unusually prolonged aerobic exercise precipitated the palsy. Compressive palsies of cranial nerves caused by a dolichoectatic basilar artery have often been documented; compressive palsy caused by a dolichoectatic vertebral artery is less well-recognized. PMID:15937439

  16. [Spontaneous dissection of the internal carotid artery: description of a case with lower cranial nerve palsy].

    PubMed

    Macarini, Luca; Zeppa, Pio; Genovese, Eugenio Annibale; Scialpi, Michele; Raucci, Antonio

    2012-11-01

    Spontaneous dissection of the extracranial internal carotid artery is a well recognized cause of headache and juvenile stroke; lower cranial nerve palsy as a complication of dissection is rare. We report the case of a female patient with bilateral dissecting aneurysm of the internal carotid artery, associated with unilateral cranial nerve XII palsy and oculosympathetic palsy. Neuroradiological findings, in particular those obtained by Magnetic Resonance imaging, allow the identification of the dissecting pathology and the correlation of the aneurysmal formation with nerve palsy. PMID:23096747

  17. Complete Oculomotor Nerve Palsy Caused by Direct Compression of the Posterior Cerebral Artery.

    PubMed

    Jo, Yoon-Sik; Kim, Shin Kyoung; Kim, Dae Ho; Kim, Jae-Hwan; Na, Sang-Jun

    2015-07-01

    Oculomotor nerve palsy frequently occurs because of external compression by an internal carotid-posterior communicating artery aneurysm and diabetes mellitus. In addition, pontine infarction, cavernous sinus tumors, demyelinating disease, and autoimmune disorder are well-known causes of oculomotor nerve palsy. However, cases of complete oculomotor nerve palsy by neurovascular conflicts presented with a sudden onset of clinical symptoms are extremely rare. We experienced a rare case of complete oculomotor nerve palsy because of direct vascular compression of the oculomotor nerve by the posterior cerebral artery. PMID:25939862

  18. Relationship between static postural control and the level of functional abilities in children with cerebral palsy

    PubMed Central

    Pavão, Sílvia L.; Nunes, Gabriela S.; Santos, Adriana N.; Rocha, Nelci A. C. F.

    2014-01-01

    Background: Postural control deficits can impair functional performance in children with cerebral palsy (CP) in daily living activities. Objective: To verify the relationship between standing static postural control and the functional ability level in children with CP. Method: The postural control of 10 children with CP (gross motor function levels I and II) was evaluated during static standing on a force platform for 30 seconds. The analyzed variables were the anteroposterior (AP) and mediolateral (ML) displacement of the center of pressure (CoP) and the area and velocity of the CoP oscillation. The functional abilities were evaluated using the mean Pediatric Evaluation of Disability Inventory (PEDI) scores, which evaluated self-care, mobility and social function in the domains of functional abilities and caregiver assistance. Results: Spearman's correlation test found a relationship between postural control and functional abilities. The results showed a strong negative correlation between the variables of ML displacement of CoP, the area and velocity of the CoP oscillation and the PEDI scores in the self-care and caregiver assistance domains. Additionally, a moderate negative correlation was found between the area of the CoP oscillation and the mobility scores in the caregiver assistance domain. We used a significance level of 5% (p <0.05). Conclusions: We observed that children with cerebral palsy with high CoP oscillation values had lower caregiver assistance scores for activities of daily living (ADL) and consequently higher levels of caregiver dependence. These results demonstrate the repercussions of impairments to the body structure and function in terms of the activity levels of children with CP such that postural control impairments in these children lead to higher requirements for caregiver assistance. PMID:25054383

  19. Trends in communicative access solutions for children with cerebral palsy.

    PubMed

    Myrden, Andrew; Schudlo, Larissa; Weyand, Sabine; Zeyl, Timothy; Chau, Tom

    2014-08-01

    Access solutions may facilitate communication in children with limited functional speech and motor control. This study reviews current trends in access solution development for children with cerebral palsy, with particular emphasis on the access technology that harnesses a control signal from the user (eg, movement or physiological change) and the output device (eg, augmentative and alternative communication system) whose behavior is modulated by the user's control signal. Access technologies have advanced from simple mechanical switches to machine vision (eg, eye-gaze trackers), inertial sensing, and emerging physiological interfaces that require minimal physical effort. Similarly, output devices have evolved from bulky, dedicated hardware with limited configurability, to platform-agnostic, highly personalized mobile applications. Emerging case studies encourage the consideration of access technology for all nonverbal children with cerebral palsy with at least nascent contingency awareness. However, establishing robust evidence of the effectiveness of the aforementioned advances will require more expansive studies. PMID:24820337

  20. Cerebral Palsy: A Lifelong Challenge Asks for Early Intervention

    PubMed Central

    Panteliadis, Christos P; Hagel, Christian; Karch, Dieter; Heinemann, Karl

    2015-01-01

    One of the oldest and probably well-known examples of cerebral palsy is the mummy of the Pharaoh Siptah about 1196–1190 B.C., and a letter from Hippocrates (460–390 B.C.). Cerebral palsy (CP) is one of the most common congenital or acquired neurological impairments in paediatric patients, and refers to a group of children with motor disability and related functional defects. The visible core of CP is characterized by abnormal coordination of movements and/or muscle tone which manifest very early in the development. Resulting from pre- or perinatal brain damage CP is not a progressive condition per se. However, without systematic medical and physiotherapeutic support the dystonia leads to muscle contractions and to deterioration of the handicap. Here we review the three general spastic manifestations of CP hemiplegia, diplegia and tetraplegia, describe the diagnostic procedures and delineate a time schedule for an early intervention. PMID:26191093

  1. [Etiologies of cerebral palsy and classical treatment possibilities].

    PubMed

    Maurer, Ute

    2002-01-01

    Cerebral palsy is a non-progressive disorder of the developing brain with different etiologies in the pre-, peri- or postnatal period. The most important of these diseases is cystic periventricular leukomalacia (PVL), followed by intra- and periventricular hemorrhage, hypoxic-ischemic encephalopathy, vascular disorders, infections or brain malformations. The underlying cause is always a damage of the first motor neuron. Prevalence of cerebral palsy in Europe is 2-3 per 1000 live births with a broad spectrum in different birth weight groups. Our own data concerning only pre-term infants in the NICU with birth weight below 1500 g (VLBW) are between 10%-20%. Established classical treatment methods include physiotherapy (Bobath, Vojta, Hippotherapy), methods of speech and occupational therapists (Castillo-Morales, Sensory Integration) and other therapeutical concepts (Petö, Affolter, Frostig). PMID:11862678

  2. Complete Spinal Accessory Nerve Palsy From Carrying Climbing Gear.

    PubMed

    Coulter, Jess M; Warme, Winston J

    2015-09-01

    We report an unusual case of spinal accessory nerve palsy sustained while transporting climbing gear. Spinal accessory nerve injury is commonly a result of iatrogenic surgical trauma during lymph node excision. This particular nerve is less frequently injured by blunt trauma. The case reported here results from compression of the spinal accessory nerve for a sustained period-that is, carrying a load over the shoulder using a single nylon rope for 2.5 hours. This highlights the importance of using proper load-carrying equipment to distribute weight over a greater surface area to avoid nerve compression in the posterior triangle of the neck. The signs and symptoms of spinal accessory nerve palsy and its etiology are discussed. This report is particularly relevant to individuals involved in mountaineering and rock climbing but can be extended to anyone carrying a load with a strap over one shoulder and across the body. PMID:25937552

  3. The changing pattern of cerebral palsy in Avon.

    PubMed

    MacGillivray, I; Campbell, D M

    1995-04-01

    The overall rate of cerebral palsy excluding post-neonatal cases, has remained relatively constant, varying between 1.93 and 2.27 per 1000 births over the 20-year period between 1969 and 1988 in Avon. The rate of the subgroup of spastic cerebral palsy varied from 1.40 to 1.78 per 1000 births over the same time period. In singletons the corresponding figures were 1.80-2.17 per 1000 births and 1.28-1.67 per 1000 births, and in multiple births the figures were 7.12-8.80 per 1000 births and 7.12-8.44 per 1000 births. Although the overall rates have remained fairly constant there was an increase in incidence in the later years among children with low birthweight and short gestation. PMID:7596892

  4. Treated and untreated unstable hips in severe cerebral palsy.

    PubMed

    Pritchett, J W

    1990-01-01

    One hundred patients with severe cerebral palsy (total body involvement) and dislocated hips were examined to determine their level of pain, sitting ability, pelvic obliquity, scoliosis, nursing care difficulties and complications of decubitus ulcers and fractures. 50 of the patients had undergone surgical procedures to treat the hip; 50 had received no treatment. No significant differences were found in the frequency of pain or other complications between the two groups. Nursing care difficulties and the ability to sit did not depend on the status of the hip. Pelvic obliquity and scoliosis were related to the severity of neurological damage rather than to hip stability. These findings suggest that surgical treatment of already dislocated hips of patients with severe cerebral palsy is not helpful. PMID:2105251

  5. Cognitive Profile in Young Icelandic Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Sigurdardottir, Solveig; Eiriksdottir, Audur; Gunnarsdottir, Eva; Meintema, Marrit; Arnadottir, Unnur; Vik, Torstein

    2008-01-01

    We describe the cognitive profile in a complete national cohort of children with cerebral palsy (CP). One hundred and twenty-seven Icelandic children (67 females, 60 males) with CP, born between 1985 and 2000 and assessed between the ages of 4 and 6 years 6 months (mean age 5y 5mo, SD 6mo), were included in the study. IQ was measured using the…

  6. Treatment of the spasticity in children with cerebral palsy.

    PubMed

    Meholjić-Fetahović, Ajsa

    2007-11-01

    Botulinum toxin is a natural purified protein and one of the strongest biological poisons--neurotoxin. It is produced by the bacterium Clostridium botulinum. Its medical usage started in USA in 1981 and in Europe in 1992. There are seven different immune types of the toxin: A, B, C1, D, E, F and G. Toxin types A and B are used to decrease muscular spasticity. Botulinum toxin prevents the formation of acetylcholine from cholinergic nerve tissues in muscles, which in the end irreversibly destroys neuromuscular synapses. It is called temporary local chemodenervation. It does not affect the synthesis of acetylcholine. As it affects neuromuscular bond it also affects one of the symptoms of cerebral palsy--spasticity. Decreasing the spasticity of children with cerebral palsy leads to the improvement of conscious movements, muscles are less toned, passive mobility is improved, orthosis tolerance is also improved, and the child is enabled to perform easier and better motor functions such as crawling, standing and walking. Since the action of Botulinum toxin is limited to 2-6 months, new neural collaterals are formed and neuromuscular conductivity is reestablished which in the end once again develops a muscular spasm. This leads to a conclusion that botulinum toxin should again be applied into spastic muscles. It is very important for good effect of Botulinum toxin to set the goals of the therapy in advance. The goals include improvement of a function, prevention of contractions and deformities, ease of care and decrease of pain for children with cerebral palsy. After application of botulinum toxin, it is necessary to perform adequate and intensive physical treatment with regular monitoring of effects. This work shows a case of a boy with spastic form of cerebral palsy. After being rehabilitated using Vojta therapy and Bobath concept and the conduct of certain physical procedures, botulinum toxin is administered into his lower limbs' muscles and kinesiotherapy is intensified. After the administration of botulinum toxin significant functional improvement is noted. PMID:18039197

  7. Major surgical procedures in children with cerebral palsy.

    PubMed

    Theroux, Mary C; DiCindio, Sabina

    2014-03-01

    There are 3 surgical procedures that patients with cerebral palsy (CP) undergo that may be considered major procedures: femoral osteotomies combined with pelvic osteotomies, spine fusion, and intrathecal baclofen pump implant for the treatment of spasticity. Many complications are known to occur at a higher rate in this population, and some may be avoided with prior awareness of the preoperative pathophysiology of the patient with CP. PMID:24491650

  8. Multiple cranial nerve palsy in an HIV-positive patient.

    PubMed

    Karna, S; Biswas, J; Kumarasamy, N; Sharma, P; Solomon, S

    2001-06-01

    We report the case of a 32 -year- old HIV-positive Indian male who presented with sixth nerve (bilateral), ninth, tenth and twelfth nerve palsies; cerebellar and posterior column involvement. CT scan showed gyriform enhancement in the right occipital lobe and nodular leptomeningeal enhancement in the left frontal lobe. Cytomegalovirus serology was positive and the patient was treated as presumed CMV. HIV can present with multiple cranial neuropathy and varied neurological involvement. PMID:15884518

  9. Grip Force Coordination during Bimanual Tasks in Unilateral Cerebral Palsy

    ERIC Educational Resources Information Center

    Islam, Mominul; Gordon, Andrew M.; Skold, Annika; Forssberg, Hans; Eliasson, Ann-Christin

    2011-01-01

    Aim: The aim of the study was to investigate coordination of fingertip forces during an asymmetrical bimanual task in children with unilateral cerebral palsy (CP). Method: Twelve participants (six males, six females; mean age 14y 4mo, SD 3.3y; range 9-20y;) with unilateral CP (eight right-sided, four left-sided) and 15 age-matched typically…

  10. Feeding and Gastrointestinal Problems in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Erkin, Gulten; Culha, Canan; Ozel, Sumru; Kirbiyik, Eylem Gulsen

    2010-01-01

    The aim of our study was to identify feeding and gastrointestinal system (GIS) problems in children with cerebral palsy (CP), and to evaluate the relationship between these problems and the severity of CP. A total of 120 children with CP were enrolled consecutively into the study (67 males, 53 females; mean age: 6.0[plus or minus]2.4 years; range:…

  11. A diagnostic approach for cerebral palsy in the genomic era.

    PubMed

    Lee, Ryan W; Poretti, Andrea; Cohen, Julie S; Levey, Eric; Gwynn, Hilary; Johnston, Michael V; Hoon, Alexander H; Fatemi, Ali

    2014-12-01

    An ongoing challenge in children presenting with motor delay/impairment early in life is to identify neurogenetic disorders with a clinical phenotype, which can be misdiagnosed as cerebral palsy (CP). To help distinguish patients in these two groups, conventional magnetic resonance imaging of the brain has been of great benefit in "unmasking" many of these genetic etiologies and has provided important clues to differential diagnosis in others. Recent advances in molecular genetics such as chromosomal microarray and next-generation sequencing have further revolutionized the understanding of etiology by more precisely classifying these disorders with a molecular cause. In this paper, we present a review of neurogenetic disorders masquerading as cerebral palsy evaluated at one institution. We have included representative case examples children presenting with dyskinetic, spastic, and ataxic phenotypes, with the intent to highlight the time-honored approach of using clinical tools of history and examination to focus the subsequent etiologic search with advanced neuroimaging modalities and molecular genetic tools. A precise diagnosis of these masqueraders and their differentiation from CP is important in terms of therapy, prognosis, and family counseling. In summary, this review serves as a continued call to remain vigilant for current and other to-be-discovered neurogenetic masqueraders of cerebral palsy, thereby optimizing care for patients and their families. PMID:25280894

  12. A Diagnostic Approach for Cerebral Palsy in the Genomic Era

    PubMed Central

    Lee, Ryan W.; Poretti, Andrea; Cohen, Julie S.; Levey, Eric; Gwynn, Hilary; Johnston, Michael V.; Hoon, Alexander H.; Fatemi, Ali

    2014-01-01

    An ongoing challenge in children presenting with motor delay/impairment early in life is to identify neurogenetic disorders with a clinical phenotype which can be misdiagnosed as cerebral palsy (CP). To help distinguish patients in these two groups, conventional magnetic resonance imaging (MRI) of the brain has been of great benefit in “unmasking” many of these genetic etiologies and has provided important clues to differential diagnosis in others. Recent advances in molecular genetics such as chromosomal microarray and next generation sequencing have further revolutionized the understanding of etiology by more precisely classifying these disorders with a molecular cause. In this paper, we present a review of neurogenetic disorders masquerading as cerebral palsy evaluated at one institution. We have included representative case examples children presenting with dyskinetic, spastic and ataxic phenotypes, with the intent to highlight the time honored approach of using clinical tools of history and examination to focus the subsequent etiologic search with advanced neuroimaging modalities and molecular genetic tools. A precise diagnosis of these masqueraders and their differentiation from CP is important in terms of therapy, prognosis, and family counseling. In summary, this review serves as a continued call to remain vigilant for current and other to-be-discovered neurogenetic masqueraders of cerebral palsy, thereby optimizing care for patients and their families. PMID:25280894

  13. Food pattern and nutritional status of children with cerebral palsy

    PubMed Central

    Lopes, Patrícia Ayrosa C.; Amancio, Olga Maria S.; Araújo, Roberta Faria C.; Vitalle, Maria Sylvia de S.; Braga, Josefina Aparecida P.

    2013-01-01

    OBJECTIVES To assess the food intake pattern and the nutritional status of children with cerebral palsy. METHODS Cross-sectional study with 90 children from two to 12.8 years with cerebral palsy in the following forms: hemiplegia, diplegia, and tetraplegia. Nutritional status was assessed by weight, height, and age data. Food intake was verified by the 24-hour recall and food frequency questionnaire. The ability to chew and/or swallowing, intestinal habits, and physical activity were also evaluated. RESULTS For 2-3 year-old age group, the mean energy intake followed the recommended range; in 4-6 year-old age group with hemiplegia and tetraplegia, energy intake was below the recommended limits. All children presented low intake of carbohydrates, adequate intake of proteins and high intake of lipids. The tetraplegia group had a higher prevalence of chewing (41%) and swallowing (12.8%) difficulties compared to 14.5 and 6.6% of children with hemiplegia, respectively. Most children of all groups had a daily intestinal habit. All children presented mild physical activity, while moderate activity was not practiced by any child of the tetraplegia group, which had a significantly lower height/age Z score than those with hemiplegia (-2.14 versus -1.05; p=0.003). CONCLUSIONS The children with cerebral palsy presented inadequate dietary pattern and impaired nutritional status, with special compromise of height. Tetraplegia imposes difficulties regarding chewing/swallowing and moderate physical activity practice. PMID:24142317

  14. Cerebral palsy--an increasing contributor to severe mental retardation?

    PubMed Central

    Nicholson, A; Alberman, E

    1992-01-01

    It is estimated that the prevalence of nongenetic SMR associated with cerebral palsy has risen from 0.7 to about 0.9 per 1000 live births in the last decade. This is due to the predicted rise in total cerebral palsy prevalence to 2.5/1000 live births. This predicted prevalence of cerebral palsy is similar to that given for Western Australia in 1979-82, allowing for postnatal causes, but is higher than prevalence data from England and Sweden for that period. The estimated rise is due largely to improved survival and increased proportion of low birthweight babies since 1983 and also reflects the use of prevalence rates based on Mersey data. Improvements in prenatal diagnosis, and a parental choice of selective termination may lead to reductions in other causes of SMR, such as Down's syndrome and neural tube defects, so it seems that children both physically and mentally handicapped due to brain damage will contribute a greater proportion of the SMR population. The careload of these children is greater than that associated with many other causes of SMR and most survive into adult life. The implications for planning future services will need to be recognised. PMID:1520012

  15. Rehabilitation of spasticity and related problems in childhood cerebral palsy.

    PubMed

    Flett, P J

    2003-01-01

    Spasticity is one part of the upper motor neuron syndrome, but it is a widespread problem in cerebral palsy; it is debilitating, affects function and can lead to musculoskeletal complications. Significant advances have occurred in antispasticity management (and related musculoskeletal problems) in children with cerebral palsy during the past 5-10 years. Botulinum toxin A has been the most outstanding treatment advance; it is relatively long-lasting, easy to administer, reversible, has a favourable side-effect profile and is highly useful for focal spasticity. There is an emerging role for intrathecal baclofen in Australia. Despite being available and practised in North America for years, selective dorsal rhizotomy has not been popular in Australia. The use of orthopaedic surgery has significantly altered in recent years. There is still a place for oral drug treatment, including some newer agents and the potential for combination treatment with other modalities. The role of physical therapy in defining disability, assessing function, undertaking biomechanical assessment and providing mobility aids/casting/orthoses and motor training/stretching exercises is critical for the success of medical and surgical interventions. From an Australian perspective, the purpose of the present review is to provide a critical review of therapies available for spasticity associated with childhood cerebral palsy. PMID:12542805

  16. Saccadic palsy following cardiac surgery: a review and new hypothesis.

    PubMed

    Eggers, Scott D Z; Horn, Anja K E; Roeber, Sigrun; Härtig, Wolfgang; Nair, Govind; Reich, Daniel S; Leigh, R John

    2015-04-01

    The ocular motor system provides several advantages for studying the brain, including well-defined populations of neurons that contribute to specific eye movements. Generation of rapid eye movements (saccades) depends on excitatory burst neurons (EBN) and omnipause neurons (OPN) within the brainstem, both types of cells are highly active. Experimental lesions of EBN and OPN cause slowing or complete loss of saccades. We report a patient who developed a permanent, selective saccadic palsy following cardiac surgery. When she died several years later, surprisingly, autopsy showed preservation of EBN and OPN. We therefore considered other mechanisms that could explain her saccadic palsy. Recent work has shown that both EBN and OPN are ensheathed by perineuronal nets (PN), which are specialized extracellular matrix structures that may help stabilize synaptic contacts, promote local ion homeostasis, or play a protective role in certain highly active neurons. Here, we review the possibility that damage to PN, rather than to the neurons they support, could lead to neuronal dysfunction-such as saccadic palsy. We also suggest how future studies could test this hypothesis, which may provide insights into the vulnerability of other active neurons in the nervous system that depend on PN. PMID:25721480

  17. Assessment of Abilities and Comorbidities in Children With Cerebral Palsy.

    PubMed

    Gabis, Lidia V; Tsubary, Netta Misgav; Leon, Odelia; Ashkenasi, Arie; Shefer, Shahar

    2015-10-01

    This study examines major comorbidities in children with severe cerebral palsy and the feasibility of psychological tests for measuring abilities in a more impaired population. Eighty psychological evaluations of children with cerebral palsy aged 1.8 to 15.4 years (mean = 5.6) were analyzed. Major comorbid disorders were correlated with severity of motor disability. More than half of the cohort were diagnosed with severe cerebral palsy according to the Gross Motor Function Classification System. Multiple subtests were combined in order to assess the intellectual level. Normal intelligence was found in 22.5%, and 41.3% had moderate or severe intellectual impairment. Epilepsy occurred in 32.5% and attention-deficit hyperactivity disorder (ADHD) in 22.5%. Intellectual disability correlated with motor ability and with epilepsy. In a logistic regression model, epilepsy and motor ability score predicted 29.9% of IQ score variance. Intellectual impairment and epilepsy are common comorbidities. Subtests from different scales should be applied and interpreted with caution. PMID:25855688

  18. Performance of Cerebral Palsied Children under Conditions of Reduced Auditory Input on Selected Intellectual, Cognitive and Perceptual Tasks.

    ERIC Educational Resources Information Center

    Fassler, Joan

    The study investigated the task performance of cerebral palsied children under conditions of reduced auditory input and under normal auditory conditions. A non-cerebral palsied group was studied in a similar manner. Results indicated that cerebral palsied children showed some positive change in performance, under conditions of reduced auditory…

  19. Position as a Cause of Deformity in Children with Cerebral Palsy (1976)

    ERIC Educational Resources Information Center

    Scrutton, David

    2008-01-01

    Deformities in the child with cerebral palsy have been ascribed to muscle imbalance (Sharrard 1961) and increased tone (Pollock 1959) or to the type of cerebral palsy (Bobath and Bobath 1975). As far as we know, the position in which the child is nursed, especially during the first year of life, has not been considered as a cause of deformity. It…

  20. Effect of Translucency on Transparency and Symbol Learning for Children with and without Cerebral Palsy

    ERIC Educational Resources Information Center

    Huang, Chih-Hsiung; Chen, Ming-Chung

    2011-01-01

    Based on the concept of iconicity, the iconicity hypothesis was emphasized for decades. The aims of this study were to explore the effect of translucency on transparency and symbol learning for children with and without cerebral palsy. Twenty children with cerebral palsy and forty typical peers participated in the study. Ten symbols with high…

  1. Theory of Mind and Irony Comprehension in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Caillies, Stephanie; Hody, Anais; Calmus, Arnaud

    2012-01-01

    The main goal of the present study was to characterise the pragmatic abilities of French children with cerebral palsy through their understanding of irony and other people's mental states. We predicted that children with cerebral palsy would have difficulty understanding false-belief and ironic remarks, due to the executive dysfunction that…

  2. Communicating about Loss: Experiences of Older Australian Adults with Cerebral Palsy and Complex Communication Needs

    ERIC Educational Resources Information Center

    Dark, Leigha; Balandin, Susan; Clemson, Lindy

    2011-01-01

    Loss and grief is a universal human experience, yet little is known about how older adults with a lifelong disability, such as cerebral palsy, and complex communication needs (CCN) experience loss and manage the grieving process. In-depth interviews were conducted with 20 Australian participants with cerebral palsy and CCN to determine the types…

  3. Understanding Mealtime Changes for Adults with Cerebral Palsy and the Implications for Support Services

    ERIC Educational Resources Information Center

    Balandin, Susan; Hemsley, Bronwyn; Hanley, Leah; Sheppard, Justine Joan

    2009-01-01

    Background: Changes in the swallowing capabilities of adults with cerebral palsy as they age may impact on their health, safety, and well-being. Method: Thirty-two adults with cerebral palsy aged between 30 and 69 years participated in in-depth interviews about their experiences of changes in their swallowing and related management of their…

  4. "I Do Lots of Things": Children with Cerebral Palsy's Competence for Everyday Activities

    ERIC Educational Resources Information Center

    Kramer, Jessica M.; Hammel, Joy

    2011-01-01

    This study explored how children with cerebral palsy describe competent performance in everyday activities and sought to better understand the processes by which the children developed competence. Five children with cerebral palsy aged six to 17 years participated in a three-step procedure that included two observations, one semi-structured…

  5. Arithmetic Difficulties in Children with Cerebral Palsy Are Related to Executive Function and Working Memory

    ERIC Educational Resources Information Center

    Jenks, Kathleen M.; de Moor, Jan; van Lieshout, Ernest C. D. M.

    2009-01-01

    Background: Although it is believed that children with cerebral palsy are at high risk for learning difficulties and arithmetic difficulties in particular, few studies have investigated this issue. Methods: Arithmetic ability was longitudinally assessed in children with cerebral palsy in special (n = 41) and mainstream education (n = 16) and…

  6. Treadmill Training in a Child with Cerebral Palsy: A Case Report

    ERIC Educational Resources Information Center

    Crowley, Julie P.; Arnold, Sandra H.; McEwen, Irene R.; James, Shirley

    2009-01-01

    This case report describes the use of treadmill training without body weight support to improve walking speed in a child with diplegic cerebral palsy. The child was a six-year-old girl with spastic diplegic cerebral palsy. She walked short distances independently using a posterior support walker but was unable to keep up with her peers walking…

  7. Effects of Frequency of Feedback on the Learning of Motor Skill in Individuals with Cerebral Palsy

    ERIC Educational Resources Information Center

    Hemayattalab, Rasool; Rostami, Leila Rashidi

    2010-01-01

    The purpose of this study was to investigate the effect of frequency of knowledge of results (KR) on the learning of dart in individuals with cerebral palsy type I. Twenty-four individuals with cerebral palsy (CP) between the ages of 5 and 17 were chosen for this study. They were put into 3 homogenous groups according to their records after 20…

  8. The Relationship between Quality of Life and Functioning for Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Shelly, A.; Davis, E.; Waters, E.; Mackinnon, A.; Reddihough, D.; Boyd, R.; Reid, S.; Graham, H. K.

    2008-01-01

    Given that quality of life (QOL) is commonly confused with functioning, the aim of this study was to examine the association between functioning and QOL domains for children with cerebral palsy (CP). Two hundred and five parents of children aged 4 to 12 years with CP and 53 children aged 9 to 12 years with CP, completed the Cerebral Palsy Quality…

  9. Growth of an NGO: The Indian Institute of Cerebral Palsy from 1974 to 2006

    ERIC Educational Resources Information Center

    Sen, Reena; Goldbart, Juliet; Kaul, Sudha

    2008-01-01

    The Indian Institute for Cerebral Palsy (IICP) has been providing education and other services in Kolkata, Eastern India, for children and young adults with cerebral palsy and related disabilities for over 30 years. IICP started by providing education for just two children, and was highly dependent on western expertise. Described is the history of…

  10. Electropalatography in the Description and Treatment of Speech Disorders in Five Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Nordberg, Ann; Carlsson, Goran; Lohmander, Anette

    2011-01-01

    Some children with cerebral palsy have articulation disorders that are resistant to conventional speech therapy. The aim of this study was to investigate whether the visual feedback method of electropalatography (EPG) could be an effective tool for treating five children (mean age of 9.4 years) with dysarthria and cerebral palsy and to explore

  11. Psychological Problems in Children with Cerebral Palsy: A Cross-Sectional European Study

    ERIC Educational Resources Information Center

    Parkes, Jackie; White-Koning, Melanie; Dickinson, Heather O.; Thyen, Ute; Arnaud, Catherine; Beckung, Eva; Fauconnier, Jerome; Marcelli, Marco; McManus, Vicki; Michelsen, Susan I.; Parkinson, Kathryn; Colver, Allan

    2008-01-01

    Objectives: To describe psychological symptoms in 8-12-year-old children with cerebral palsy; to investigate predictors of these symptoms and their impact on the child and family. Design: A cross-sectional multi-centre survey. Participants: Eight hundred and eighteen children with cerebral palsy, aged 8-12 years, identified from population-based

  12. Bell's Palsy in Children: Role of the School Nurse in Early Recognition and Referral

    ERIC Educational Resources Information Center

    Gordon, Shirley C.

    2008-01-01

    Bell's palsy is the most common condition affecting facial nerves. It is an acute, rapidly progressing, idiopathic, unilateral facial paralysis that is generally self-limiting and non-life threatening that occurs in all age groups (Okuwobi, Omole, & Griffith, 2003). The school nurse may be the first person to assess facial palsy and muscle

  13. Position as a Cause of Deformity in Children with Cerebral Palsy (1976)

    ERIC Educational Resources Information Center

    Scrutton, David

    2008-01-01

    Deformities in the child with cerebral palsy have been ascribed to muscle imbalance (Sharrard 1961) and increased tone (Pollock 1959) or to the type of cerebral palsy (Bobath and Bobath 1975). As far as we know, the position in which the child is nursed, especially during the first year of life, has not been considered as a cause of deformity. It

  14. Microcephaly and Vulnerability of the Brain in the Etiology of Cerebral Palsy

    PubMed Central

    Whittaker, John S.

    1976-01-01

    A review of data showed that of 100 children referred with a diagnosis or suspected diagnosis of cerebral palsy (CP), 37 children had microcephaly, defined as a head with a circumference of two standard deviations (SD) or more below the mean for age and sex. Is such a finding of any significance in the etiology of cerebral palsy? PMID:21308072

  15. Australia and the Australian Cerebral Palsy Register for the birth cohort 1993 to 2006.

    PubMed

    2016-02-01

    This is a brief background paper for a supplementary issue of Developmental Medicine & Child Neurology by the Australian Cerebral Palsy Register Group. It provides context for the reader of the supplement including a description of the establishment and development of state and territory cerebral palsy registers in Australia. PMID:26806361

  16. Understanding Mealtime Changes for Adults with Cerebral Palsy and the Implications for Support Services

    ERIC Educational Resources Information Center

    Balandin, Susan; Hemsley, Bronwyn; Hanley, Leah; Sheppard, Justine Joan

    2009-01-01

    Background: Changes in the swallowing capabilities of adults with cerebral palsy as they age may impact on their health, safety, and well-being. Method: Thirty-two adults with cerebral palsy aged between 30 and 69 years participated in in-depth interviews about their experiences of changes in their swallowing and related management of their

  17. European study of frequency of participation of adolescents with and without cerebral palsy

    PubMed Central

    Michelsen, Susan I.; Flachs, Esben M.; Damsgaard, Mogens T.; Parkes, Jacqueline; Parkinson, Kathryn; Rapp, Marion; Arnaud, Catherine; Nystrand, Malin; Colver, Allan; Fauconnier, Jerome; Dickinson, Heather O.; Marcelli, Marco; Uldall, Peter

    2014-01-01

    Children with cerebral palsy participate less in everyday activities than children in the general populations. During adolescence, rapid physical and psychological changes occur which may be more difficult for adolescents with impairments. Within the European SPARCLE project we measured frequency of participation of adolescents with cerebral palsy by administering the Questionnaire of Young People's Participation to 667 adolescents with cerebral palsy or their parents from nine European regions and to 4666 adolescents from the corresponding general populations. Domains and single items were analysed using respectively linear and logistic regression. Adolescents with cerebral palsy spent less time with friends and had less autonomy in their daily life than adolescents in the general populations. Adolescents with cerebral palsy participated much less in sport but played electronic games at least as often as adolescents in the general populations. Severity of motor and intellectual impairment had a significant impact on frequency of participation, the more severely impaired being more disadvantaged. Adolescents with an only slight impairment participated in some domains as often as adolescents in the general populations. Regional variation existed. For example adolescents with cerebral palsy in central Italy were most disadvantaged according to decisional autonomy, while adolescents with cerebral palsy in east Denmark and northern England played sports as often as their general populations. Participation is an important health outcome. Personal and environmental predictors of participation of adolescents with cerebral palsy need to be identified in order to design interventions directed to such predictors; and in order to inform the content of services. PMID:24412031

  18. Theory of Mind and Irony Comprehension in Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Caillies, Stephanie; Hody, Anais; Calmus, Arnaud

    2012-01-01

    The main goal of the present study was to characterise the pragmatic abilities of French children with cerebral palsy through their understanding of irony and other people's mental states. We predicted that children with cerebral palsy would have difficulty understanding false-belief and ironic remarks, due to the executive dysfunction that

  19. European study of frequency of participation of adolescents with and without cerebral palsy.

    PubMed

    Michelsen, Susan I; Flachs, Esben M; Damsgaard, Mogens T; Parkes, Jacqueline; Parkinson, Kathryn; Rapp, Marion; Arnaud, Catherine; Nystrand, Malin; Colver, Allan; Fauconnier, Jerome; Dickinson, Heather O; Marcelli, Marco; Uldall, Peter

    2014-05-01

    Children with cerebral palsy participate less in everyday activities than children in the general populations. During adolescence, rapid physical and psychological changes occur which may be more difficult for adolescents with impairments. Within the European SPARCLE project we measured frequency of participation of adolescents with cerebral palsy by administering the Questionnaire of Young People's Participation to 667 adolescents with cerebral palsy or their parents from nine European regions and to 4666 adolescents from the corresponding general populations. Domains and single items were analysed using respectively linear and logistic regression. Adolescents with cerebral palsy spent less time with friends and had less autonomy in their daily life than adolescents in the general populations. Adolescents with cerebral palsy participated much less in sport but played electronic games at least as often as adolescents in the general populations. Severity of motor and intellectual impairment had a significant impact on frequency of participation, the more severely impaired being more disadvantaged. Adolescents with an only slight impairment participated in some domains as often as adolescents in the general populations. Regional variation existed. For example adolescents with cerebral palsy in central Italy were most disadvantaged according to decisional autonomy, while adolescents with cerebral palsy in east Denmark and northern England played sports as often as their general populations. Participation is an important health outcome. Personal and environmental predictors of participation of adolescents with cerebral palsy need to be identified in order to design interventions directed to such predictors; and in order to inform the content of services. PMID:24412031

  20. Intensive Dysarthria Therapy for Older Children with Cerebral Palsy: Findings from Six Cases

    ERIC Educational Resources Information Center

    Pennington, Lindsay; Smallman, Claire; Farrier, Faith

    2006-01-01

    Children with cerebral palsy often have speech, language and communication difficulties that affect their access to social and educational activities. Speech and language therapy to improve the intelligibility of the speech of children with cerebral palsy has long been advocated, but there is a dearth of research investigating therapy…

  1. Electropalatography in the Description and Treatment of Speech Disorders in Five Children with Cerebral Palsy

    ERIC Educational Resources Information Center

    Nordberg, Ann; Carlsson, Goran; Lohmander, Anette

    2011-01-01

    Some children with cerebral palsy have articulation disorders that are resistant to conventional speech therapy. The aim of this study was to investigate whether the visual feedback method of electropalatography (EPG) could be an effective tool for treating five children (mean age of 9.4 years) with dysarthria and cerebral palsy and to explore…

  2. Psychological Problems in Children with Cerebral Palsy: A Cross-Sectional European Study

    ERIC Educational Resources Information Center

    Parkes, Jackie; White-Koning, Melanie; Dickinson, Heather O.; Thyen, Ute; Arnaud, Catherine; Beckung, Eva; Fauconnier, Jerome; Marcelli, Marco; McManus, Vicki; Michelsen, Susan I.; Parkinson, Kathryn; Colver, Allan

    2008-01-01

    Objectives: To describe psychological symptoms in 8-12-year-old children with cerebral palsy; to investigate predictors of these symptoms and their impact on the child and family. Design: A cross-sectional multi-centre survey. Participants: Eight hundred and eighteen children with cerebral palsy, aged 8-12 years, identified from population-based…

  3. The Cerebral Palsy Quality of Life for Children (CP QOL-Child): Evidence of Construct Validity

    ERIC Educational Resources Information Center

    Chen, Kuan-Lin; Wang, Hui-Yi; Tseng, Mei-Hui; Shieh, Jeng-Yi; Lu, Lu; Yao, Kai-Ping Grace; Huang, Chien-Yu

    2013-01-01

    The Cerebral Palsy Quality of Life for Children (CP QOL-Child) is the first health condition-specific questionnaire designed for measuring QOL in children with cerebral palsy (CP). However, its construct validity has not yet been confirmed by confirmatory factor analysis (CFA). Hence, this study assessed the construct validity of the caregiver…

  4. Bell's Palsy in Children: Role of the School Nurse in Early Recognition and Referral

    ERIC Educational Resources Information Center

    Gordon, Shirley C.

    2008-01-01

    Bell's palsy is the most common condition affecting facial nerves. It is an acute, rapidly progressing, idiopathic, unilateral facial paralysis that is generally self-limiting and non-life threatening that occurs in all age groups (Okuwobi, Omole, & Griffith, 2003). The school nurse may be the first person to assess facial palsy and muscle…

  5. The Cerebral Palsy Quality of Life for Children (CP QOL-Child): Evidence of Construct Validity

    ERIC Educational Resources Information Center

    Chen, Kuan-Lin; Wang, Hui-Yi; Tseng, Mei-Hui; Shieh, Jeng-Yi; Lu, Lu; Yao, Kai-Ping Grace; Huang, Chien-Yu

    2013-01-01

    The Cerebral Palsy Quality of Life for Children (CP QOL-Child) is the first health condition-specific questionnaire designed for measuring QOL in children with cerebral palsy (CP). However, its construct validity has not yet been confirmed by confirmatory factor analysis (CFA). Hence, this study assessed the construct validity of the caregiver

  6. The Early Needs of Children with Cerebral Palsy: A Comprehensive View.

    ERIC Educational Resources Information Center

    Blackman, James A.; Healy, Alfred

    Intended for professionals and parents, this monograph focuses on the service needs of young children with cerebral palsy. Section I presents an overview of cerebral palsy, including etiology, incidence, and history of management. Section II describes service needs in the following areas: prevention; early identification; treatment; the…

  7. Services for Children with Cerebral Palsy; A Guide for Public Health Personnel.

    ERIC Educational Resources Information Center

    American Public Health Association, Inc., New York, NY. Program Area Committee on Child Health.

    Directed to persons in voluntary or official agencies and to planning groups whose decisions determine or affect the extent, coverage, content, and operation of community services to children who are handicapped by cerebral palsy, this guide has as its objectives (1) to present background information on cerebral palsy as it affects the individual…

  8. Reversible facial nerve palsy due to parotid abscess☆☆☆

    PubMed Central

    Hajiioannou, Jiannis K.; Florou, Vasiliki; Kousoulis, Panagiotis; Kretzas, Dimitris; Moshovakis, Eustratios

    2013-01-01

    INTRODUCTION A facial nerve palsy combined with parotid enlargement usually suggests malignancy. It is highly unusual for facial nerve palsy to result from a benign situation such as inflammation or infection of the gland. PRESENTATION OF CASE We present a rare case of facial nerve palsy due to parotid abscess. DISCUSSION A literature search retrieved thirty-two cases of facial nerve palsy due to benign parotid lesions since 1969. Only nine reported the presence of a parotid abscess. The etiology of paralysis remains unknown although certain factors such as the virulence of the offending organisms or perineuritis, have been suggested. Best diagnostic evaluation and management are discussed. CONCLUSION In clinical practice, exclusion of malignancy is mandatory, as it represents the most common cause of facial palsy in the presence of a parotid lump. PMID:24096025

  9. Assessment Method of Facial Palsy by Amount of Feature Point Movements at Facial Expressions

    NASA Astrophysics Data System (ADS)

    Tanaka, Toshiyuki; Nemoto, Junko; Ohta, Manami; Kunihiro, Takanobu

    At present in medical field, the 40 point method and facial nerve grading system (House-Brackmann method) are generally used for assessment of facial palsy. However, those methods have limitation in the precise assessment, because of subjectivity in diagnosis. Purpose of this paper is to propose objective and quantitative assessment of facial palsy based on the amount of feature point movements on the face. Facial nerve symptoms generally appear in either of right and left side on the face. In facial expression of palsy subjects, the motion on the diseased side becomes smaller than that on the healthy side. We defined some indices of palsy severity from the observation of facial expression. Those indices showed the asymmetry of the facial motion quantitatively. We confirmed that our proposed method was valid for assessment of the facial palsy by comparison with the 40 point method.

  10. Isolated sixth nerve palsy: an uncommon presenting sign of multiple sclerosis.

    PubMed

    Barr, D; Kupersmith, M J; Turbin, R; Bose, S; Roth, R

    2000-09-01

    We describe three patients in whom an isolated sixth nerve palsy was the only clinical symptom or sign of multiple sclerosis (MS). Data were collected prospectively over 6 years on these three patients, who showed no other signs of brainstem dysfunction or prior symptoms; in addition. Retrospective analysis of all patients with MS and all patients with sixth nerve palsy referred to a neuro-ophthalmology service between 1982 and 1998 showed isolated sixth nerve palsy to be the presenting sign of MS in only 0.5% of these patients. MS was the cause of isolated sixth nerve palsy in 0.8% of all patients and in 1.6% of those aged 18-50 years. Although it has been previously suggested that sixth nerve palsy is a not uncommon presenting sign of MS, our results suggest it is rare. PMID:11081810

  11. Risk Factor Analysis for C5 Palsy after Double-Door Laminoplasty for Cervical Spondylotic Myelopathy

    PubMed Central

    Ikuta, Ko; Ikeuchi, Hiroko; Shiraki, Makoto; Komiya, Norihiro; Kitamura, Takahiro; Senba, Hideyuki; Shidahara, Satoshi

    2016-01-01

    Study Design A retrospective comparative study. Purpose To clarify the risk factors related to the development of postoperative C5 palsy through radiological studies after cervical double-door laminoplasty (DDL). Overview of Literature Although postoperative C5 palsy is generally considered to be the result of damage to the nerve root or segmental spinal cord, the associated pathology remains controversial. Methods A consecutive case series of 47 patients with cervical spondylotic myelopathy treated by DDL at our institution between April 2008 and April 2015 were reviewed. Postoperative C5 palsy occurred in 5 of 47 cases after DDL. We investigated 9 radiologic factors that have been reported to be risk factors for C5 palsy in various studies, and statistically examined these between the two groups of palsy and the non-palsy patients. Results We found a significant difference between patients with and without postoperative C5 palsy with regards to the posterior shift of spinal cord at C4/5 (p=0.008). The logistic regression analyses revealed posterior shift of the spinal cord at C4/5 (odds ratio, 12.066; p=0.029; 95% confidence interval, 1.295–112.378). For the other radiologic factors, there were no statistically significant differences between the two groups. Conclusions In the present study, we showed a significant difference in the posterior shift of the spinal cord at C4/5 between the palsy and the non-palsy groups, indicating that the "tethering phenomenon" was likely a greater risk factor for postoperative C5 palsy. PMID:27114771

  12. Persistence of Cerebral Palsy Diagnosis: Assessment of a Low-Birth-Weight Cohort at Ages 2, 6, and 9 Years.

    PubMed

    Korzeniewski, Steven J; Feldman, Judith F; Lorenz, John M; Pinto-Martin, Jennifer A; Whitaker, Agnes H; Paneth, Nigel

    2016-03-01

    We examined the stability of nondisabling and disabling cerebral palsy at age 2 in a longitudinally followed tri-county low-birth-weight (<2000 g) birth cohort. A total of 1105 newborns were enrolled, 901 (81.5%) survived to age 2, and 86% (n = 777) were followed up. Of the 113 cerebral palsy diagnoses at age 2, 61 (9% of the cohort, n = 61/777) had disabling cerebral palsy and 52 (7%, n = 52/777) had nondisabling cerebral palsy. Of 48 followed children diagnosed with disabling cerebral palsy at age 2, 98% were again classified as having cerebral palsy at school age, and 1 had an uncertain cerebral palsy status. By contrast, 41% (n = 17) of the 43 children diagnosed with nondisabling cerebral palsy at age 2 were classified as not having cerebral palsy. Of the 517 followed children who were not diagnosed with cerebral palsy at age 2, 7% (n = 35) were classified as having late emerging nondisabling cerebral palsy at school age. PMID:26271791

  13. Surgical correction of unilateral and bilateral facial palsy

    PubMed Central

    Harrison, D

    2005-01-01

    Unilateral and bilateral facial palsies are debilitating and depressing conditions for the patient. For the past 30 years attempts have been made to improve the reanimation of these patients. The ability to transfer axons over significant distances with nerve grafts and the transfer of muscle that can be revascularised by microvascular surgery greatly improves results of this surgery. The revascularisation of muscle has been the important step forward but the re-focusing of interest in this condition has brought about a number of peripheral advances. PMID:16143684

  14. Bilateral traumatic hip dislocation with sciatic nerve palsy.

    PubMed

    Fan, Ka Yuk; Lui, Tun Hing

    2015-01-01

    Bilateral hip dislocation is a rare condition. We report a case of traumatic bilateral hip dislocation and unilateral sciatic nerve palsy in a young woman with known idiopathic scoliosis. With prompt reduction of the dislocated hips, there was reasonable neurological recovery. There was no avascular necrosis of the femoral head or post-traumatic arthritis up to 3-year follow-up. The gender difference in incidence, as well as the predisposition of hip dislocation in scoliosis is discussed. In our case, the decreased femoral anteversion was the culprit. PMID:25809426

  15. Evaluation and management of brachial plexus birth palsy.

    PubMed

    Abzug, Joshua M; Kozin, Scott H

    2014-04-01

    Brachial plexus birth palsy can result in permanent lifelong deficits and unfortunately continues to be relatively common despite advancements in obstetric care. The diagnosis can be made shortly after birth by physical examination, noting a lack of movement in the affected upper extremity. Treatment begins with passive range-of-motion exercises to maintain flexibility and tactile stimulation to provide sensory reeducation. Primary surgery consists of microsurgical nerve surgery, whereas secondary surgery consists of alternative microsurgical procedures, tendon transfers, or osteotomies, all of which improve outcomes in the short term. However, the long-term outcomes of current treatment recommendations remain unknown. PMID:24684916

  16. Does cross-innervation occur after facial palsy?

    PubMed Central

    Trojaborg, W

    1977-01-01

    When the unaffected facial nerve was stimulated in 30 patients with facial palsy, evoked action potentials could be recorded from the contralateral (paralysed) perioral muscles. Similarly, in four normal subjects responses were evoked contralateral to the stimulated facial nerve. The latency of these responses in the patients remained unchanged over several months, and they were conducted at a rate compatible with conduction along muscle fibres. The contralateral responses are suggested to be due to conduction along muscle fibres crossing the midline rather than to cross-innervation. PMID:915517

  17. Lithium Battery Ingestion: An Unusual Cause of Bilateral Cord Palsy.

    PubMed

    Singh, Gautam Bir; Chauhan, Ravinder; Kumar, Deepak; Arora, Rubeena; Ranjan, Shruti

    2015-01-01

    Bilateral vocal cord palsy is a rare but life threatening complication of lithium battery ingestion in children. This complication is mostly missed by otorhinolaryngologists due to lack of awareness on the cited subject. We present one such rare case in an infant, where the clinical presentation was found to be unique but hitherto unreported in the medical literature. This clinical record discusses this case in light of the scant current medical literature on the subject and highlights the importance of cautious monitoring of patients presenting with signs of respiratory distress after lithium battery removal. PMID:26457218

  18. Lithium Battery Ingestion: An Unusual Cause of Bilateral Cord Palsy

    PubMed Central

    Singh, Gautam Bir; Chauhan, Ravinder; Kumar, Deepak; Arora, Rubeena; Ranjan, Shruti

    2015-01-01

    Bilateral vocal cord palsy is a rare but life threatening complication of lithium battery ingestion in children. This complication is mostly missed by otorhinolaryngologists due to lack of awareness on the cited subject. We present one such rare case in an infant, where the clinical presentation was found to be unique but hitherto unreported in the medical literature. This clinical record discusses this case in light of the scant current medical literature on the subject and highlights the importance of cautious monitoring of patients presenting with signs of respiratory distress after lithium battery removal. PMID:26457218

  19. Isolated abducens nerve palsy secondary to Lemierre syndrome.

    PubMed

    Bababeygy, Simon R; Almarzouki, Hashem; Buffenn, Angela N

    2011-12-01

    Lemierre syndrome is characterized by pharyngotonsillitis that is typically secondary to Fusobacterium necrophorum infection and causes adjacent septic thrombophlebitis and thrombosis with subsequent metastatic abscesses. A 3-year-old boy presented with fever, tonsillar enlargement, and bilateral otomastoiditis with purulent discharge. Physical examination revealed abducens nerve palsy on the left side, with a compensatory left head turn. Otomastoid discharge culture was positive for anaerobic F. necrophorum. Magnetic resonance venography of the head and neck revealed thromboses in left internal jugular vein, left cavernous sinus, left superior ophthalmic vein, and left sigmoid sinus. The patient was treated with anticoagulation and a 10-week course of intravenous antibiotics, including ceftriaxone and metronidazole. PMID:22153405

  20. Dynamic evaluation of facial palsy by moire topography video

    NASA Astrophysics Data System (ADS)

    Yuen, Koji; Inokuchi, Ikuo; Maeta, Manabu; Kawakami, Shinichiro; Masuda, Yu

    1994-02-01

    Several visual assessment methods have been proposed for evaluating facial nerve function. They are of value clinically, but they have drawbacks when objective, quantitative, and reproducible assessment is required. To solve these problems, we used moire topography, which helps visualize information in three dimensions. We previously reported that one could evaluate the severity of facial palsy by observing characteristic patterns of the moire strips produced by facial movement. Accordingly, we developed a new form of the dynamic evaluation by recording the dynamic changes in moire strip patterns on the face on a videotape.

  1. Forefoot problems in cerebral palsy-diagnosis and management.

    PubMed

    Bleck, E E

    1984-01-01

    Forefoot deformities in cerebral palsy include cavus, metatarsus adductus, hallux valgus and bunion, dorsal bunion, and toe flexion contractures. Both prevention and correction of these deformities are surgical. Some can be prevented by lengthening or tenotomy of the spastic muscle that causes the dynamic deformity which eventually becomes the fixed skeletal deformity. Fixed deformities need osteotomies and arthrodesis of the bones in addition to removing the force of the deforming muscle. Some forefoot problems are related to the mid and hind foot, e.g., hallux valgus due to spastic pes valgus. Severe cavus can develop after overzealous Achilles tendon lengthening and failure to separate forefoot from hindfoot equinus. PMID:6714860

  2. Foreign body resulting in chronic otomastoiditis and facial palsy.

    PubMed

    Verma, Roshan Kumar; Gupta, Bhumika; Panda, Naresh K

    2015-02-01

    We present a case of a foreign body in the ear of 5-year-old girl child. She presented with features of chronic suppurative otitis media with facial nerve palsy. On exploration exuberant granulation was found in attic and middle ear. A foreign body (seed) was found buried within the granulation tissue which was removed. Bony facial canal was dehiscent in the tympanic segment. She had recovery of facial nerve function. The case is being reported to increase awareness among otolaryngologist and to consider foreign body as a differential diagnosis in cases of complicated CSOM; especially in children. PMID:25500549

  3. A reassessment of spinal stabilization in severe cerebral palsy.

    PubMed

    Cassidy, C; Craig, C L; Perry, A; Karlin, L I; Goldberg, M J

    1994-01-01

    A homogenous population of 37 institutionalized patients with scoliosis and severe cerebral palsy was evaluated to assess the impact of spinal stabilization on comfort, function, health, and ease of nursing care. Through a prospective care-burden study, a 34-month retrospective analysis, and a healthcare worker questionnaire, 17 fused patients with a mean current scoliosis of 35 degrees were compared with 20 nonfused patients with a mean scoliosis of 76 degrees. No clinically significant differences were noted in pain or pulmonary medication utilization or therapy, decubiti, function, or time for daily care. Nevertheless, the majority of healthcare workers believed that the fused patients were more comfortable. PMID:7814585

  4. A population-based study of communication impairment in cerebral palsy.

    PubMed

    Zhang, James Yue; Oskoui, Maryam; Shevell, Michael

    2015-03-01

    To explore factors associated with communication impairments in children with cerebral palsy. Data were obtained on children born between 1999 and 2008 from the Quebec Cerebral Palsy Registry (REPACQ). Out of 535 children with cerebral palsy, 297 were identified to have communication impairments (55.5%). Of these, 96 were unable to communicate verbally (32.3%), 195 had some verbal communication (65.7%), and 6 were unspecified (2.0%). These children were significantly more likely to have a more severe motor deficit (Gross Motor Function Classification System levels IV and V and Manual Ability Classification System levels IV and V), to have spastic quadriplegia or dyskinetic subtypes of cerebral palsy, and gray matter injury on neuroimaging. Communication impairment is a common comorbidity in cerebral palsy and is associated with a more severe motor deficit, spastic quadriplegic or dyskinetic subtype of cerebral palsy, and gray matter injury on neuroimaging. This information allows clinicians to better predict and manage communication impairment in children with cerebral palsy. PMID:25051968

  5. Rehabilitation outcomes in children with cerebral palsy during a 2 year period

    PubMed Central

    İçağasıoğlu, Afitap; Mesci, Erkan; Yumusakhuylu, Yasemin; Turgut, Selin Turan; Murat, Sadiye

    2015-01-01

    [Purpose] To observe motor and functional progress of children with cerebral palsy during 2 years. [Subjects and Methods] Pediatric cerebral palsy patients aged 3–15 years (n = 35/69) with 24-month follow-up at our outpatient cerebral palsy clinic were evaluated retrospectively. The distribution of cerebral palsy types was as follows: diplegia (n = 19), hemiplegia (n = 4), and quadriplegia (n = 12). Participants were divided into 3 groups according to their Gross Motor Functional Classification System scores (i.e., mild, moderate, and severe). All participants were evaluated initially and at the final assessment 2 years later. During this time, patients were treated 3 times/week. Changes in motor and functional abilities were assessed based on Gross Motor Function Measure-88 and Wee Functional Independence Measure. [Results] Significant improvements were observed in Gross Motor Function Measure-88 and Wee Functional Independence Measure results in all 35 patients at the end of 2 years. The Gross Motor Function Measure-88 scores correlated with Wee Functional Independence Measure Scores. Marked increases in motor and functional capabilities in mild and moderate cerebral palsy patients were observed in the subgroup assessments, but not in those with severe cerebral palsy. [Conclusion] Rehabilitation may greatly help mild and moderate cerebral palsy patients achieve their full potential. PMID:26644677

  6. Center-of-pressure movements during equine-assisted activities.

    PubMed

    Clayton, Hilary M; Kaiser, Leeann J; de Pue, Bonnie; Kaiser, Lana

    2011-01-01

    We compared anteroposterior and mediolateral range of motion and velocity of the center of pressure (COP) on the horse's back between riders without disabilities and riders with cerebral palsy. An electronic pressure mat was used to track COP movements beneath the saddle in 4 riders without disabilities and 4 riders with cerebral palsy. Comparisons between rider groups were made using the Mann-Whitney test (p < .05). The two rider groups differed significantly in anteroposterior range of COP motion, mediolateral range of COP motion, and mediolateral COP velocity. Anteroposterior COP velocity did not differ between groups. The results suggest that measurements of COP range of motion and velocity are potentially useful for monitoring changes in balance as an indicator of core stability during equine-assisted activities. PMID:21476369

  7. Peroneal neuropathy after weight loss.

    PubMed

    Cruz-Martinez, A; Arpa, J; Palau, F

    2000-06-01

    The objectives of this study were to evaluate the clinical and electrophysiological findings in peroneal mononeuropathies following a weight-reduction diet. Thirty patients with acute peroneal palsy and weight loss were studied. Complete nerve conduction studies (NCS) were performed in upper and lower limbs. NCS showed conduction block (CB) of the peroneal nerve at the fibular head that recovered in 29 patients within 3 weeks to 3 months. Severity of CB was correlated with clinical weakness. Three patients had abnormalities consistent with polyneuropathy (PNP). NCS in asymptomatic relatives confirmed familial neuropathy. Nerve biopsy and molecular study were consistent with hereditary neuropathy with liability to pressure palsies (HNPP). One of these peroneal palsies (6 months) recovered after neurolysis. Weight loss might be a risk factor in peroneal mononeuropathies. NCS is a tool in the diagnosis of the site and severity of the nerve injury. Testing should be considered for relatives of patients with PNP because peroneal mononeuropathies may be the first expression of HNPP. PMID:10905469

  8. Habitual physical activity and cardiometabolic risk factors in adults with cerebral palsy.

    PubMed

    Ryan, Jennifer M; Crowley, Vivion E; Hensey, Owen; Broderick, Julie M; McGahey, Ailish; Gormley, John

    2014-09-01

    Adults with cerebral palsy (CP) are known to participate in reduced levels of total physical activity. There is no information available however, regarding levels of moderate-to-vigorous physical activity (MVPA) in this population. Reduced participation in MVPA is associated with several cardiometabolic risk factors. The purpose of this study was firstly to compare levels of sedentary, light, MVPA and total activity in adults with CP to adults without CP. Secondly, the objective was to investigate the association between physical activity components, sedentary behavior and cardiometabolic risk factors in adults with CP. Adults with CP (n=41) age 18-62 yr (mean ± SD=36.5 ± 12.5 yr), classified in Gross Motor Function Classification System level I (n=13), II (n=18) and III (n=10) participated in this study. Physical activity was measured by accelerometry in adults with CP and in age- and sex-matched adults without CP over 7 days. Anthropometric indicators of obesity, blood pressure and several biomarkers of cardiometabolic disease were also measured in adults with CP. Adults with CP spent less time in light, moderate, vigorous and total activity, and more time in sedentary activity than adults without CP (p<0.01 for all). Moderate physical activity was associated with waist-height ratio when adjusted for age and sex (β=-0.314, p<0.05). When further adjustment was made for total activity, moderate activity was associated with waist-height ratio (β=-0.538, p<0.05), waist circumference (β=-0.518, p<0.05), systolic blood pressure (β=-0.592, p<0.05) and diastolic blood pressure (β=-0.636, p<0.05). Sedentary activity was not associated with any risk factor. The findings provide evidence that relatively young adults with CP participate in reduced levels of MVPA and spend increased time in sedentary behavior, potentially increasing their risk of developing cardiometabolic disease. PMID:24864052

  9. Prevention of cerebral palsy in motor risk infants by treatment ad modum Vojta. A controlled study.

    PubMed

    Brandt, S; Lønstrup, H V; Marner, T; Rump, K J; Selmar, P; Schack, L K; d'Avignon, M; Norén, L; Arman, T

    1980-05-01

    The proposal by V. Vojta in 1974 to prevent development of cerebral palsy in "motor risk" infants by special treatment has been investigated in 11 Danish and 10 Swedish babies and compared with 30 control infants with similar risk, who were not given Vojta treatment. We found a tendency for "uncomplicated" cerebral palsy cases to accumulate in the control group, although the difference was non-significant on 1 5% level. Further controlled studies must be completed before it is possible to accept the prophylactive treatment of cerebral palsy recommended by Vojta. PMID:7376854

  10. Unilateral abducens and bilateral facial nerve palsies associated with posterior fossa exploration surgery

    PubMed Central

    Khalil, Ayman; Clerkin, James; Mandiwanza, Tafadzwa; Green, Sandra; Javadpour, Mohsen

    2016-01-01

    Multiple cranial nerves palsies following a posterior fossa exploration confined to an extradural compartment is a rare clinical presentation. This case report describes a young man who developed a unilateral abducens and bilateral facial nerve palsies following a posterior fossa exploration confined to an extradural compartment. There are different theories to explain this presentation, but the exact mechanism remains unclear. We propose that this patient cranial nerve palsies developed following cerebrospinal fluid (CSF) leak, potentially as a consequence of rapid change in CSF dynamics. PMID:26951144

  11. Unilateral abducens and bilateral facial nerve palsies associated with posterior fossa exploration surgery.

    PubMed

    Khalil, Ayman; Clerkin, James; Mandiwanza, Tafadzwa; Green, Sandra; Javadpour, Mohsen

    2016-01-01

    Multiple cranial nerves palsies following a posterior fossa exploration confined to an extradural compartment is a rare clinical presentation. This case report describes a young man who developed a unilateral abducens and bilateral facial nerve palsies following a posterior fossa exploration confined to an extradural compartment. There are different theories to explain this presentation, but the exact mechanism remains unclear. We propose that this patient cranial nerve palsies developed following cerebrospinal fluid (CSF) leak, potentially as a consequence of rapid change in CSF dynamics. PMID:26951144

  12. Rehabilitation Needs of People with Cerebral Palsy: a qualitative Study

    PubMed Central

    sharifi, Azam; Kamali, Mohammad; Chabok, Ali

    2014-01-01

    Background: Cerebral palsy (CP) describes a group of disorders regarding the development of movement and posture, which causes limitations in activity. In fact, it is attributed to non-progressive disturbances that occur during brain development in fetus or infant. CP disorders may accompany by speech, auditory, visual abnormality, seizure, learning disorder, mental retardation and etc. Due to the variation in disorders and ultimately the needs that are made in the wake of the diseases, understanding the needs of these patients is essential. Methods: This research was a qualitative study, with phenomenology method and sampling was purposeful. The participants were 17 cerebral palsy people (6 female and 11 male, with aged 15 to 43). Data were collected by deep interview with open-end questions and analyzed by collaizi method. Results: During the interview sessions, notes and ideas were classified and assorted, so that, the rehabilitation needs of people with CP were understood according to the statements of participants. The results of this study were placed in four domains, 3 themes and 22 subthemes. The domains included social, emotional needs, economic, and therapeutic needs. Conclusion: The requirements studies in this research were particularly introduced by patients with CP. People in the society, who might have contact with these patients, are responsible to help them to overcome their problems and disabilities. PMID:25250261

  13. Rehabilitation and neuroplasticity in children with unilateral cerebral palsy.

    PubMed

    Reid, Lee B; Rose, Stephen E; Boyd, Roslyn N

    2015-07-01

    Cerebral palsy is a childhood-onset, lifelong neurological disorder that primarily impairs motor function. Unilateral cerebral palsy (UCP), which impairs use of one hand and perturbs bimanual co-ordination, is the most common form of the condition. The main contemporary upper limb rehabilitation strategies for UCP are constraint-induced movement therapy and bimanual intensive therapy. In this Review, we outline the factors that are crucial to the success of motor rehabilitation in children with UCP, including the dose of training, the relevance of training to daily life, the suitability of training to the age and goals of the child, and the ability of the child to maintain close attention to the tasks. Emerging evidence suggests that the first 2 years of life are a critical period during which interventions for UCP could be more effective than in later life. Abnormal brain organization in UCP, and the effects of development on rehabilitation, must also be understood to develop new effective interventions. Therefore, we also consider neuroimaging methods that can provide insight into the neurobiology of UCP and how the condition responds to existing therapies. We discuss how these methods could shape future rehabilitative strategies based on the neurobiology of UCP and the therapy-induced changes seen in the brain. PMID:26077839

  14. Occipital Condyle Fracture With Isolated Unilateral Hypoglossal Nerve Palsy

    PubMed Central

    Yoon, Jin Won; Lim, Oh Kyung; Park, Ki Deok

    2014-01-01

    Occipital condyle fractures (OCFs) with selective involvement of the hypoglossal canal are rare. OCFs usually occur after major trauma and combine multiple fractures. We describe a 38-year-old man who presented with neck pain and a tongue deviation to the right side after a traffic accident. Severe limitations were detected during active and passive range of neck motion in all directions. A physical examination revealed a normal gag reflex and normal mobility of the palate, larynx, and shoulder girdle. He had normal taste and general sensation in his tongue. However, he presented with a tongue deviation to the right side on protrusion. A videofluoroscopic swallowing study revealed piecemeal deglutition due to decreased tongue mobility but no aspiration of food. Plain X-ray film findings were negative, but a computed tomography study with coronal reconstruction demonstrated a right OCF involving the hypoglossal canal. An electrodiagnostic study revealed evidence of right hypoglossal nerve palsy. We report a rare case of isolated hypoglossal nerve palsy caused by an OCF. PMID:25379499

  15. Causes of Secondary Radial Nerve Palsy and Results of Treatment

    PubMed Central

    Reichert, Paweł; Wnukiewicz, Witold; Witkowski, Jarosław; Bocheńska, Aneta; Mizia, Sylwia; Gosk, Jerzy; Zimmer, Krzysztof

    2016-01-01

    Background The aim of this study was to analyze the causes that lead to secondary damage of the radial nerve and to discuss the results of reconstructive treatment. Material/Methods The study group consisted of 33 patients treated for radial nerve palsy after humeral fractures. Patients were diagnosed based on clinical examinations, ultrasonography, electromyography, or nerve conduction velocity. During each operation, the location and type of nerve damage were analyzed. During the reconstructive treatment, neurolysis, direct neurorrhaphy, or reconstruction with a sural nerve graft was used. The outcomes were evaluated using the Medical Research Council (MRC) scales and the quick DASH score. Results Secondary radial nerve palsy occurs after open reduction and internal fixation (ORIF) by plate, as well as by closed reduction and internal fixation (CRIF) by nail. In the case of ORIF, it most often occurs when the lateral approach is used, as in the case of CRIF with an insertion interlocking screws. The results of the surgical treatment were statistically significant and depended on the time between nerve injury and revision (reconstruction) surgery, type of damage to the radial nerve, surgery treatment, and type of fixation. Treatment results were not statistically significant, depending on the type of fracture or location of the nerve injury. Conclusions The potential risk of radial nerve neurotmesis justifies an operative intervention to treat neurological complications after a humeral fracture. Adequate surgical treatment in many of these cases allows for functional recovery of the radial nerve. PMID:26895570

  16. Bell's Palsy: Symptoms Preceding and Accompanying the Facial Paresis

    PubMed Central

    Mancini, Patrizia; Minni, Antonio; Prosperini, Luca; De Seta, Elio; Attanasio, Giuseppe; Covelli, Edoardo; De Carlo, Andrea; Filipo, Roberto

    2014-01-01

    This individual prospective cohort study aims to report and analyze the symptoms preceding and accompanying the facial paresis in Bell's palsy (BP). Two hundred sixty-nine patients affected by BP with a maximum delay of 48 hours from the onset were enrolled in the study. The evolution of the facial paresis expressed as House-Brackmann grade in the first 10 days and its correlation with symptoms were analyzed. At the onset, 136 patients presented postauricular pain, 114 were affected by dry eye, and 94 reported dysgeusia. Dry mouth was present in 54 patients (19.7%), facial pain, hyperlacrimation, aural fullness, and hyperacusis represented a smaller percentage of the reported symptoms. After 10 days, 39.9% of the group had a severe paresis while 10.2% reached a complete recovery. Dry mouth at the onset was correlated with severe grade of palsy and was prognostic for poor recovery in the early period. These outcomes lead to the deduction that the nervus intermedius plays an important role in the presentation of the BP and it might be responsible for most of the accompanying symptomatology of the paresis. Our findings could be of important interest to early address a BP patient to further examinations and subsequent therapy. PMID:25544960

  17. Communication impairments in people with progressive supranuclear palsy: A tutorial.

    PubMed

    Kim, Jae-Hyun; McCann, Clare M

    2015-01-01

    Progressive supranuclear palsy (PSP) is a progressive neurological condition, whose main features include supranuclear gaze palsy, frequent falls, bradykinesia, axial rigidity, cognitive decline and communication impairments. Even though communication impairments are early and prominent manifestations, there is a significant lack of research on the nature of these impairments in PSP and the role of speech-language pathologists (SLPs). This tutorial article aims to describe the communication impairments observed in people with PSP; provide clinical guidelines for SLPs when assessing motor speech, language and other communication impairments; and to present facilitation and compensation approaches to treatment for people with PSP. The predominant motor speech impairment is mixed dysarthria, but there is a lack of consensus about the classification of language impairments. The involvement of SLPs in the assessment and treatment of people with PSP should be early, on-going and in collaboration with other health professionals, with the primary focus of maintaining quality of life for these patients and their family members. PMID:26184056

  18. Peripheral magnetic stimulation to decrease spasticity in cerebral palsy.

    PubMed

    Flamand, Véronique H; Beaulieu, Louis-David; Nadeau, Line; Schneider, Cyril

    2012-11-01

    Muscle spasticity in pediatric cerebral palsy limits movement and disrupts motor performance, thus its reduction is important in rehabilitation to optimize functional motor development. Our pilot study used repetitive peripheral magnetic stimulation, because this emerging technology influences spinal and cerebral synaptic transmission, and its antispastic effects were reported in adult neurologic populations. We tested whether five sessions of tibial and common peroneal nerve stimulation exerted acute and long-term effects on spasticity of the ankle plantar flexor muscles in five spastic diparetic children (mean age, 8 years and 3 months; standard deviation, 1 year and 10 months). Muscle resistance to fast stretching was measured with a manual dynamometer as a spasticity indicator. A progressive decrease was observed for the more impaired leg, reaching significance at the third session. This sustained reduction of spasticity may reflect that the peripheral stimulation improved the controls over the spinal circuitry. It thus suggests that a massive stimulation-induced recruitment of sensory afferents may be able to influence central nervous system plasticity in pediatric cerebral palsy. PMID:23044016

  19. Multiple myeloma presenting with unilateral abducens and trigeminal nerve palsies.

    PubMed

    Thiruvengadam, Sushrut S; Prayson, Richard A

    2016-04-01

    Petrous apex masses can manifest with neurologic symptoms due to their involvement of various structures, including cranial nerves (CN) V and VI. The differential diagnosis of petrous masses is broad and includes a variety of both non-neoplastic and neoplastic lesions. We report a rare case of multiple myeloma confined to the right petrous apex, presenting with ipsilateral abducens and trigeminal nerve palsies. A 63-year-old woman presented with a 6-8week history of facial numbness and a 2week history of diplopia, with examination showing right-sided facial hypoesthesia in the CN V1-V3 region and right-sided lateral rectus palsy. MRI of the brain showed a solitary 2.0cm lesion confined to the right petrous apex involving the right cavernous internal carotid artery and Meckel's cave. A transnasal biopsy showed a proliferation of plasmacytoid cells, which showed diffuse immunoreactivity with antibodies to CD138 and kappa, consistent with a plasma cell dyscrasia. A bone scan subsequently revealed multiple lytic bone lesions involving the skull, left humerus, bilateral femurs and possibly the L4 vertebral body. Bone marrow biopsy and serum laboratory results confirmed the diagnosis of kappa-type multiple myeloma. Although rare, multiple myeloma may initially present with petrous involvement and associated cranial nerve deficits. PMID:26602603

  20. Model of cerebral palsy in the perinatal rabbit.

    PubMed

    Tan, Sidhartha; Drobyshevsky, Alexander; Jilling, Tamas; Ji, Xinhai; Ullman, Lauren M; Englof, Ila; Derrick, Matthew

    2005-12-01

    Perinatal brain injury results in one of the highest burdens of disease in view of the lifelong consequences and is of enormous cost to society. This makes it imperative to develop better animal models that mimic the human condition. Many neurodevelopmental deficits, such as cerebral palsy, are believed to be a result of prenatal hypoxia-ischemia in humans. Fetal global hypoxia-ischemia is most commonly a consequence of acute placental insufficiency. Our laboratory has modeled in utero sustained and repetitive hypoxia-ischemia in the pregnant rabbit to mimic the insults of abruptio placenta and labor, respectively. Sustained hypoxia-ischemia at 70% (22 days' gestation) and 79% (25 days' gestation) and repetitive hypoxia-ischemia at 90% gestation (28 days' gestation) caused stillbirths and multiple deficits in the postnatal survivors. The deficits included impairment in multiple tests of spontaneous locomotion, reflex motor activity, motor responses to olfactory stimuli, and the coordination of suck and swallow. Hypertonia was observed in the 22 and 25 days' gestation survivors but not in the 28 days' gestation group. Hypertonic survivors were artificially fed and found to have the motor deficits persist for at least 11 postnatal days. A spectrum of brain abnormalities is found on magnetic resonance imaging. This is the first animal model to mimic cerebral palsy. The findings also suggest a window of vulnerability during brain development when the injury results in hypertonia in newborn pups. PMID:16417845

  1. Children with cerebral palsy effectively modulate postural control to perform a supra-postural task.

    PubMed

    Schmit, Jennifer; Riley, Michael; Cummins-Sebree, Sarah; Schmitt, Laura; Shockley, Kevin

    2015-06-01

    The purpose of this study was to determine whether signatures of adaptive postural control remain present in children with cerebral palsy (CP) when they performed a supra-postural task (i.e., a task performed above and beyond the control of posture) requiring them to balance a marble inside a tube held in the hands. Measures of center of pressure (COP) dynamics (how regular or predictable were the COP data as quantified by the sample entropy metric) and variability (as quantified by the COP standard deviation) were obtained from a sample of children with CP (n=30) and compared to the same measures taken from typically developing (TD) children. Children with CP demonstrated an apparent inefficiency in postural control (greater irregularity, greater sway variability) relative to TD peers during a quiet-stance (no supra-postural task) condition (p<.05). During supra-postural task performance, those differences were attenuated, though they remained statistically different (p<.05). The findings illustrate flexibility and adaptability in the postural control system, despite the pathological features associated with CP. PMID:25913503

  2. Kinetic comparison of walking on a treadmill versus over ground in children with cerebral palsy.

    PubMed

    van der Krogt, Marjolein M; Sloot, Lizeth H; Buizer, Annemieke I; Harlaar, Jaap

    2015-10-15

    Kinetic outcomes are an essential part of clinical gait analysis, and can be collected for many consecutive strides using instrumented treadmills. However, the validity of treadmill kinetic outcomes has not been demonstrated for children with cerebral palsy (CP). In this study we compared ground reaction forces (GRF), center of pressure, and hip, knee and ankle moments, powers and work, between overground (OG) and self-paced treadmill (TM) walking for 11 typically developing (TD) children and 9 children with spastic CP. Considerable differences were found in several outcome parameters. In TM, subjects demonstrated lower ankle power generation and more absorption, and increased hip moments and work. This shift from ankle to hip strategy was likely due to a more backward positioning of the hip and a slightly more forward trunk lean. In mediolateral direction, GRF and hip and knee joint moments were increased in TM due to wider step width. These findings indicate that kinetic data collected on a TM cannot be readily compared with OG data in TD children and children with CP, and that treadmill-specific normative data sets should be used when performing kinetic gait analysis on a treadmill. PMID:26315918

  3. Seat surface inclination may affect postural stability during Boccia ball throwing in children with cerebral palsy.

    PubMed

    Tsai, Yung-Shen; Yu, Yi-Chen; Huang, Po-Chang; Cheng, Hsin-Yi Kathy

    2014-12-01

    The aim of the study was to examine how seat surface inclination affects Boccia ball throwing movement and postural stability among children with cerebral palsy (CP). Twelve children with bilateral spastic CP (3 with gross motor function classification system Level I, 5 with Level II, and 4 with Level III) participated in this study. All participants underwent pediatric reach tests and ball throwing performance analyses while seated on 15° anterior- or posterior-inclined, and horizontal surfaces. An electromagnetic motion analysis system was synchronized with a force plate to assess throwing motion and postural stability. The results of the pediatric reach test (p = 0.026), the amplitude of elbow movement (p = 0.036), peak vertical ground reaction force (PVGRF) (p < 0.001), and movement range of the center of pressure (COP) (p < 0.020) were significantly affected by seat inclination during throwing. Post hoc comparisons showed that anterior inclination allowed greater amplitude of elbow movement and PVGRF, and less COP movement range compared with the other inclines. Posterior inclination yielded less reaching distance and PVGRF, and greater COP movement range compared with the other inclines. The anterior-inclined seat yielded superior postural stability for throwing Boccia balls among children with bilateral spastic CP, whereas the posterior-inclined seat caused difficulty. PMID:25241116

  4. Weather conditions and Bell's palsy: five-year study and review of the literature

    PubMed Central

    Danielides, Vasilis; Patrikakos, George; Nousia, Christina-Sophia; Bartzokas, Aristides; Milionis, Haralampos J; Lolis, Christos; Skevas, Antonios

    2001-01-01

    Background Climatic or meteorological condition changes have been implicated in the pathogenesis of Bell's palsy (BP). We evaluate the influence of meteorological parameters, such as temperature, humidity, and atmospheric pressure, and their variation and covariation on the incidence of BP and present a review of the literature on the effect of meteorological conditions on facial nerve function. Methods A total of 171 cases of BP admitted to our Department over a five-year period were studied. The meteorological database included daily values of 13 distinct parameters recorded at the meteorological station of the University of Ioannina during this period. A relationship between each meteorological variable and the incidence of BP was investigated by applying (Χ2) test on data from 13 contingency tables. In addition, the influence of different weather types on the incidence of BP was also investigated. For this purpose Cluster Analysis was used to create eight clusters (weather types) for the Ioannina prefecture and (Χ2) test was applied on the contingency tables consisting of the days of BP cases for each cluster. Results No significant correlation was found either between BP and each distinct meteorological parameter or between BP and any specific weather. Conclusions Meteorological conditions, such as those dominating in the Northwestern Greece, and/or their changes have little effect on the incidence of BP. Multicenter studies taking into account atmospheric pollution, and climatic differences between countries, are necessary to scrutinize the environmental effects on facial nerve function. PMID:11737872

  5. Computer and microswitch-based programs to improve academic activities by six children with cerebral palsy.

    PubMed

    Stasolla, Fabrizio; Damiani, Rita; Perilli, Viviana; D'Amico, Fiora; Caffò, Alessandro O; Stella, Anna; Albano, Vincenza; Damato, Concetta; Leone, Antonia Di

    2015-01-01

    This study was aimed at extending the use of assistive technology (i.e. microswitch such as a pressure sensor, interface and laptop) with a new setup, allowing six children with cerebral palsy and extensive motor disabilities to improve their academic activities during classroom. A second objective of the study was to assess a maintenance/generalization phase, occurring three months after the end of the intervention, at participants' homes, involving their parents. A third purpose of the study was to monitor the effects of the intervention program on the indices of positive participations (i.e. constructive engagement) of participants involved. Finally, a social validation procedure involving 36 support teachers as raters was conducted. The study was carried out according to a multiple probe design across behaviours followed by maintenance/generalization phase for each participant. That is, the two behaviours (i.e. choice among academic disciplines and literacy) were learned first singly, then combined together. Results showed an increasing of the performances for all participants involved during intervention phases. Furthermore, during maintenance phase participants consolidated their results. Moreover, positive participation augmented as well. Support teachers, involved in the social validation assessment, considered the combined intervention as more favourable with respect to those singly learned. Clinical, educational and practical implications of the findings are discussed. PMID:26196086

  6. [Measurement of external pressure of peroneal nerve tract coming in contact with lithotomy leg holders using pressure distribution measurement system BIG-MAT®].

    PubMed

    Mizuno, Ju; Namba, Chikara; Takahashi, Toru

    2014-10-01

    We investigated external pressure on peroneal nerve tract coming in contact with two kinds of leg holders using pressure distribution measurement system BIG- MAT® (Nitta Corp., Osaka) in the lithotomy position Peak contact (active) pressure at the left fibular head region coming in contact with knee-crutch-type leg holder M® (Takara Belmont Corp., Osaka), which supports the left popliteal fossa, was 78.0 ± 26.4 mmHg. On the other hand, peak contact pressure at the left lateral lower leg region coming in contact with boot-support-type leg holder Bel Flex® (Takara Belmont Corp., Osaka), which supports the left lower leg and foot was 26.3±7.9 mmHg. These results suggest that use of knee-crutch-type leg holder is more likely to induce common peroneal nerve palsy at the fibular head region, but use of boot-support-type leg holder dose not easily induce superficial peroneal nerve palsy at the lateral lower leg region, because capillary blood pressure is known to be 32 mmHg. Safer holders for positioning will be developed to prevent nerve palsy based on the analysis of chronological change in external pressure using BIG-MAT® system during anesthesia. PMID:25693355

  7. Screening for mutations in the peripheral myelin genes PMP22, MPZ and Cx32 (GJB1) in Russian Charcot-Marie-Tooth neuropathy patients.

    PubMed

    Mersiyanova, I V; Ismailov, S M; Polyakov, A V; Dadali, E L; Fedotov, V P; Nelis, E; Löfgren, A; Timmerman, V; van Broeckhoven, C; Evgrafov, O V

    2000-01-01

    Charcot-Marie-Tooth disease (CMT) and related inherited peripheral neuropathies, including Dejerine-Sottas syndrome, congenital hypomyelination, and hereditary neuropathy with liability to pressure palsies (HNPP), are caused by mutations in three myelin genes: PMP22, MPZ and Cx32 (GJB1). The most common mutations are the 1.5 Mb CMT1A tandem duplication on chromosome 17p11.2-p12 in CMT1 patients and the reciprocal 1.5 Mb deletion in HNPP patients. We performed a mutation screening in 174 unrelated CMT patients and three HNPP families of Russian origin. The unrelated CMT patients included 108 clinically and electrophysiologically diagnosed CMT1 cases, 32 CMT2 cases, and 34 cases with unspecified CMT. Fifty-nine CMT1A duplications were found, of which 58 belonged to the CMT1 patient group. We found twelve distinct mutations in Cx32, six mutations in MPZ, and two mutations in PMP22. Of these respectively, eight, five, and two lead to a CMT1 phenotype. Eight mutations (Cx32: Ile20Asn/Gly21Ser, Met34Lys, Leu90Val, and Phe193Leu; MPZ: Asp134Gly, Lys138Asn, and Thr139Asn; PMP22: ValSer25-26del) were not reported previously. Phenotype-genotype correlations were based on nerve conduction velocity studies and mutation type. PMID:10737979

  8. Charcot-Marie-Tooth disease: frequency of genetic subtypes in a Southern Italy population.

    PubMed

    Manganelli, Fiore; Tozza, Stefano; Pisciotta, Chiara; Bellone, Emilia; Iodice, Rosa; Nolano, Maria; Geroldi, Alessandro; Capponi, Simona; Mandich, Paola; Santoro, Lucio

    2014-12-01

    The objective of this study is to assess the genetic distribution of Charcot-Marie-Tooth (CMT) disease in Campania, a region of Southern Italy. We analyzed a cohort of 197 index cases and reported the type and frequency of mutations for the whole CMT population and for each electrophysiological group (CMT1, CMT2, and hereditary neuropathy with susceptibility to pressure palsies [HNPP]) and for familial and isolated CMT cases. Genetic diagnosis was achieved in 148 patients (75.1%) with a higher success rate in HNPP and CMT1 than CMT2. Only four genes (PMP22, GJB1, MPZ, and GDAP1) accounted for 92% of all genetically confirmed CMT cases. In CMT1, PMP22 duplication was the most common mutation while the second gene in order of frequency was MPZ in familial and SH3TC2 in isolated cases. In CMT2, GJB1 was the most frequent mutated gene and GJB1 with GDAP1 accounted for almost 3/4 of genetically defined CMT2 patients. The first gene in order of frequency was GJB1 in familial and GDAP1 in isolated cases. In HNPP, the majority of patients harbored the PMP22 gene deletion. The novelty of our data is the relatively high frequency of SH3TC2 and GDAP1 mutations in demyelinating and axonal forms, respectively. These epidemiological data can help in panel design for our patients' population. PMID:25429913

  9. Abnormal Junctions and Permeability of Myelin in PMP22-Deficient Nerves

    PubMed Central

    Guo, Jiasong; Wang, Leiming; Zhang, Yang; Wu, Jiawen; Arpag, Sezgi; Hu, Bo; Imhof, Beat A.; Tian, Xinxia; Carter, Bruce D.; Suter, Ueli; Li, Jun

    2014-01-01

    Objective The peripheral myelin protein-22 (PMP22) gene is associated with the most common types of inherited neuropathies, including hereditary neuropathy with liability to pressure palsies (HNPP) caused by PMP22 deficiency. However, the function of PMP22 has yet to be defined. Our previous study has shown that PMP22 deficiency causes an impaired propagation of nerve action potentials in the absence of demyelination. In the present study, we tested an alternative mechanism relating to myelin permeability. Methods Utilizing Pmp22+/− mice as a model of HNPP, we evaluated myelin junctions and their permeability using morphological, electrophysiological, and biochemical approaches. Results We show disruption of multiple types of cell junction complexes in peripheral nerve, resulting in increased permeability of myelin and impaired action potential propagation. We further demonstrate that PMP22 interacts with immunoglobulin domain–containing proteins known to regulate tight/adherens junctions and/or transmembrane adhesions, including junctional adhesion molecule-C (JAM-C) and myelin-associated glycoprotein (MAG). Deletion of Jam-c or Mag in mice recapitulates pathology in HNPP. Interpretation Our study reveals a novel mechanism by which PMP22 deficiency affects nerve conduction not through removal of myelin, but through disruption of myelin junctions. PMID:24339129

  10. Detection of genomic rearrangements by DHPLC: a prospective study of 90 patients with inherited peripheral neuropathies associated with 17p11.2 rearrangements.

    PubMed

    Naïmi, Mourad; Tardieu, Sandrine; Depienne, Christel; Ruberg, Merle; Brice, Alexis; Dubourg, Odile; Leguern, Eric

    2005-07-15

    Large genomic duplications and deletions are increasingly recognized as a cause of human disease. Charcot-Marie-Tooth type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsy (HNPP) result, respectively, from a duplication or deletion of a 1.5 Mb genomic region in 17p11.2-12, containing the PMP22 gene. In routine diagnostic analysis, CMT1A status is inferred from the detection of an imbalanced dosage of two alleles or the presence of three alleles of a polymorphic marker flanking the PMP22 gene. HNPP is suspected if only one allele is seen, but hemizygosity must be confirmed by analyzing allele segregation in the family or by other techniques such as Southern blotting or fluorescence in situ hybridization (FISH). PCR-based methodologies have also been developed that allow single-step determination of the PMP22 gene copy number, wherein amplicons are typically labeled and/or separated by gel electrophoresis. We describe here a fast and reliable PCR-based method for the diagnosis of CMT1A and HNPP in which the PMP22 gene is co-amplified with a reference gene, and the amplicons are separated according to their size and quantified by DHPLC. Our results suggest that this method for quantifying gene dosage could be applied to other genomic rearrangements. PMID:15940694

  11. Hereditary neuropathy.

    PubMed

    Heidenreich, Wayne F

    2010-01-01

    A 58-year-old male presented with a history of slowly progressive bilateral hand weakness manifested by decreased grip strength and pinch strength associated with some pain in the first metacarpal-carpal joints with atrophy of the muscles of the web space. An evaluation based on history, physical exam, and judicious diagnostic testing yielded a finding of motor and sensory peripheral polyneuropathy and a working diagnosis of hereditary nerve pressure palsy syndrome (HNPP) or hereditary neuropathy with liability to pressure palsies. The clinical findings and diagnostic tests for sensory and motor peripheral neuropathy are discussed. The case details over time, hereditary features, and the natural history of this disorder lead to a favorable clinical and insurance medicine prognosis. PMID:21290997

  12. [Acupuncture and Vojta therapy in infantile cerebral palsy--a comparison of the effects].

    PubMed

    Stockert, K

    1998-01-01

    Acupuncture and Vojta therapy are using more or less identical points and identical muscle chains for the treatment of infantile cerebral palsy. Therefore a common utilization seems to be sensible. PMID:10025039

  13. A smartphone-based automatic diagnosis system for facial nerve palsy.

    PubMed

    Kim, Hyun Seok; Kim, So Young; Kim, Young Ho; Park, Kwang Suk

    2015-01-01

    Facial nerve palsy induces a weakness or loss of facial expression through damage of the facial nerve. A quantitative and reliable assessment system for facial nerve palsy is required for both patients and clinicians. In this study, we propose a rapid and portable smartphone-based automatic diagnosis system that discriminates facial nerve palsy from normal subjects. Facial landmarks are localized and tracked by an incremental parallel cascade of the linear regression method. An asymmetry index is computed using the displacement ratio between the left and right side of the forehead and mouth regions during three motions: resting, raising eye-brow and smiling. To classify facial nerve palsy, we used Linear Discriminant Analysis (LDA) and Support Vector Machine (SVM), and Leave-one-out Cross Validation (LOOCV) with 36 subjects. The classification accuracy rate was 88.9%. PMID:26506352

  14. A Smartphone-Based Automatic Diagnosis System for Facial Nerve Palsy

    PubMed Central

    Kim, Hyun Seok; Kim, So Young; Kim, Young Ho; Park, Kwang Suk

    2015-01-01

    Facial nerve palsy induces a weakness or loss of facial expression through damage of the facial nerve. A quantitative and reliable assessment system for facial nerve palsy is required for both patients and clinicians. In this study, we propose a rapid and portable smartphone-based automatic diagnosis system that discriminates facial nerve palsy from normal subjects. Facial landmarks are localized and tracked by an incremental parallel cascade of the linear regression method. An asymmetry index is computed using the displacement ratio between the left and right side of the forehead and mouth regions during three motions: resting, raising eye-brow and smiling. To classify facial nerve palsy, we used Linear Discriminant Analysis (LDA) and Support Vector Machine (SVM), and Leave-one-out Cross Validation (LOOCV) with 36 subjects. The classification accuracy rate was 88.9%. PMID:26506352

  15. Isolated and bilateral simultaneous facial palsy disclosing early human immunodeficiency virus infection

    PubMed Central

    Sathirapanya, Pornchai

    2015-01-01

    Bilateral lower motor neuron type facial palsy is an unusual neurological disorder. There are few reports that associate it with the human immunodeficiency virus (HIV) infection on initial presentation. A 51-year-old married woman, who was previously healthy and had no risk of HIV infection, presented solely with bilateral simultaneous facial palsy. A positive HIV serology test was confirmed by an enzyme-linked immunosorbent assay test. Following a short course of oral prednisolone, the patient recovered completely from facial palsy in three months, even though an antiretroviral treatment was suspended. Exclusion of HIV infection in patients with bilateral facial palsy is essential for early diagnosis and management of HIV. PMID:26106247

  16. Platelet-Rich Plasma in a Patient with Cerebral Palsy

    PubMed Central

    Alcaraz, Jesús; Oliver, Antonio; Sánchez, Juana María

    2015-01-01

    Patient: Male, 6 Final Diagnosis: Cerebral palsy secundary perinatal hypoxia Symptoms: Cognitive impairment • epilectic seizure Medication: Platelet rich plasma Clinical Procedure: Cognitive improvement with neuroestimulator and neuroregenerator power of platelet rich plasma injection Specialty: Hematology Objective: Unusual clinical course Background: The use of platelet-rich plasma is a now a common medical technique known as regenerative medicine, through power cell activation and differentiation, which produces growth factors called platelets derived both locally and systematically. Here, we report the case of a cerebral palsy patient who received intravenous platelet-rich plasma. Case Report: We administered an intravenous injection of concentrated platelet-rich plasma (25 cc) in a 6-year-old boy with perinatal cerebral palsy, cognitive impairment, and marked and severe generalized spasticity. We performed follow-up at 3 and 6 months after the injection. All serum samples for determination were obtained by ELISA technique. Cognitive scales (Bayley, Battelle, M.S.C.A, Kaufman ABC, and Stanford-Binet Intelligence scale) were used before and after treatment. The determination protocol that was applied before the analysis was performed manually and the autotransfusion was considered suitable for treatment. We determined the plasma levels of factor similar to insulin-1 (IGF-1), platelet-derived growth factor (PDGF), vasculo-endothelial growth factor (VEGF), and transforming growth factor B (TGF-B) before and during treatment monitoring. Conclusions: No adverse effects were observed in the patient except for a small hematoma in the area channeling venous access. We observed a clear improvement in the cognitive sphere (memory, ability to perform more complex tasks, and acquisition of new skills) and in language, maintaining stable levels of growth factor in plasma 3–5 times higher than average for his age group at both 3- and 6-month follow-up. Positron emission tomography (PET) images showed an evident increased demarcation in the cerebral cortex. We propose that this therapy is useful in these patients to harness the neurostimulative and neuroregenerative power of endogenous growth factors derived from platelets. PMID:26185982

  17. Effects of Prolonged Standing on Gait in Children with Spastic Cerebral Palsy

    ERIC Educational Resources Information Center

    Salem, Yasser; Lovelace-Chandler, Venita; Zabel, Reta J.; McMillan, Amy Gross

    2010-01-01

    The purpose of this study was to determine the effects of prolonged standing on gait characteristics in children with spastic cerebral palsy. Six children with spastic cerebral palsy participated in this study with an average age of 6.5 years (SD = 2.5, range = 4.0-9.8 years). A reverse baseline design (A-B-A) was used over a 9-week period. During…

  18. Peripheral facial palsy in the past: contributions from Avicenna, Nicolaus Friedreich and Charles Bell.

    PubMed

    Resende, Luiz Antonio de Lima; Weber, Silke

    2008-09-01

    This study provides historical documents of peripheral facial palsy from Egypt, Greece and Rome, through the middle ages, and the renaissance, and into the last four centuries. We believe that the history of peripheral facial palsy parallels history of the human race itself. Emphasis is made on contributions by Avicenna and Nicolaus Friedreich. Controversies about the original clinical description by Charles Bell are also discussed. PMID:18949283

  19. Effects of Prolonged Standing on Gait in Children with Spastic Cerebral Palsy

    ERIC Educational Resources Information Center

    Salem, Yasser; Lovelace-Chandler, Venita; Zabel, Reta J.; McMillan, Amy Gross

    2010-01-01

    The purpose of this study was to determine the effects of prolonged standing on gait characteristics in children with spastic cerebral palsy. Six children with spastic cerebral palsy participated in this study with an average age of 6.5 years (SD = 2.5, range = 4.0-9.8 years). A reverse baseline design (A-B-A) was used over a 9-week period. During

  20. Laser Posterior Cordectomy for Bilateral Abductor Vocal Cord Palsy : A Case Report

    PubMed Central

    Mohamad, Irfan; Jihan, Wan Shah; Mohamad, Hazama; Abdullah, Baharuddin

    2008-01-01

    Bilateral abductor vocal cord palsy is comparatively a rare vocal cord lesion, especially in a patient with no history of neck mass, previous surgery or trauma. Many patients are not stridulous. A patient presenting with stridor may need emergency airway management before the other treatment is commenced. We report a case of bilateral abductor palsy which required an emergency tracheostomy and subsequently a laser posterior cordectomy. PMID:22589614

  1. Visual disorders in children with brain lesions: 2. Visual impairment associated with cerebral palsy.

    PubMed

    Guzzetta, A; Mercuri, E; Cioni, G

    2001-01-01

    Disorders of visual function are a common finding in children with cerebral palsy. In some cases they are secondary to ophthalmologic abnormalities such as cataract or retinopathy, but more often they are due to damage of the central visual pathway. We review the literature on the prevalence and distribution of visual abnormalities in children with cerebral palsy and their relation to cognitive, motor and emotional development. PMID:11589165

  2. Orthopaedic aspects of children with infectious (central nervous system) postnatal cerebral palsy.

    PubMed

    Loder, R T

    1992-01-01

    Twenty-two children (15 girls, 7 boys) with postnatal infectious cerebral palsy were reviewed at an average age of 8 years. Orthopaedic deformities were noted in 82% of all children and involved the upper extremity (41%), spine (45%), hips (68%), knees (32%), and feet and ankles (55%). Both typical and atypical cerebral palsy deformities were noted, often severe. Surgical correction of deformities achieved 77% satisfactory results. Bony procedures did better than soft tissue procedures, especially around the hip. PMID:1613101

  3. An unusual presentation of Bell's palsy: A case report and review of literature

    PubMed Central

    McFarlin, Anna; Peckler, Bradley

    2008-01-01

    In clinical medicine there may be times when clinical conditions manifest differently both when they present individually or concomitantly. Such scenarios warrant a broader differential diagnosis with thorough investigations. We present one such case of a patient of Bell's palsy with unexplained eye pain on the ipsilateral side. The patient had a chronic retinal detachment which became worse due to the concomitant Bell's palsy. PMID:19561942

  4. A Clinical Study of Autologous Bone Marrow Mononuclear Cells for Cerebral Palsy Patients: A New Frontier

    PubMed Central

    Sharma, Alok; Sane, Hemangi; Gokulchandran, Nandini; Kulkarni, Pooja; Sundaram, Jyothi; Paranjape, Amruta; Shetty, Akshata; Bhagwanani, Khushboo; Biju, Hema; Badhe, Prerna

    2015-01-01

    Cerebral palsy is a nonprogressive heterogeneous group of neurological disorders with a growing rate of prevalence. Recently, cellular therapy is emerging as a potential novel treatment strategy for cerebral palsy. The various mechanisms by which cellular therapy works include neuroprotection, immunomodulation, neurorestoration, and neurogenesis. We conducted an open label, nonrandomized study on 40 cases of cerebral palsy with an aim of evaluating the benefit of cellular therapy in combination with rehabilitation. These cases were administered autologous bone marrow mononuclear cells intrathecally. The follow-up was carried out at 1 week, 3 months, and 6 months after the intervention. Adverse events of the treatment were also monitored in this duration. Overall, at six months, 95% of patients showed improvements. The study population was further divided into diplegic, quadriplegic, and miscellaneous group of cerebral palsy. On statistical analysis, a significant association was established between the symptomatic improvements and cell therapy in diplegic and quadriplegic cerebral palsy. PET-CT scan done in 6 patients showed metabolic improvements in areas of the brain correlating to clinical improvements. The results of this study demonstrate that cellular therapy may accelerate the development, reduce disability, and improve the quality of life of patients with cerebral palsy. PMID:25788947

  5. De novo point mutations in patients diagnosed with ataxic cerebral palsy.

    PubMed

    Parolin Schnekenberg, Ricardo; Perkins, Emma M; Miller, Jack W; Davies, Wayne I L; D'Adamo, Maria Cristina; Pessia, Mauro; Fawcett, Katherine A; Sims, David; Gillard, Elodie; Hudspith, Karl; Skehel, Paul; Williams, Jonathan; O'Regan, Mary; Jayawant, Sandeep; Jefferson, Rosalind; Hughes, Sarah; Lustenberger, Andrea; Ragoussis, Jiannis; Jackson, Mandy; Tucker, Stephen J; Németh, Andrea H

    2015-07-01

    Cerebral palsy is a sporadic disorder with multiple likely aetiologies, but frequently considered to be caused by birth asphyxia. Genetic investigations are rarely performed in patients with cerebral palsy and there is little proven evidence of genetic causes. As part of a large project investigating children with ataxia, we identified four patients in our cohort with a diagnosis of ataxic cerebral palsy. They were investigated using either targeted next generation sequencing or trio-based exome sequencing and were found to have mutations in three different genes, KCNC3, ITPR1 and SPTBN2. All the mutations were de novo and associated with increased paternal age. The mutations were shown to be pathogenic using a combination of bioinformatics analysis and in vitro model systems. This work is the first to report that the ataxic subtype of cerebral palsy can be caused by de novo dominant point mutations, which explains the sporadic nature of these cases. We conclude that at least some subtypes of cerebral palsy may be caused by de novo genetic mutations and patients with a clinical diagnosis of cerebral palsy should be genetically investigated before causation is ascribed to perinatal asphyxia or other aetiologies. PMID:25981959

  6. De novo point mutations in patients diagnosed with ataxic cerebral palsy

    PubMed Central

    Parolin Schnekenberg, Ricardo; Perkins, Emma M.; Miller, Jack W.; Davies, Wayne I. L.; D’Adamo, Maria Cristina; Pessia, Mauro; Fawcett, Katherine A.; Sims, David; Gillard, Elodie; Hudspith, Karl; Skehel, Paul; Williams, Jonathan; O’Regan, Mary; Jayawant, Sandeep; Jefferson, Rosalind; Hughes, Sarah; Lustenberger, Andrea; Ragoussis, Jiannis

    2015-01-01

    Cerebral palsy is a sporadic disorder with multiple likely aetiologies, but frequently considered to be caused by birth asphyxia. Genetic investigations are rarely performed in patients with cerebral palsy and there is little proven evidence of genetic causes. As part of a large project investigating children with ataxia, we identified four patients in our cohort with a diagnosis of ataxic cerebral palsy. They were investigated using either targeted next generation sequencing or trio-based exome sequencing and were found to have mutations in three different genes, KCNC3, ITPR1 and SPTBN2. All the mutations were de novo and associated with increased paternal age. The mutations were shown to be pathogenic using a combination of bioinformatics analysis and in vitro model systems. This work is the first to report that the ataxic subtype of cerebral palsy can be caused by de novo dominant point mutations, which explains the sporadic nature of these cases. We conclude that at least some subtypes of cerebral palsy may be caused by de novo genetic mutations and patients with a clinical diagnosis of cerebral palsy should be genetically investigated before causation is ascribed to perinatal asphyxia or other aetiologies. PMID:25981959

  7. Deep peroneal nerve palsy with isolated lateral compartment syndrome secondary to peroneus longus tear: a report of two cases and a review of the literature.

    PubMed

    Hiramatsu, Kunihiko; Yonetani, Yasukazu; Kinugasa, Kazutaka; Nakamura, Norimasa; Yamamoto, Koji; Yoshikawa, Hideki; Hamada, Masayuki

    2016-06-01

    Drop foot is typically caused by neurologic disease such as lumbar disc herniation, but we report two rare cases of deep peroneal nerve palsy with isolated lateral compartment syndrome secondary to peroneus longus tears. Both patients developed mild pain in the lower legs while playing sport, and were aware of drop foot. As compartment pressures were elevated, fasciotomy was performed immediately, and the tendon of the peroneus longus was completely detached from its proximal origin. The patients were able to return their original sports after 3 months, and clinical examination revealed no hypesthesia or muscle weakness in the deep peroneal nerve area at the time of last follow-up. The common peroneal nerve pierced the deep fascia and lay over the fibular neck, which formed the floor of a short tunnel (the so-called fibular tunnel), then passed the lateral compartment just behind the peroneus longus. The characteristic anatomical situation between the fibular tunnel and peroneus longus might have caused deep peroneal nerve palsy in these two cases after hematoma adjacent to the fibular tunnel increased lateral compartment pressure. PMID:26362782

  8. Systemic inflammation and cerebral palsy risk in extremely preterm infants

    PubMed Central

    Kuban, KCK; O’Shea, TM; Allred, EN; Paneth, N; Hirtz, D; Fichorova, RN; Leviton, A

    2013-01-01

    We hypothesized that among extremely preterm infants, elevated concentrations of inflammation-related proteins in neonatal blood are associated with cerebral palsy (CP) at 24 months. Methods In 939 infants born before 28 weeks gestation, we measured blood concentrations of 25 proteins on postnatal days 1, 7, and 14 and evaluated associations between elevated protein concentrations and CP diagnosis. Results Protein elevations within three days of birth were not associated with CP. Elevations of TNF-α, TNF-R1, IL-8, ICAM-1, on at least two days were associated with diparesis. Recurrent-persistent elevations of IL-6, E-SEL, or IGFBP-1 were associated with hemiparesis. Diparesis and hemiparesis were more likely among infants who had at least four of nine proteins elevations that previously have been associated with cognitive impairment and microcephaly. Interpretation Repeated elevations of inflammation-related proteins during the first two postnatal weeks are associated with increased risk of CP. PMID:24646503

  9. Therapeutic potential of autologous stem cell transplantation for cerebral palsy.

    PubMed

    Purandare, Chaitanya; Shitole, D G; Belle, Vaijayantee; Kedari, Aarti; Bora, Neeta; Joshi, Meghnad

    2012-01-01

    Background. Cerebral palsy (CP) is a severe disabling disease with worldwide incidence being 2 to 3 per 1000 live births. CP was considered as a noncurable, nonreparative disorder, but stem cell therapy offers a potential treatment for CP. Objective. The present study evaluates the safety and efficacy of autologous bone-marrow-derived mononuclear cell (BMMNCs) transplantation in CP patient. Material and Methods. In the present study, five infusions of autologous stem cells were injected intrathecally. Changes in neurological deficits and improvements in function were assessed using Gross Motor Function Classification System (GMFCS-E&R) scale. Results. Significant motor, sensory, cognitive, and speech improvements were observed. Bowel and bladder control has been achieved. On the GMFCS-E&R level, the patient was promoted from grade III to I. Conclusion. In this study, we report that intrathecal infusion of autologous BMMNCs seems to be feasible, effective, and safe with encouraging functional outcome improvements in CP patient. PMID:23259143

  10. Therapeutic Potential of Autologous Stem Cell Transplantation for Cerebral Palsy

    PubMed Central

    Purandare, Chaitanya; Shitole, D. G.; Belle, Vaijayantee; Kedari, Aarti; Bora, Neeta; Joshi, Meghnad

    2012-01-01

    Background. Cerebral palsy (CP) is a severe disabling disease with worldwide incidence being 2 to 3 per 1000 live births. CP was considered as a noncurable, nonreparative disorder, but stem cell therapy offers a potential treatment for CP. Objective. The present study evaluates the safety and efficacy of autologous bone-marrow-derived mononuclear cell (BMMNCs) transplantation in CP patient. Material and Methods. In the present study, five infusions of autologous stem cells were injected intrathecally. Changes in neurological deficits and improvements in function were assessed using Gross Motor Function Classification System (GMFCS-E&R) scale. Results. Significant motor, sensory, cognitive, and speech improvements were observed. Bowel and bladder control has been achieved. On the GMFCS-E&R level, the patient was promoted from grade III to I. Conclusion. In this study, we report that intrathecal infusion of autologous BMMNCs seems to be feasible, effective, and safe with encouraging functional outcome improvements in CP patient. PMID:23259143

  11. Cycling patterns in children with and without cerebral palsy.

    PubMed

    Kaplan, S L

    1995-07-01

    Pedaling smoothness and electromyography patterns were quantified in children with spastic diplegic cerebral palsy (CP) and a cohort of children with typical development. Video analysis of the pedaling rhythm yielded equivalent time periods for the control group and irregular time periods in the group with CP, with greater time spent at the bottom of the pedaling cycle. Electromyography patterns of the tibialis anterior, rectus femoris, medial hamstring and lateral gastrocnemius muscle groups yielded greater percentages of muscle activity time and co-contraction time at both the ankle and knee in the group with CP; however, the control group had longer ankle co-contraction times than were expected from previous adult studies. PMID:7615148

  12. Botulinum Toxin Treatment for Limb Spasticity in Childhood Cerebral Palsy.

    PubMed

    Pavone, Vito; Testa, Gianluca; Restivo, Domenico A; Cannavò, Luca; Condorelli, Giuseppe; Portinaro, Nicola M; Sessa, Giuseppe

    2016-01-01

    CP is the most common cause of chronic disability in childhood occurring in 2-2.5/1000 births. It is a severe disorder and a significant number of patients present cognitive delay and difficulty in walking. The use of botulinum toxin (BTX) has become a popular treatment for CP especially for spastic and dystonic muscles while avoiding deformity and pain. Moreover, the combination of physiotherapy, casting, orthotics and injection of BTX may delay or decrease the need for surgical intervention while reserving single-event, multi-level surgery for fixed musculotendinous contractures and bony deformities in older children. This report highlights the utility of BTX in the treatment of cerebral palsy in children. We include techniques for administration, side effects, and possible resistance as well as specific use in the upper and lower limbs muscles. PMID:26924985

  13. Delayed diagnosis of neuroborreliosis presenting as bell palsy and meningitis.

    PubMed

    Smith, Irving Shelby; Rechlin, David P

    2010-08-01

    Lyme disease is most prevalent in the northeast and upper Midwest regions of the United States. While early symptoms may be mild (eg, rash, flu-like symptoms, joint pain), late or persistent infection can cause chronic neurologic impairments. Because of this range of symptoms, physicians can have difficulty diagnosing Lyme disease, especially in the absence of erythema chronicum migrans. We report a case of a woman who initially presented with severe vertigo and vomiting and later with fever, headache, and facial droop. After more than 3 weeks of misdiagnosis, the patient tested positive for Lyme disease and was diagnosed as having neuroborreliosis presenting as Bell palsy and meningitis. The authors review the history, diagnosis, and management of Lyme disease. PMID:20805550

  14. The Role of Prematurity in Patients With Hemiplegic Cerebral Palsy.

    PubMed

    Zelnik, Nathanel; Lahat, Eli; Heyman, Eli; Livne, Amir; Schertz, Mitchell; Sagie, Liora; Fattal-Valevski, Aviva

    2016-05-01

    A multicenter retrospective study was conducted to investigate the perinatal factors, imaging findings and clinical characteristics of hemiplegic cerebral palsy with a particular focus on children born prematurely. Our cohort included 135 patients of whom 42% were born prematurely; 16% were extreme premature infants who were born at 30 weeks or earlier. Nineteen (14%) were twins. Right hemiplegia was slightly more common and accounted for 59% of the patients. Imaging findings of intraventricular hemorrhage and periventricular leukomalacia were more prevalent in premature children whereas stroke, porencephaly, cerebral hemorrhage and cerebral atrophy were more evenly distributed in both term-born and prematurely-born children (p< 0.01). The overall prevalence of epilepsy in the cohort was 26% with no differences in full-term compared to prematurely-born children. Regardless of the gestational birth age, intellectual deficits were more common in the presence of comorbidity of both hemiplegia and epilepsy (p< 0.05). PMID:26500242

  15. Nuclear facial palsy in multiple sclerosis: a case report.

    PubMed

    Schnorpfeil, F; Braune, H J

    1997-01-01

    We encountered an unusual case of isolated subacute nuclear palsy of the facial nerve in a 31-year-old man with a 3-year history of clinically established relapsing form of multiple sclerosis. Typical positive sharp waves and fibrillation potentials in the affected facial muscles detected by means of electromyography showed that the lesion was located in the lower motor neuron. Transcranial magnetic stimulation of the cortex could not evoke normal compound motor unit potentials, however, transcranial magnetic stimulation of the facial nerve, which is strongly supposed to depolarize the peripheral nerve in the area of the root exit zone near the internal acoustic meatus, elicited an adequate response. Taking these findings as well as the results of positive magnetic resonance imaging into consideration, we concluded that the clinical symptom was due to a plaque of demyelinization in the nucleus of the facial nerve or in the proximal intramedullary course of facial fibres. PMID:9208215

  16. Reliability of balance evaluation in children with cerebral palsy.

    PubMed

    Iatridou, G; Dionyssiotis, Y

    2013-10-01

    The evaluation of balance in children with cerebral palsy (CP) is an extremely difficult and complex procedure. The purpose of the present study was the evaluation of three balance tests: Berg Balance Scale (BBS), time up and go (TUG) and Bruininks-Oseretsky Test of Motor Proficiency (BOTMP) and their reliability in children with CP. The control of the reliability of the tests was based on the successive application to twenty children with CP within a short period in order to substantiate their ability to give the same results in a stable sample. No difference was found according to scores between the measures but important statistical differences were found of the scores of the children in all tests. The tests BBS, TUG and BOTMP are considered to be reliable and valid tests, able to objectively define the quantitative mutation of the balance of the child in test with CP. PMID:25031506

  17. Treatment for facial neuronitis: a new approach to Bell's palsy.

    PubMed

    Pisonero, P; Vallejo, L; Menéndez, E; Evangelista, C R; Alonso, A

    1991-01-01

    The presence of sensitive symptoms, symptoms of parasympathetic ganglion's affection, as well as signs of affection of other cranial nerves accompanying to Bell's palsy, led us to consider and treat this disorder as a symptom of motor lesion of a possible primary neuron inflammation. Although we have not found valid statistical correlations between the lesion and location patterns, we have found, however, that continues electromyographic studies, and the early treatment carried out, may be of great interest to avoid sequels. The treatment was focussed to eliminate both the possible cause and the inflammatory process, as to improve neuron regeneration. From 60 patients selected to enter this study, 91,66 percent recovered the motor function. PMID:1716859

  18. Hip fusion as hip salvage procedure in cerebral palsy.

    PubMed

    Fucs, Patricia M De Moraes Barros; Yamada, Helder H

    2014-01-01

    The treatment of the spastic hip in Cerebral Palsy (CP) remains a challenge especially in cases of advance changes. Many options are available and the key for a good outcome is to find the best surgical procedure to an individualized patient. The hip fusion is one of the surgical options. The authors presented a group of spastic CP with painful chronic hip subluxation and dislocation treated with hip fusion with a mean follow-up period of 14.5 years. Surgical technique, post-operative management and outcomes were shown, also with the observations done regarding the evolution of the contralateral hip after the hip fusion. They concluded that the hip arthrodesis is an option for patients with spastic CP with painful subluxation or dislocated hips with the goal of pain relief maintain or improve functional status, and facilitating the care. The best candidate is a young ambulatory patient with normal contralateral hip and normal spinal alignment. PMID:25207734

  19. Cerebral palsy and developmental coordination disorder in children born preterm.

    PubMed

    Spittle, Alicia Jane; Orton, Jane

    2014-04-01

    Children born early (<37 weeks of gestation) are at high risk of a range of motor impairments due to a variety of biological and environmental risk factors. Cerebral palsy occurs more frequently in those children born preterm, with the risk increasing with decreasing gestational age. Mild and moderate motor impairments, consistent with developmental coordination disorder, occur in almost half of those children born preterm and include difficulties with balance, manual dexterity and ball skills. All forms of motor impairment are associated with comorbidities, which may have a greater effect on quality of life, academic achievement and participation in extracurricular activities than the motor impairment itself. Infants at risk of motor impairment can be identified in early infancy with a combination of clinical assessment tools and perinatal risk factors. However, the reliable diagnosis of motor impairment requires follow-up into early childhood and it is important to ensure that the appropriate intervention is implemented. PMID:24290908

  20. Bone health in children with cerebral palsy and epilepsy.

    PubMed

    Aronson, Elizabeth; Stevenson, Sharon B

    2012-01-01

    Children with disabilities that limit mobility are at increased risk for osteoporosis. In the United States, 10 million people have osteoporosis and 34 million people are estimated to be at risk of acquiring this condition. Typically, bone fragility and osteoporosis have been associated with older adults; however, these problems can also affect children. The childhood and adolescent years are critically important in producing healthy bone mass. Yet cerebral palsy and epilepsy, which are both chronic disorders that frequently coexist, are predictors of muscular and skeletal compromise. Nurse practitioners should be aware of recommendations for promoting and achieving optimal bone health in children with these disabilities and screening patients who are at risk of sustaining fractures. PMID:22525999

  1. Rehabilitation of Bell's palsy patient with complete dentures.

    PubMed

    Muthuvignesh, J; Kumar, N Suman; Reddy, D Narayana; Rathinavelu, Pradeep; Egammai, S; Adarsh, A

    2015-08-01

    Facial nerve disorders may be of sudden onset and more often of unknown etiology. Edema of the facial nerve within the fallopian canal results in Bell's palsy. This causes compression of the nerve and affects the microcirculation. Many authors have suggested treatment for facial nerve paralysis ranging from simple physiotherapy to complicated microvascular decompression. It more often results in symptoms like synkinesis and muscle spasm after the decompression surgery of the nerve because of the inability to arrange the nerve fibers within the canal. The treatment choice also depends on patient's age, extent of the nerve damage, and patient's needs and desires. Many patients who cannot be rehabilitated functionally can be treated for esthetics of the involved muscles. This case report elaborates about a patient who was rehabilitated for esthetics and to some extent for function. PMID:26538967

  2. Rehabilitation of Bell's palsy patient with complete dentures

    PubMed Central

    Muthuvignesh, J.; Kumar, N. Suman; Reddy, D. Narayana; Rathinavelu, Pradeep; Egammai, S.; Adarsh, A.

    2015-01-01

    Facial nerve disorders may be of sudden onset and more often of unknown etiology. Edema of the facial nerve within the fallopian canal results in Bell's palsy. This causes compression of the nerve and affects the microcirculation. Many authors have suggested treatment for facial nerve paralysis ranging from simple physiotherapy to complicated microvascular decompression. It more often results in symptoms like synkinesis and muscle spasm after the decompression surgery of the nerve because of the inability to arrange the nerve fibers within the canal. The treatment choice also depends on patient's age, extent of the nerve damage, and patient's needs and desires. Many patients who cannot be rehabilitated functionally can be treated for esthetics of the involved muscles. This case report elaborates about a patient who was rehabilitated for esthetics and to some extent for function. PMID:26538967

  3. A case study on the Ayurvedic management of cerebral palsy

    PubMed Central

    Bhinde, Sagar Mahendrabhai

    2015-01-01

    Cerebral palsy (CP) is the leading cause of childhood disability affecting function and development. CP is defined as a nonprogressive neuromotor disorder of cerebral origin. It cannot be correlated with any single disease or condition in Ayurveda, as it is a multi-factorial disease with clinical features of a wide variation. According to Vāgbhaṭa, it is classified in the disease categories of sahaja (hereditary) and garbhaja (congenital) and jātaja (psychosomatic) type of diseases. Of the many types and subtypes of CP, none has any known “cure.” Here, an effort was made to treat a 3-year-old male child with spastic type of CP using multiple Ayurveda treatment modalities. At the end of 94 days of treatment, Pañcakarma procedures along with internal medication resulted in 10–15% improvement in the overall effect of therapy. PMID:26120232

  4. Care of Adults With Intellectual and Developmental Disabilities: Cerebral Palsy.

    PubMed

    Jones, Kyle Bradford; Wilson, Benjamin; Weedon, Dean; Bilder, Deborah

    2015-12-01

    Cerebral palsy (CP) is a group of disorders that primarily affect motor function. This developmental disability is becoming more common in adults as life expectancy increases for individuals with CP. Many physical, medical, mental, and behavioral health conditions are associated with CP, and assistance should be provided to patients with CP to optimize function, when available. These comorbidities include intellectual disabilities, seizures, muscle contractures, abnormal gait, osteoporosis, communication disorders, malnutrition, sleep disorders, and mental health disorders, such as depression and anxiety. The physician should be familiar with screening for and assisting patients with these issues. Optimizing quality of life requires individualized care plans that may include physical therapy, muscle relaxants, surgery, and nutritional support. Other issues to be addressed include methods to facilitate employment; sexual concerns; and support through local and national organizations for patients, families, and caregivers. PMID:26669212

  5. Inspection Time and ADHD Symptoms in Children with Cerebral Palsy

    PubMed Central

    Shank, Laura K.; Kaufman, Jacqueline; Leffard, Stacie; Warschausky, Seth

    2010-01-01

    Objective To examine between-group differences in the associations between aspects of processing speed assessed with an inspection time task, and attention-deficit/hyperactivity disorder (ADHD) symptoms. Research Design Two groups comprised of 34 children with cerebral palsy (CP) and 70 nonaffected peers (Control), ages 8 – 16, participated in a prospective correlational study. Measures included a visual inspection time task and the Conners’ Parent Rating Scale – Revised: Long Version. Results Children with CP exhibited significantly slower processing speed, and more symptoms of inattention and hyperactivity than Controls. Significant associations between inspection time and ADHD symptoms were found only in the Control group. Conclusions Findings have implications for clinical assessment and understanding of attentional risks associated with CP. PMID:20496973

  6. Botulinum Toxin Treatment for Limb Spasticity in Childhood Cerebral Palsy

    PubMed Central

    Pavone, Vito; Testa, Gianluca; Restivo, Domenico A.; Cannavò, Luca; Condorelli, Giuseppe; Portinaro, Nicola M.; Sessa, Giuseppe

    2016-01-01

    CP is the most common cause of chronic disability in childhood occurring in 2–2.5/1000 births. It is a severe disorder and a significant number of patients present cognitive delay and difficulty in walking. The use of botulinum toxin (BTX) has become a popular treatment for CP especially for spastic and dystonic muscles while avoiding deformity and pain. Moreover, the combination of physiotherapy, casting, orthotics and injection of BTX may delay or decrease the need for surgical intervention while reserving single-event, multi-level surgery for fixed musculotendinous contractures and bony deformities in older children. This report highlights the utility of BTX in the treatment of cerebral palsy in children. We include techniques for administration, side effects, and possible resistance as well as specific use in the upper and lower limbs muscles. PMID:26924985

  7. Thoracolumbar scoliosis in cerebral palsy. Results of surgical treatment.

    PubMed

    Bonnett, C; Brown, J C; Grow, T

    1976-04-01

    Of 294 patients with cerebral palsy seen from 1960 to 1972, forty-two had clinically significant lumbar and thoracolumbar scoliosis (31 to 135 degrees) and thirty-three were treated by spine surgery: ten by Harrington instrumentation and posterior spine fusion, eighteen by the Dwyer procedure and anterior fusion, and five by a two-stage combined anterior and posterior fusion. Evaluation of the results after eighteen to sixty-eight months showed: relief of pain in seventeen cases, improved sitting tolerance in seventeen, less nursing care needed in three, less equipment required in six, ability to use equipment providing more function in three, placement in a facility where less care was required in two, and improved eating patterns in two. Only the combined procedure appeared to give adequate correction and a low incidence of pseudarthrosis. PMID:1262364

  8. Pain, motor function and health-related quality of life in children with cerebral palsy as reported by their physiotherapists

    PubMed Central

    2014-01-01

    Background Children and adolescents with cerebral palsy suffer from higher levels of pain than their peers without disability. The aim of this study was to explore the impact of pain on health-related quality of life and motor function in individuals with cerebral palsy as reported by health professionals. Methods Cross-sectional study carried out in Associations for Care of Individuals with Cerebral Palsy and Related Disabilities (ASPACE) in Balearic Islands and Castile Leon (Spain). Thirty-five physiotherapists rated pain, health-related quality of life and motor function in 91 children and adolescents with cerebral palsy [8-19y]. A semi-structured interview was used to collect demographic and clinical data according with the Study of Participation of Children with Cerebral Palsy Living in Europe (SPARCLE). Results Physiotherapists reported that 51% of individuals with cerebral palsy suffered from pain. Physiotherapists also perceived that pain in individuals with cerebral palsy was responsible for reductions of psychological but not physical domains of health-related quality of life. According with physiotherapists estimations, motor impairment scores were not correlated with pain scores in individuals with cerebral palsy, but they were significantly associated with physical and autonomy domains of health-related quality of life. Conclusions These findings highlighted the importance of assessing and providing interventions for pain relief in persons with cerebral palsy even at an early age. PMID:25066900

  9. Effect of postural insoles on static and functional balance in children with cerebral palsy: A randomized controlled study

    PubMed Central

    Christovão, Thaluanna C. L.; Pasini, Hugo; Grecco, Luanda A. C.; Ferreira, Luiz A. B.; Duarte, Natália A. C.; Oliveira, Cláudia S.

    2015-01-01

    BACKGROUND: Improved gait efficiency is one of the goals of therapy for children with cerebral palsy (CP). Postural insoles can allow more efficient gait by improving biomechanical alignment. OBJECTIVE: The aim of the present study was to determine the effect of the combination of postural insoles and ankle-foot orthoses on static and functional balance in children with CP. METHOD: A randomized, controlled, double-blind, clinical trial. After meeting legal requirements and the eligibility criteria, 20 children between four and 12 years of age were randomly allocated either to the control group (CG) (n=10) or the experimental group (EG) (n=10). The CG used placebo insoles and the EG used postural insoles. The Berg Balance Scale, Timed Up-and-Go Test, Six-Minute Walk Test, and Gross Motor Function Measure-88 were used to assess balance as well as the determination of oscillations from the center of pressure in the anteroposterior and mediolateral directions with eyes open and closed. Three evaluations were carried out: 1) immediately following placement of the insoles; 2) after three months of insole use; and 3) one month after suspending insole use. RESULTS: The EG achieved significantly better results in comparison to the CG on the Timed Up-and-Go Test as well as body sway in the anteroposterior and mediolateral directions. CONCLUSION: Postural insoles led to an improvement in static balance among children with cerebral palsy, as demonstrated by the reduction in body sway in the anteroposterior and mediolateral directions. Postural insole use also led to a better performance on the Timed Up-and-Go Test. PMID:25651134

  10. The phenotype of the Gly94fsX222 PMP22 insertion.

    PubMed

    de Vries, Sara D J; Verhamme, Camiel; van Ruissen, Fred; van Paassen, Barbara W; Arts, Willem F; Kerkhoff, Henk; van Engelen, Baziel G M; Lammens, Martin; de Visser, Marianne; Baas, Frank; van der Kooi, Anneke J

    2011-06-01

    Point mutations in PMP22 are relatively rare and the phenotype may vary from mild hereditary neuropathy with liability to pressure palsies (HNPP) to severe Charcot-Marie-Tooth type 1 (CMT1). We describe the phenotype of the Gly94fsX222 mutation in the PMP22 gene. Medical records of all patients were reviewed and 11 patients were re-examined. EMG was carried out in nine patients and nerve biopsy in one. Thirteen patients originating from seven families with a Gly94fsX222 mutation were included and consisted of 10 women and 3 men with a median age of 41 years (range 7-67). Five index patients were originally suspected of CMT1. Ten patients had abnormal motor skills during childhood. Nine patients had a history of pressure palsies. Involvement of the olfactory, trigeminal, facial, and pudendal nerves occurred in three patients. Twelve patients had pes cavus and one scoliosis. Distal anterior leg and distal arm weakness were found in 12 and 4 patients, respectively. Twelve patients had distal leg sensory abnormalities. Electrophysiological examination revealed a demyelinating sensorimotor neuropathy, both resembling CMT1 and HNPP. Sural nerve biopsy showed demyelinating neuropathy with presence of tomacula. More than three-fourths of the patients with Gly94fsX222 mutation demonstrated a CMT1 phenotype combined with transient deficits. Clinicians should test for this mutation in those patients exhibiting a generalised neuropathy combined with compressive like episodes. PMID:21692910

  11. The 5' regulatory sequence of the PMP22 in the patients with Charcot-Marie-Tooth disease.

    PubMed

    Sinkiewicz-Darol, Elena; Kabzińska, Dagmara; Moszyńska, Izabela; Kochański, Andrzej

    2010-01-01

    Little is known about the molecular background of clinical variability of Charcot-Marie-Tooth type 1A (CMT1A) disease and hereditary neuropathy with liability to pressure palsies (HNPP). The CMT1A and HNPP disorders result from duplication and deletion of the PMP22 gene respectively. In a series of studies performed on affected animal transgenic models of CMT1A disease, expression of the PMP22 gene (gene dosage) was shown to correlete with severity of CMT course (gene dosage effect). In this study we hypothesized that single nucleotide polymorphisms (SNPs) located within the 5' regulatory sequence of PMP22 gene may be responsible for the CMT1A/HNPP clinical variability. We have sequenced the PMP22 5' upstream regulatory sequence in a group of 45 CMT1A/HNPP patients harboring the PMP22 duplication (37) /deletion (8). We have identified five SNPs in the regulatory sequence of the PMP22 gene. Three of them i.e. -819C>T, -4785G>T, -4800C>T were detected both in the patients and in the control group. Thus, their pathogenic role in the regulation of the expression of the PMP22 gene seems not to be significant. Two SNPs i.e. -4210T>C and -4759T>A were found only in the CMT patients. Their role in the regulation of the PMP22 gene expression can not be excluded. Additionally we have detected the Thr118Met variant in exon 4 of the PMP22 gene, which was previously reported by other authors, in one patient. We conclude that the 5' regulatory sequence of the PMP22 gene is conserved at the nucleotiode level, however rarely occurring SNPs variant in the PMP22 regulatory sequence may be associated with the gene dosage effect. PMID:20842290

  12. C5 Nerve root palsies following cervical spine surgery: A review

    PubMed Central

    Epstein, Nancy E.; Hollingsworth, Renee

    2015-01-01

    Background: Cervical C5 nerve root palsies may occur in between 0% and 30% of routine anterior or posterior cervical spine operations. They are largely attributed to traction injuries/increased cord migration following anterior/posterior decompressions. Of interest, almost all studies cite spontaneous resolution of these deficits without surgery with 3–24 postoperative months. Methods: Different studies cite various frequencies for C5 root palsies following anterior or posterior cervical spine surgery. In their combined anterior/posterior series involving C4-C5 level decompressions, Libelski et al. cited up to a 12% incidence of C5 palsies. In Gu et al. series, C5 root palsies occurred in 3.1% of double-door laminoplasty, 4.5% of open-door laminoplasty, and 11.3% of laminectomy. Miller et al. observed an intermediate 6.9% frequency of C5 palsies followed by posterior cervical decompressions and fusions (PCDF). Results: Gu et al. also identified multiple risk factors for developing C5 palsies following posterior surgery; male gender, ossification of the posterior longitudinal ligament (OPLL), narrower foramina, laminectomy, and marked dorsal spinal cord drift. Miller et al. also identified an average $1918 increased cost for physical/occupational therapy for patients with C5 palsies. Conclusions: The incidence of C5 root deficits for anterior/posterior cervical surgery at C4-C5 was 12% in one series, and ranged up to 11.3% for laminectomies, while others cited 0–30%. Although identification of preoperative risk factors for C5 root deficits may help educate patients regarding these risks, there is no clear method for their avoidance at this time. PMID:26005577

  13. New Predictive Parameters of Bell’s Palsy: Neutrophil to Lymphocyte Ratio and Platelet to Lymphocyte Ratio

    PubMed Central

    Atan, Doğan; İkincioğulları, Aykut; Köseoğlu, Sabri; Özcan, Kürşat Murat; Çetin, Mehmet Ali; Ensari, Serdar; Dere, Hüseyin

    2015-01-01

    Background: Bell’s palsy is the most frequent cause of unilateral facial paralysis. Inflammation is thought to play an important role in the pathogenesis of Bell’s palsy. Aims: Neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) are simple and inexpensive tests which are indicative of inflammation and can be calculated by all physicians. The aim of this study was to reveal correlations of Bell’s palsy and degree of paralysis with NLR and PLR. Study Design: Case-control study. Methods: The retrospective study was performed January 2010 and December 2013. Ninety-nine patients diagnosed as Bell’s palsy were included in the Bell’s palsy group and ninety-nine healthy individuals with the same demographic characteristics as the Bell’s palsy group were included in the control group. As a result of analyses, NLR and PLR were calculated. Results: The mean NLR was 4.37 in the Bell’s palsy group and 1.89 in the control group with a statistically significant difference (p<0.001). The mean PLR was 137.5 in the Bell’s palsy group and 113.75 in the control group with a statistically significant difference (p=0.008). No statistically significant relation was detected between the degree of facial paralysis and NLR and PLR. Conclusion: The NLR and the PLR were significantly higher in patients with Bell’s palsy. This is the first study to reveal a relation between Bell’s palsy and PLR. NLR and PLR can be used as auxiliary parameters in the diagnosis of Bell’s palsy. PMID:26167340

  14. When the Sensory Handicapped Child Has Cerebral Palsy. Part I: Physical Management. Part II: Deaf-Blind Curriculum and the Child with Cerebral Palsy. Working Papers in Developmental Disabilities.

    ERIC Educational Resources Information Center

    Fieber, Nancy M.

    The paper addresses issues involved in the management of and curriculum for children with sensory impairments combined with cerebral palsy. The first part details typical deformities and their causes in children with cerebral palsy, and describes (with illustrations) techniques for therapeutic handling and positioning in daily living tasks at home…

  15. Effect of a single session of transcranial direct-current stimulation combined with virtual reality training on the balance of children with cerebral palsy: a randomized, controlled, double-blind trial

    PubMed Central

    Lazzari, Roberta Delasta; Politti, Fabiano; Santos, Cibele Alimedia; Dumont, Arislander Jonathan Lopes; Rezende, Fernanda Lobo; Grecco, Luanda André Collange; Braun Ferreira, Luiz Alfredo; Oliveira, Claudia Santos

    2015-01-01

    [Purpose] The aim of the present study was to investigate the effects of a single session of transcranial direct current stimulation combined with virtual reality training on the balance of children with cerebral palsy. [Subjetcs and Methods] Children with cerebral palsy between four and 12 years of age were randomly allocated to two groups: an experimental group which performed a single session of mobility training with virtual reality combined with active transcranial direct current stimulation; and a control group which performed a single session of mobility training with virtual reality combined with placebo transcranial direct current stimulation. The children were evaluated before and after the training protocols. Static balance (sway area, displacement, velocity and frequency of oscillations of the center of pressure on the anteroposterior and mediolateral axes) was evaluated using a force plate under four conditions (30-second measurements for each condition): feet on the force plate with the eyes open, and with the eyes closed; feet on a foam mat with the eyes open, and with the eyes closed. [Results] An increase in sway velocity was the only significant difference found. [Conclusion] A single session of anodal transcranial direct current stimulation combined with mobility training elicited to lead to an increase in the body sway velocity of children with cerebral palsy. PMID:25931726

  16. Is There a Relationship Between Bell's Palsy and Internal Auditory Canal?

    PubMed

    Yilmaz, Hüseyin Baki; Safak Yalcin, Kadihan; Çakan, Doğan; Paksoy, Mustafa; Erdogan, Banu Atalay; Sanli, Arif

    2015-09-01

    In the present study, we evaluated the diameter of internal acoustic canal in patients with Bells palsy to investigate the role of anatomical differences of the temporal bone in etiology of Bell's palsy. Sixty-four patients who were diagnosed as Bells Palsy and temporal bone computed tomography imagings of them were included into the study group (Group 1). The control group (Group 2) was consisted of 35 healthy subjects without Bell's Palsy. All patients had temporal bone computed tomography imaging. The internal auditory canal inlet, mid-canal, outlet and canal lengths were measured at the most distinctive cross-section of the seventh and eighth cranial nerves bifurcation. In the study group, Bells palsy was on the right side in 26 patients (40.6 %) and on the left side in 38 patients (59.4 %). Initial House-Brackmann (HB) score was HB-2 in 29 patients (45.3 %), HB-3 in 18 patients (28.1 %), HB-4 in 13 patients (20.3 %) and HB-5 in 4 patients (6.2 %). At 6-month evaluation, HB-score of the patients were HB-1 in 37 patients (57.8 %), HB-2 in 25 patients (39.1 %) and HB-3 in 2 patients (3.1 %). Internal auditory canal (IAC) measurements of the groups showed that there were no significant differences between the measurements of right-mid canal, right canal length; and left canal outlet and left canal length of the study and control groups. Right inlet and outlet; and left inlet and mid-canal values of the study group (Bell's palsy) were significantly lower than those of the control group. In Bell's palsy group, left inlet, outlet and canal length values were significantly higher than those of the right ones. Correlation analysis showed that there were no significant correlation between paralysis side; initial HB stage; and IAC measurement results. In patients with higher initial HB score, their 6-month later HB-score was also higher. In patients with higher 6-month HB score; R canal inlet, R mid-canal, L-canal inlet, and L-mid canal values were lower. Lower IAC inlet and mid-canal values were detected in patients with Bell's palsy. Therefore narrow IAC inlet and mid-canal values may be one of the risk factors for Bell's palsy. PMID:26405671

  17. Analysis of correlative risk factors for C5 palsy after anterior cervical decompression and fusion

    PubMed Central

    Wang, Haiying; Zhang, Xu; Lv, Bing; Ding, Wenyuan; Shen, Yong; Yang, Dalong; Bai, Zhilong

    2015-01-01

    Background: It has been reported that C5 palsy is a potential complication of both anterior and posterior cervical spine surgery, although several mechanisms of C5 palsy following posterior cervical surgery have been proposed, few reports about correlative risk factors have been elaborated on C5 palsy after anterior cervical decompression and fusion (ACDF). Objective: The purpose of this study was to investigate the correlative risk factors of C5 palsy after anterior cervical decompression and fusion. Methods: This is a retrospective study. A total of 161 patients (108 males and 53 females) who underwent ACDF between 2007 and 2012 were included in this study. C5 palsy is characterized by deltoid and/or biceps brachii weakness. The patients were divided into two groups: one that had experienced C5 palsy (group A) and the other one had not (group B). In both groups, the age, gender, duration of disease, diagnosis, No. of surgical levels, cervical curvature correction, occupying rate of spinal canal at C4/5, diameter of the C4/5 foramen, intervertebral height variation, decompression width and preoperative high-signal intensity zone (HIZ) of spinal cord in T2-weighted MRI at C4/5 were measured and evaluated. The risk factors of C5 palsy were detected with logistic regression analysis. Results: There were no significant differences in age, gender, duration of disease, diagnosis, No. of surgical levels, rate of spinal canal at C4/5 and HIZ of spinal cord in T2-weighted MRI at C4/5. Cervical curvature correction, diameter of the C4/5 foramen, intervertebral height variation and decompression width had significant differences between the two groups (P<0.05). Logistic regression analysis revealed that cervical curvature, diameter of the C4/5 foramen, intervertebral height and decompression width were the pivotal risk factors for the incidence of C5 palsy. Conclusion: For patients with ACDF, greater cervical curvature correction, narrow diameter of the C4/5 foramen, improper intervertebral height variation and larger decompression width were the correlative risk factors for C5 palsy after ACDF. PMID:26064300

  18. Leg and Joint Stiffness in Children with Spastic Diplegic Cerebral Palsy during Level Walking

    PubMed Central

    Wang, Ting-Ming; Huang, Hsing-Po; Li, Jia-Da; Hong, Shih-Wun; Lo, Wei-Ching; Lu, Tung-Wu

    2015-01-01

    Individual joint deviations are often identified in the analysis of cerebral palsy (CP) gait. However, knowledge is limited as to how these deviations affect the control of the locomotor system as a whole when striving to meet the demands of walking. The current study aimed to bridge the gap by describing the control of the locomotor system in children with diplegic CP in terms of their leg stiffness, both skeletal and muscular components, and associated joint stiffness during gait. Twelve children with spastic diplegia CP and 12 healthy controls walked at a self-selected pace in a gait laboratory while their kinematic and forceplate data were measured and analyzed during loading response, mid-stance, terminal stance and pre-swing. For calculating the leg stiffness, each of the lower limbs was modeled as a non-linear spring, connecting the hip joint center and the corresponding center of pressure, with varying stiffness that was calculated as the slope (gradient) of the axial force vs. the deformation curve. The leg stiffness was further decomposed into skeletal and muscular components considering the alignment of the lower limb. The ankle, knee and hip of the limb were modeled as revolute joints with torsional springs whose stiffness was calculated as the slope of the moment vs. the angle curve of the joint. Independent t-tests were performed for between-group comparisons of all the variables. The CP group significantly decreased the leg stiffness but increased the joint stiffness during stance phase, except during terminal stance where the leg stiffness was increased. They appeared to rely more on muscular contributions to achieve the required leg stiffness, increasing the muscular demands in maintaining the body posture against collapse. Leg stiffness plays a critical role in modulating the kinematics and kinetics of the locomotor system during gait in the diplegic CP. PMID:26629700

  19. Impaired visually guided weight-shifting ability in children with cerebral palsy.

    PubMed

    Ballaz, Laurent; Robert, Maxime; Parent, Audrey; Prince, François; Lemay, Martin

    2014-09-01

    The ability to control voluntary weight shifting is crucial in many functional tasks. To our knowledge, weight shifting ability in response to a visual stimulus has never been evaluated in children with cerebral palsy (CP). The aim of the study was (1) to propose a new method to assess visually guided medio-lateral (M/L) weight shifting ability and (2) to compare weight-shifting ability in children with CP and typically developing (TD) children. Ten children with spastic diplegic CP (Gross Motor Function Classification System level I and II; age 7-12 years) and 10 TD age-matched children were tested. Participants played with the skiing game on the Wii Fit game console. Center of pressure (COP) displacements, trunk and lower-limb movements were recorded during the last virtual slalom. Maximal isometric lower limb strength and postural control during quiet standing were also assessed. Lower-limb muscle strength was reduced in children with CP compared to TD children and postural control during quiet standing was impaired in children with CP. As expected, the skiing game mainly resulted in M/L COP displacements. Children with CP showed lower M/L COP range and velocity as compared to TD children but larger trunk movements. Trunk and lower extremity movements were less in phase in children with CP compared to TD children. Commercially available active video games can be used to assess visually guided weight shifting ability. Children with spastic diplegic CP showed impaired visually guided weight shifting which can be explained by non-optimal coordination of postural movement and reduced muscular strength. PMID:24858794

  20. Leg and Joint Stiffness in Children with Spastic Diplegic Cerebral Palsy during Level Walking.

    PubMed

    Wang, Ting-Ming; Huang, Hsing-Po; Li, Jia-Da; Hong, Shih-Wun; Lo, Wei-Ching; Lu, Tung-Wu

    2015-01-01

    Individual joint deviations are often identified in the analysis of cerebral palsy (CP) gait. However, knowledge is limited as to how these deviations affect the control of the locomotor system as a whole when striving to meet the demands of walking. The current study aimed to bridge the gap by describing the control of the locomotor system in children with diplegic CP in terms of their leg stiffness, both skeletal and muscular components, and associated joint stiffness during gait. Twelve children with spastic diplegia CP and 12 healthy controls walked at a self-selected pace in a gait laboratory while their kinematic and forceplate data were measured and analyzed during loading response, mid-stance, terminal stance and pre-swing. For calculating the leg stiffness, each of the lower limbs was modeled as a non-linear spring, connecting the hip joint center and the corresponding center of pressure, with varying stiffness that was calculated as the slope (gradient) of the axial force vs. the deformation curve. The leg stiffness was further decomposed into skeletal and muscular components considering the alignment of the lower limb. The ankle, knee and hip of the limb were modeled as revolute joints with torsional springs whose stiffness was calculated as the slope of the moment vs. the angle curve of the joint. Independent t-tests were performed for between-group comparisons of all the variables. The CP group significantly decreased the leg stiffness but increased the joint stiffness during stance phase, except during terminal stance where the leg stiffness was increased. They appeared to rely more on muscular contributions to achieve the required leg stiffness, increasing the muscular demands in maintaining the body posture against collapse. Leg stiffness plays a critical role in modulating the kinematics and kinetics of the locomotor system during gait in the diplegic CP. PMID:26629700

  1. Trends in Prevalence and Characteristics of Post-Neonatal Cerebral Palsy Cases: A European Registry-Based Study

    ERIC Educational Resources Information Center

    Germany, Laurence; Ehlinger, Virginie; Klapouszczak, Dana; Delobel, Malika; Hollody, Katalin; Sellier, Elodie; De La Cruz, Javier; Alberge, Corine; Genolini, Christophe; Arnaud, Catherine

    2013-01-01

    The present paper aims to analyze trends over time in prevalence of cerebral palsy of post-neonatal origin, to investigate whether changes are similar according to severity and to describe the disability profile by etiology. Post-neonatal cases, birth years 1976 to 1998, were identified from the Surveillance of Cerebral Palsy in Europe…

  2. Classification of Speech and Language Profiles in 4-Year-Old Children with Cerebral Palsy: A Prospective Preliminary Study

    ERIC Educational Resources Information Center

    Hustad, Katherine C.; Gorton, Kristin; Lee, Jimin

    2010-01-01

    Purpose: In this study, the authors proposed and tested a preliminary speech and language classification system for children with cerebral palsy. Method: Speech and language assessment data were collected in a laboratory setting from 34 children with cerebral palsy (CP; 18 male, 16 female) with a mean age of 54 months (SD = 1.8). Measures of…

  3. The Use of Computers and Augmentative and Alternative Communication Devices by Children and Young with Cerebral Palsy

    ERIC Educational Resources Information Center

    Garcia, Thais Pousada; Loureiro, Javier Pereira; Gonzalez, Betania Groba; Riveiro, Laura Nieto; Sierra, Alejandro Pazos

    2011-01-01

    The purpose of the study was to determine the use of computers and assistive devices amongst children with cerebral palsy (CP) and establish the satisfaction level of both users and educational staff. The study was carried out with 30 children with cerebral palsy. A questionnaire was designed to characterize the use of new technologies and…

  4. Effect of Cardiorespiratory Training on Aerobic Fitness and Carryover to Activity In Children with Cerebral Palsy: A Systematic Review

    ERIC Educational Resources Information Center

    Butler, Jane M.; Scianni, Aline; Ada, Louise

    2010-01-01

    The question under consideration was does cardiorespiratory training improve aerobic fitness in children with cerebral palsy and is there any carryover into activity? The study design consisted of a systematic review of randomized trials using the Cochrane Collaboration guidelines. Participants were children of school age with cerebral palsy.…

  5. Physical Activity in the Life of a Woman with Cerebral Palsy: Physiotherapy, Social Exclusion, Competence, and Intimacy

    ERIC Educational Resources Information Center

    Gaskin, Cadeyrn J.; Andersen, Mark B.; Morris, Tony

    2012-01-01

    Although physical activity can have substantial mental and physical health benefits, people with cerebral palsy usually lead sedentary lives. To understand, at an individual level, this inactivity, we interviewed a 29-year-old minimally active woman with cerebral palsy (Alana) about the meanings and experiences of physical activity throughout her…

  6. Physical Activity in the Life of a Woman with Cerebral Palsy: Physiotherapy, Social Exclusion, Competence, and Intimacy

    ERIC Educational Resources Information Center

    Gaskin, Cadeyrn J.; Andersen, Mark B.; Morris, Tony

    2012-01-01

    Although physical activity can have substantial mental and physical health benefits, people with cerebral palsy usually lead sedentary lives. To understand, at an individual level, this inactivity, we interviewed a 29-year-old minimally active woman with cerebral palsy (Alana) about the meanings and experiences of physical activity throughout her

  7. Effect of Cardiorespiratory Training on Aerobic Fitness and Carryover to Activity In Children with Cerebral Palsy: A Systematic Review

    ERIC Educational Resources Information Center

    Butler, Jane M.; Scianni, Aline; Ada, Louise

    2010-01-01

    The question under consideration was does cardiorespiratory training improve aerobic fitness in children with cerebral palsy and is there any carryover into activity? The study design consisted of a systematic review of randomized trials using the Cochrane Collaboration guidelines. Participants were children of school age with cerebral palsy.

  8. Trends in Prevalence and Characteristics of Post-Neonatal Cerebral Palsy Cases: A European Registry-Based Study

    ERIC Educational Resources Information Center

    Germany, Laurence; Ehlinger, Virginie; Klapouszczak, Dana; Delobel, Malika; Hollody, Katalin; Sellier, Elodie; De La Cruz, Javier; Alberge, Corine; Genolini, Christophe; Arnaud, Catherine

    2013-01-01

    The present paper aims to analyze trends over time in prevalence of cerebral palsy of post-neonatal origin, to investigate whether changes are similar according to severity and to describe the disability profile by etiology. Post-neonatal cases, birth years 1976 to 1998, were identified from the Surveillance of Cerebral Palsy in Europe

  9. Constraint-Induced Movement Therapy for Children with Obstetric Brachial Plexus Palsy: Two Single-Case Series

    ERIC Educational Resources Information Center

    Buesch, Francisca Eugster

    2010-01-01

    The objective of this pilot study was to investigate the feasibility of constraint-induced movement therapy (CIMT) in children with obstetric brachial plexus palsy and receive preliminary information about functional improvements. Two patients (age 12 years) with obstetric brachial plexus palsy were included for a 126-h home-based CIMT…

  10. Description and Psychometric Properties of the CP QOL-Teen: A Quality of Life Questionnaire for Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Davis, Elise; Mackinnon, Andrew; Davern, Melanie; Boyd, Roslyn; Bohanna, India; Waters, Elizabeth; Graham, H. Kerr; Reid, Susan; Reddihough, Dinah

    2013-01-01

    To assess the measurement properties of a new QOL instrument, the Cerebral Palsy Quality of Life Questionnaire-Teen (CP QOL-Teen), in adolescents with cerebral palsy (CP) aged 13-18 years, examining domain structure, reliability, validity and adolescent-caregiver concordance. Based on age, 695 eligible families were invited to participate by mail.

  11. Description and Psychometric Properties of the CP QOL-Teen: A Quality of Life Questionnaire for Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Davis, Elise; Mackinnon, Andrew; Davern, Melanie; Boyd, Roslyn; Bohanna, India; Waters, Elizabeth; Graham, H. Kerr; Reid, Susan; Reddihough, Dinah

    2013-01-01

    To assess the measurement properties of a new QOL instrument, the Cerebral Palsy Quality of Life Questionnaire-Teen (CP QOL-Teen), in adolescents with cerebral palsy (CP) aged 13-18 years, examining domain structure, reliability, validity and adolescent-caregiver concordance. Based on age, 695 eligible families were invited to participate by mail.…

  12. Cerebral Palsy: General Information. Fact Sheet Number 2 = La Paralisis Cerebral: Informacion General. Fact Sheet Number 18.

    ERIC Educational Resources Information Center

    Interstate Research Associates, McLean, VA.

    This fact sheet on cerebral palsy is offered in both English and Spanish. First, it provides a definition and considers various causes (e.g., an insufficient amount of oxygen reaching the fetal or newborn brain). The fact sheet then offers incidence figures and explains characteristics of the three main types of cerebral palsy: spastic, athetoid,…

  13. Recurrent laryngeal nerve palsy and substernal goiter. An Italian multicenter study.

    PubMed

    Testini, M; Gurrado, A; Bellantone, R; Brazzarola, P; Cortese, R; De Toma, G; Fabiola Franco, I; Lissidini, G; Pio Lombardi, C; Minerva, F; Di Meo, G; Pasculli, A; Piccinni, G; Rosato, L

    2014-06-01

    The aim of this retrospective multicenter study was to verify whether the substernal goiter and the type of surgical access could be risk factors for recurrent laryngeal nerve palsy during total thyroidectomy. Between 1999-2008, 14,993 patients underwent total thyroidectomy. Patients were divided into three groups: group A (control; n=14.200, 94.7%), cervical goiters treated through collar incision; group B (n=743, 5.0%) substernal goiters treated by cervical approach; group C (n=50, 0.3%) in which a manubriotomy was performed. Transient and permanent unilateral palsy occurred significantly more frequently in B+C vs. A (P≤.001) and in B vs. A (P≤.001). Transient bilateral palsy was significantly more frequent in B+C vs. A (P≤.043) and in C vs. A (P≤.016). Permanent bilateral palsy was significantly more frequent in B+C vs. A (P≤.041), and in B vs. A (P≤.037). Extension of the goiter into the mediastinum was associated to increased risk of recurrent nerve palsy during total thyroidectomy. PMID:24880605

  14. Bilateral facial nerve palsy as the sole initial symptom of syphilis: a case report.

    PubMed

    Ting, Chi-Hsin; Wang, Chih-Wei; Lee, Jiunn-Tay; Peng, Giia-Sheun; Yang, Fu-Chi

    2015-09-01

    Bilateral facial nerve palsy is an exceedingly rare condition and presents a diagnostic challenge. Bilateral facial nerve palsy may result from cranial trauma, congenital abnormalities, inflammation, infiltration, or infection, but is rarely associated with syphilis. Here, we report a case of syphilis in which bilateral facial nerve palsy was the only initial symptom. A 22-year-old man presented at our emergency department with isolated bilateral facial nerve palsy. Results for initial serum and cerebrospinal fluid examinations were normal, including the rapid plasma reagin titer. One week later, the patient developed rashes on the torso, palms, and soles. At this time, a high serum rapid plasma reagin titer was detected, and the Treponema pallidum particle agglutination test was positive. Once the tests were confirmed, the patient admitted to a history of unprotected sexual behavior. Penicillin G treatment was effective, and a 3-month follow-up examination demonstrated a complete recovery. We recommend that syphilis be considered when diagnosing sexually experienced young men presenting with bilateral facial nerve palsy, even in the absence of skin manifestations. Failure to recognize facial signs of syphilis could result in inappropriate management, affecting the patient's clinical outcome. PMID:26166431

  15. Interpeduncular fossa lipoma: a novel cause of oculomotor nerve palsy in childhood.

    PubMed

    Malone, Jay R; Bogie, Amanda; Crittenden-Byers, Cathryn

    2012-02-01

    Oculomotor nerve palsy is a rare finding in children and, when reported, is most frequently either congenital or acquired from postnatal trauma, infection, aneurysm, or migraine. Intracranial lipomas also represent an uncommon finding in children, and although their development is not completely understood, they are now thought to be congenital in nature. Here, we describe the case of a 23-month-old boy presenting to the emergency department with left-sided, complete, pupil-involving oculomotor nerve palsy. On magnetic resonance imaging, he was found to have an intracranial lipoma of the left interpeduncular fossa. The patient had gradual and spontaneous improvement of symptoms, with complete resolution reported at the 4-month follow-up visit. However, a second magnetic resonance image at 6 months revealed that the lipoma did not change in size. To our knowledge, intracranial lipomas have been previously reported as a possible cause of partial oculomotor nerve palsy in only one adult and have never been reported in a child. In addition, we did not find any reports of intracranial lipomas as a cause of complete, pupil-involving oculomotor palsy, although they are known to cause other cranial nerve pathology. We conclude that intracranial lipomas, although rare, should be considered in the differential diagnosis for oculomotor nerve palsy in children. Further investigation is needed to determine the true incidence of this association. PMID:22307184

  16. Autologous Cord Blood Therapy for Infantile Cerebral Palsy: From Bench to Bedside

    PubMed Central

    Jensen, A.

    2014-01-01

    About 17 million people worldwide live with cerebral palsy, the most common disability in childhood, with hypoxic-ischemic encephalopathy, preterm birth, and low birth weight being the most important risk factors. This review will focus on recent developments in cell therapy for infantile cerebral palsy by transplantation of autologous umbilical cord blood. There are only 4 publications available at present; however, the observations made along with experimental data in vivo and in vitro may be of utmost importance clinically, so that a review at an early developmental stage of this new therapeutic concept seems justified. Particularly, since the first published double-blind randomized placebo-controlled trial in a paradigm using allogeneic cord blood and erythropoietin to treat cerebral palsy under immunosuppression showed beneficial therapeutic effects in infantile cerebral palsy, long-held doubts about the efficacy of this new cell therapy are dispelled and a revision of therapeutic views upon an ailment, for which there is no cure at present, is warranted. Hence, this review will summarize the available information on autologous cord blood therapy for cerebral palsy and that on the relevant experimental work as far as potential mechanisms and modes of action are concerned. PMID:24695413

  17. Detection of third and sixth cranial nerve palsies with a novel method for eye tracking while watching a short film clip

    PubMed Central

    Samadani, Uzma; Farooq, Sameer; Ritlop, Robert; Warren, Floyd; Reyes, Marleen; Lamm, Elizabeth; Alex, Anastasia; Nehrbass, Elena; Kolecki, Radek; Jureller, Michael; Schneider, Julia; Chen, Agnes; Shi, Chen; Mendhiratta, Neil; Huang, Jason H.; Qian, Meng; Kwak, Roy; Mikheev, Artem; Rusinek, Henry; George, Ajax; Fergus, Robert; Kondziolka, Douglas; Huang, Paul P.; Smith, R. Theodore

    2015-01-01

    OBJECT Automated eye movement tracking may provide clues to nervous system function at many levels. Spatial calibration of the eye tracking device requires the subject to have relatively intact ocular motility that implies function of cranial nerves (CNs) III (oculomotor), IV (trochlear), and VI (abducent) and their associated nuclei, along with the multiple regions of the brain imparting cognition and volition. The authors have developed a technique for eye tracking that uses temporal rather than spatial calibration, enabling detection of impaired ability to move the pupil relative to normal (neurologically healthy) control volunteers. This work was performed to demonstrate that this technique may detect CN palsies related to brain compression and to provide insight into how the technique may be of value for evaluating neuropathological conditions associated with CN palsy, such as hydrocephalus or acute mass effect. METHODS The authors recorded subjects’ eye movements by using an Eyelink 1000 eye tracker sampling at 500 Hz over 200 seconds while the subject viewed a music video playing inside an aperture on a computer monitor. The aperture moved in a rectangular pattern over a fixed time period. This technique was used to assess ocular motility in 157 neurologically healthy control subjects and 12 patients with either clinical CN III or VI palsy confirmed by neuro-ophthalmological examination, or surgically treatable pathological conditions potentially impacting these nerves. The authors compared the ratio of vertical to horizontal eye movement (height/width defined as aspect ratio) in normal and test subjects. RESULTS In 157 normal controls, the aspect ratio (height/width) for the left eye had a mean value ± SD of 1.0117 ± 0.0706. For the right eye, the aspect ratio had a mean of 1.0077 ± 0.0679 in these 157 subjects. There was no difference between sexes or ages. A patient with known CN VI palsy had a significantly increased aspect ratio (1.39), whereas 2 patients with known CN III palsy had significantly decreased ratios of 0.19 and 0.06, respectively. Three patients with surgically treatable pathological conditions impacting CN VI, such as infratentorial mass effect or hydrocephalus, had significantly increased ratios (1.84, 1.44, and 1.34, respectively) relative to normal controls, and 6 patients with supratentorial mass effect had significantly decreased ratios (0.27, 0.53, 0.62, 0.45, 0.49, and 0.41, respectively). These alterations in eye tracking all reverted to normal ranges after surgical treatment of underlying pathological conditions in these 9 neurosurgical cases. CONCLUSIONS This proof of concept series of cases suggests that the use of eye tracking to detect CN palsy while the patient watches television or its equivalent represents a new capacity for this technology. It may provide a new tool for the assessment of multiple CNS functions that can potentially be useful in the assessment of awake patients with elevated intracranial pressure from hydrocephalus or trauma. PMID:25495739

  18. Clinical profile and predisposing factors of cerebral palsy.

    PubMed

    Anwar, S; Chowdhury, J; Khatun, M; Mollah, A H; Begum, H A; Rahman, Z; Nahar, N

    2006-07-01

    This was an analysis of one hundred ten children with cerebral palsy (CP) attending at the Child Development & Neurology Care Center of Dhaka Medical College Hospital during January 2002 to December 2003. These children were observed to study their clinical profile, etiological factors and associated problems. The mean age of these children was 2.9 +/- 2.9 years, mostly being males (n=79, 71.8%). Most of the families of these children belonged to poorer socioeconomic strata. Spastic diplegia constituted the predominant group (34.5 per cent), followed by spastic quadriplegia (25.5 per cent). Dyskinetic CP was present in 15.5 percent of the cases. Hypotonic CP constituted a significant proportion of cases. Delayed Motor Function was observed in all 110 cases, delayed speech was complained in 67.3% cases and impaired hearing was identified in 26.8% CP children. Mothers of 43.6% CP cases reported that they had prolonged labour during delivery and almost one third (29.1%) had suffered from pre-eclampsia or eclampsia. Majority (53.6%) of the CP cases were reported to have had perinatal asphyxia. Comprehensive assessment and early management of these problems are emphasized, which can minimize the extent of disabilities. By proper perinatal care, CP can be prevented. PMID:16878093

  19. Ablative neurosurgery for movement disorders related to cerebral palsy.

    PubMed

    Sitthinamsuwan, B; Nunta-Aree, S

    2015-12-01

    This article aims to describe the roles, operative strategies and outcomes of neuroablative procedures in treatment of movement disorders related to cerebral palsy (CP). The authors reviewed relevant medical literatures concerning ablative neurosurgical procedures for CP. Neurosurgery is an appropriate option for treatment of intractable movement disorders in CP. Destructive therapies can be selectively operated upon, on the brain, spinal cord, nerve root and peripheral nerve. Because all of them carry irreversible properties, presurgical evaluation and decision making for the surgery are critical. Selection of the procedures should be tailored for individual cases. Selective dorsal rhizotomy (SDR) is mostly suitable for CP children with spastic diplegia who are potential ambulators. Selective peripheral neurotomy (SPN) aims to diminish localized hypertonia. Intractable painful spasticity in an entire useless limb can be effectively treated by dorsal root entry zone lesion (DREZotomy). Stereotactic coagulation of specific targets in the brain is appropriate for more diffuse movement disorders or hyperkinesias confined to one side of the body. Combined surgery should be employed in management of more complicated abnormalities or coexisting neurologic and orthopedic disorders. Neuroablation remains an alternative to neuromodulation therapy, especially in circumstances when the latter is unavailable. PMID:26635190

  20. Clinically relevant copy number variations detected in cerebral palsy

    PubMed Central

    Oskoui, Maryam; Gazzellone, Matthew J.; Thiruvahindrapuram, Bhooma; Zarrei, Mehdi; Andersen, John; Wei, John; Wang, Zhuozhi; Wintle, Richard F.; Marshall, Christian R.; Cohn, Ronald D.; Weksberg, Rosanna; Stavropoulos, Dimitri J.; Fehlings, Darcy; Shevell, Michael I.; Scherer, Stephen W.

    2015-01-01

    Cerebral palsy (CP) represents a group of non-progressive clinically heterogeneous disorders that are characterized by motor impairment and early age of onset, frequently accompanied by co-morbidities. The cause of CP has historically been attributed to environmental stressors resulting in brain damage. While genetic risk factors are also implicated, guidelines for diagnostic assessment of CP do not recommend for routine genetic testing. Given numerous reports of aetiologic copy number variations (CNVs) in other neurodevelopmental disorders, we used microarrays to genotype a population-based prospective cohort of children with CP and their parents. Here we identify de novo CNVs in 8/115 (7.0%) CP patients (∼1% rate in controls). In four children, large chromosomal abnormalities deemed likely pathogenic were found, and they were significantly more likely to have severe neuromotor impairments than those CP subjects without such alterations. Overall, the CNV data would have impacted our diagnosis or classification of CP in 11/115 (9.6%) families. PMID:26236009

  1. Anterior knee pain in children with cerebral palsy.

    PubMed

    Senaran, Hakan; Holden, Candice; Dabney, Kirk W; Miller, Freeman

    2007-01-01

    Anterior knee pain arising from the patellofemoral joint may be a significant problem in the cerebral palsy (CP) population. The aim of this study was to classify patients with anterior knee pain based on etiology and organize orthopaedic management based on this classification. This study was a retrospective chart review of patients with CP and anterior knee pain admitted to the orthopaedic outpatient clinic between 1991 and 2003. Children with CP who had intractable anterior knee pain despite 6 months of conservative treatment with at least 2 years' follow-up were identified and included. The medical records, radiographs, and treatment protocols were screened. Patients were classified into 3 groups according to pathology. Group I consisted of 7 children with patella alta only. Group II consisted of 7 children with patellar inferior pole fractures. Group III consisted of 13 children with subluxated or dislocated patellas. Conservative treatment methods such as anti-inflammatory drugs, local ice packs, stretching exercises, and braces should be the first step in treatment. For those patients in whom conservative treatment was not effective, surgical treatment alternatives were discussed. Knee pain resolved in all patients after surgical treatment of the underlying pathology. Gait analysis can be performed for patients with patellofemoral subluxations to evaluate the rotational malalignment of the tibia and femur. In conclusion, anterior knee pain arising from the patellofemoral joint in patients with CP should be evaluated and treated to prevent future functional deterioration. PMID:17195790

  2. Peripheral Facial Palsy: Does Patients' Religiousness Matter for the Otorhinolaryngologist?

    PubMed

    Lucchetti, Giancarlo; De Rossi, Janaina; Gonçalves, Juliane P B; Lucchetti, Alessandra L Granero

    2016-06-01

    In order to deal with the suffering, a frequent strategy employed by patients is the use of religious beliefs and behaviors. Nevertheless, few studies in otorhinolaryngology have investigated this dimension. Therefore, the present study aims to investigate the role of religiousness on quality of life, mental health, self-esteem and appearance in 116 patients with peripheral facial palsy (PFP). A cross-sectional, single-center study was carried out between 2010 and 2012 in PFP outpatients. We assessed socio-demographic data, PFP characteristics, depression, anxiety, quality of life, self-esteem, appearance and religiosity. A linear regression (adjusted for confounders) was performed to investigate whether religiosity was associated with any outcomes. The present study found that religious attendance, but not other types of religiousness, was related to quality of life and mental health on PFP patients. In addition, ENT patients would like their doctors to ask them about their faith and religion as part of their medical care. These findings give further support to the importance of religious and spiritual beliefs on ENT patients. Otorhinolaryngologists should be aware of the positive and negative aspects of religion and be prepared to address these issues in clinical practice. PMID:25967695

  3. A novel muscle for electroneurography in peripheral facial palsy: occipitalis.

    PubMed

    Uzun, Nurten; Adatepe, Turgut; Azizli, Elad; Alkan, Zeynep; Yiğit, Özgür; Gündüz, Ayşegül; Karaali-Savrun, Feray

    2016-03-01

    Electroneurography (ENoG) is one of the most objective tests in grading the damage and prediction of prognosis in peripheral facial palsy (PFP). We aimed to determine temporal changes of ENoG recorded over occipitalis muscle in acute idiopathic PFP. Consecutive 21 patients with unilateral acute idiopathic PFP and age- and sex-matched 15 healthy volunteers were included in the study. Nasal and occipital ENoG values were recorded once in the control group and the same procedure was repeated daily between the second and eight days of the disorder in the PFP group. Occipital ENoG value began to increase on the third day while nasal ENoG value was still within the normal range (27.04 vs 7.69 %, p = 0.0001). In the fourth, fifth and sixth days, occipital ENoG value was significantly high compared to nasal ENoG value (p = 0.0001 for each day) whereas nasal and occipital ENoG values were very similar in the seventh and eighth days (p = 0.181 and p = 0.584, respectively). Our study presents further support for technical possibility of occipital ENoG which may reflect the degree of fiber degeneration earlier than the nasalis muscle in PFP. PMID:25721198

  4. A holistic approach to the management of Erb's palsy

    PubMed Central

    Srilakshmi, Dasari; Chaganti, Sreelakshmi

    2013-01-01

    A 4.5-month-old female baby, presenting with complete paralysis of right upper limb with typical waiter's tip deformity, diagnosed as Erb's palsy was brought to Sri Ganapati Sachchidananda Hospital. Patient was treated with an integrated approach of physiotherapy and Ayurvedic treatment with an intention of aiding faster recovery of the patient to lead a near normal life. As per Ayurvedic classics, this condition can be correlated to Ekangavata (Vata effecting any one part of the body), which is Apatarpana in nature (diseases with deprived growth of body tissue). Hence, the choice of treatment is Santarpana Chikitsa (nourishing treatment). Santarpana Bahyopakramas (nourishing external treatment modalities) such as Ashwagandhabalalakshadi Taila (Ayurvedic medicated oil) Abhyanga (oleation therapy) and Shastikashali Anna Lepa (application of processed rice paste) were administered along with electrical stimulation (physiotherapy modality), both galvanic and faradic current in three sessions. Appreciable results were observed in the form of reduction of disparity in length and mid-arm circumference of right upper limb compared to unaffected left upper limb and the muscle power too improved from zero to four, facilitating patient to near normal movement. PMID:24459391

  5. Motor Learning Characterizes Habilitation of Children With Hemiplegic Cerebral Palsy

    PubMed Central

    Krebs, Hermano I.; Fasoli, Susan E.; Dipietro, Laura; Fragala-Pinkham, Maria; Hughes, Richard; Stein, Joel; Hogan, Neville

    2015-01-01

    Background This study tested in children with cerebral palsy (CP) whether motor habilitation resembles motor learning. Methods Twelve children with hemiplegic CP ages 5 to 12 years with moderate to severe motor impairments underwent a 16-session robot-mediated planar therapy program to improve upper limb reach, with a focus on shoulder and elbow movements. Participants were trained to execute point-to-point movements (with robot assistance) with the affected arm and were evaluated (without robot assistance) in trained (point-to-point) and untrained (circle-drawing) conditions. Outcomes were measured at baseline, midpoint, immediately after the program, and 1 month postcompletion. Outcome measures were the Fugl-Meyer (FM), Quality of Upper Extremity Skills Test (QUEST), and Modified Ashworth Scale (MAS) scores; parent questionnaire; and robot-based kinematic metrics. To assess whether learning best characterizes motor habilitation in CP, the authors quantified (a) improvement on trained tasks at completion of training (acquisition) and 1 month following completion (retention) and (b) quantified generalization of improvement to untrained tasks. Results After robotic intervention, the authors found significant gains in the FM, QUEST, and parent questionnaire. Robot-based evaluations demonstrated significant improvement in trained movements and that improvement was sustained at follow-up. Furthermore, children improved their performance in untrained movements indicating generalization. Conclusions Motor habilitation in CP exhibits some traits of motor learning. Optimal treatment may not require an extensive repertoire of tasks but rather a select set to promote generalization. PMID:22331211

  6. JUMP LANDING CHARACTERISTICS IN ELITE SOCCER PLAYERS WITH CEREBRAL PALSY

    PubMed Central

    Grande, I.; Mejuto, G.; Los Arcos, A.; Yanci, J.

    2013-01-01

    The aim of the present study was to analyse the parameters that characterize the vertical ground reaction force during the landing phase of a jump, and to determine the relationship among these parameters in elite soccer players with cerebral palsy (CP). Thirteen male members of the Spanish national soccer team for people with CP (mean age: 27.1 ± 4.7 years) volunteered for the study. Each participant performed three counter movement jumps. The characteristics of the first peak of the vertical ground reaction force during the landing phase of a jump, which corresponds to the forefoot contact with the ground, were similar to the results obtained in previous studies. However, a higher magnitude of rearfoot contact with the ground (F2) was observed in participants with CP than in participants without CP. Furthermore, a significant correlation between F2 magnitude and the elapsed time until its production (T2) was not observed (r = -0.474 for p = 0.102). This result implies that a landing technique based on a delay in the production of F2 might not be effective to reduce its magnitude, contrary to what has been observed in participants without CP. The absence of a significant correlation between these two parameters in the present study, and the high magnitude of F2, suggest that elite soccer players with CP should use footwear with proper cushioning characteristics. PMID:24744473

  7. RESULTS OF ADDUCTORS MUSCLE TENOTOMY IN SPASTIC CEREBRAL PALSY

    PubMed Central

    Guglielmetti, Luiz Gabriel Betoni; Santos, Ruy Mesquita Maranhao; Mendonça, Rodrigo Góes Medea de; Yamada, Helder Henzo; Assumpçao, Rodrigo Montezuma César de; Fucs, Patricia Maria de Moraes Barros

    2015-01-01

    Objective: Radiographic evaluation of the evolution of hips that underwent soft-tissue release. Methods: This was a retrospective evaluation on 101 spastic cerebral palsy patients who underwent soft-tissue release between 1991 and 2006. Forty-four patients met the inclusion criteria: 23 boys and 21 girls; 34 diparetic and 10 quadriparetic. Functionally, 29 were non-walkers, five were able to walk at home and 10 were able to walk within the community. Reimers' index (RI) and the acetabular index (AI) were measured on pre and postoperative radiographs, with a minimum follow-up of three years. The mean age at the time of surgery was 6.4 years. Results: The results were considered good if the RI had reduced, or had increased by less than 10%. This was found in 52% of this study. We observed a clear improvement in IR, along with worse results in patients with more than five years of postoperative follow-up. Conclusion: Soft-tissue release should be performed as early as possible, regardless of age, walking condition, clinical type, RI, AI or sex, and as soon as the patient clinically presents less than 30° abduction, because of the benefits relating to walking, prevention and treatment of subluxation, hygiene and pain relief. PMID:27022574

  8. [Idiopathic facial paralysis (Bell's palsy): a study of 180 patients].

    PubMed

    Valença, M M; Valença, L P; Lima, M C

    2001-09-01

    The objective of this study was to analyze some clinical and epidemiologic aspects, as well as the follow up of 180 patients with Bell's palsy. In the study population there was a predominance of female (66.7%). Two peaks of incidence in the age distribution were identified: third-fourth and sixth decades of life. In the group of 180 patients there were 198 events of facial paralysis, 17 recurrences and in one patient the paralysis was bilateral at the onset. In 15 patients (8.3%) there were recurrences of the facial paralysis, in 12 cases (70.6%) the recurrences were ipsilateral. The left side of the face was involved in 55.6% of the cases. In eight patients the paralysis occurred during pregnancy (n=5) or puerperium (n=3). As associated conditions we found: arterial hypertension (11.7%), diabetes mellitus (11.1%), pregnancy or puerperium (4.4%; 6.7% in the women), and neurocysticercosis (1.1%). In 72.8% of the cases no association with such conditions was found. In 22.8% of the patients some kind of sequelae were identified: hemifacial spasm (12.8%), partial recovery of the motor deficit (10.6%), syndrome of the crocodile tears (3.3%), sincinetic contraction (2.8%), and the Marcus Gunn inverse phenomenon (1.1%). In conclusion, this study shows that the idiopathic facial paralysis may lead to important sequelae in more than 20% of the patients. PMID:11593275

  9. Towards an intelligent wheelchair system for users with cerebral palsy.

    PubMed

    Montesano, Luis; Díaz, Marta; Bhaskar, Sonu; Minguez, Javier

    2010-04-01

    This paper describes and evaluates an intelligent wheelchair, adapted for users with cognitive disabilities and mobility impairment. The study focuses on patients with cerebral palsy, one of the most common disorders affecting muscle control and coordination, thereby impairing movement. The wheelchair concept is an assistive device that allows the user to select arbitrary local destinations through a tactile screen interface. The device incorporates an automatic navigation system that drives the vehicle, avoiding obstacles even in unknown and dynamic scenarios. It provides the user with a high degree of autonomy, independent from a particular environment, i.e., not restricted to predefined conditions. To evaluate the rehabilitation device, a study was carried out with four subjects with cognitive impairments, between 11 and 16 years of age. They were first trained so as to get acquainted with the tactile interface and then were recruited to drive the wheelchair. Based on the experience with the subjects, an extensive evaluation of the intelligent wheelchair was provided from two perspectives: 1) based on the technical performance of the entire system and its components and 2) based on the behavior of the user (execution analysis, activity analysis, and competence analysis). The results indicated that the intelligent wheelchair effectively provided mobility and autonomy to the target population. PMID:20071276

  10. Mechanobiological predictions of femoral anteversion in cerebral palsy.

    PubMed

    Shefelbine, Sandra J; Carter, Dennis R

    2004-02-01

    Many morphological changes occur during development of the proximal femur. The anteversion angle is a measure of the rotation of the neck of the femur around the diaphysis. In normal development anteversion is 30 degrees at birth and decreases to 15 degrees by skeletal maturity. In children with cerebral palsy (CP) anteversion often increases slightly and remains high throughout development. Previous models have proposed that cyclic hydrostatic stress decreases the growth rate while cyclic octahedral shear stress increases the growth rate. In this study we also examine changes in the growth direction caused by deformation of the developing cartilage. Using these mechanobiological principles we considered the influence of mechanical loads on the formation of the anteversion angle in normal and CP development. Loads were applied to a three-dimensional finite element model of the proximal femur. From the resulting stresses and deformations at the growth front we calculated the growth rate and growth direction and simulated the progression of the growth front over 6 months. The model predicted a decrease in anteversion angle (-2 degrees over 6 months) under normal-loading conditions, and an increase in anteversion (+ 1 degrees over 6 months) under CP-loading conditions. These results compare well with observations during skeletalgenesis, in which the anteversion angle decreases rapidly in the first few years of normal growth and may increase in children with CP. PMID:15008378

  11. Meaning in life in patients with progressive supranuclear palsy.

    PubMed

    Fegg, Martin Johannes; Kögler, Monika; Abright, Carina; Hensler, Mira; Lorenzl, Stefan

    2014-08-01

    This exploratory study investigated meaning in life (MiL) in patients with progressive supranuclear palsy (PSP). In the "Schedule for Meaning in Life Evaluation" (SMiLE), respondents list individual MiL areas before rating their current satisfaction and importance with them (index of weighting [IoW], index of satisfaction [IoS], and index of weighted satisfaction [IoWS], range 0-100). A total of 38 patients with PSP completed the SMiLE (IoS: 68.6 ± 25.6, IoW: 79.6 ± 12.6, and IoWS: 69.2 ± 26.1). A representative sample of healthy participants (n = 977) scored significantly higher in the IoS (82.8 ± 14.7, P < .001), the IoW (85.6 ± 12.3, P = .006), and the IoWS (83.3 ± 14.8, P < .001). Compared to healthy individuals, patients with PSP were less likely to list health (P = .001) and more likely to list partner (P = .04), leisure (P = .01), home/garden (P = .01), and pleasure (P = .02). Patients with PSP seem to focus on supportive relationships and leisure, while the decreasing health status is becoming less important to them. PMID:23785042

  12. Cerebral palsy: early diagnosis, intervention and risk factors.

    PubMed

    Naletili?, Mladenka; Tomi?, Vajdana; Sabi?, Marina; Vlak, Tonko

    2009-12-01

    Early diagnosis and intervention intensity were suggested to be crucial factor in cerebral palsy (CP) treatment. Herein we observed 347 children diagnosed for CP in Clinical Hospital Mostar, Bosnia and Herzegovina, and studied the relationship between (a) intervention start point and the final motor outcome, (b) intensity of treatment and final outcome, and (c) relationship between documented risk factors and early diagnosis of the CP. Our study suggests that it is possible to relatively accurately diagnose the CP in the first trimester. Previous miscarriages, sepsis and intracerebral haemorrhage were significantly related to early diagnosis, while delivery outcome, RDS, premature birth, intracerebral haemorrhage, sepsis, meningitis, hydrocephalus and convulsions were found as significantly related to final motor CP outcome. We have found no significant influence of the intervention intensity and final diagnosis. Our results support the idea that the intervention start point has to be considered as one of the most important factors for the effective intervention program. In future studies dealing with the CP interventions and risk factors, special attention should be paid to homogeneity and size of the sample, as well as necessity of including the non-treated controls in the investigation. PMID:20120401

  13. Growth and Nutrition Disorders in Children with Cerebral Palsy

    PubMed Central

    Kuperminc, Michelle N.; Stevenson, Richard D.

    2010-01-01

    Growth and nutrition disorders are common secondary health conditions in children with cerebral palsy (CP). Poor growth and malnutrition in CP merit study because of their impact on health, including psychological and physiological function, healthcare utilization, societal participation, motor function, and survival. Understanding the etiology of poor growth has led to a variety of interventions to improve growth. One of the major causes of poor growth, malnutrition, is the best-studied contributor to poor growth; scientific evidence regarding malnutrition has contributed to improvements in clinical management and, in turn, survival over the last 20 years. Increased recognition and understanding of neurological, endocrinological, and environmental factors have begun to shape care for children with CP, as well. The investigation of these factors relies on advances made in the assessment methods available to address the challenges inherent in measuring growth in children with CP. Descriptive growth charts and norms of body composition provide information that may help clinicians to interpret growth and intervene to improve growth and nutrition in children with CP. Linking growth to measures of health will be necessary to develop growth standards for children with CP in order to optimize health and well-being. PMID:18646022

  14. Spasticity and Its Contribution to Hypertonia in Cerebral Palsy

    PubMed Central

    Molenaers, Guy; Aertbeliën, Erwin; Van Campenhout, Anja; Feys, Hilde; Desloovere, Kaat

    2015-01-01

    Spasticity is considered an important neural contributor to muscle hypertonia in children with cerebral palsy (CP). It is most often treated with antispasticity medication, such as Botulinum Toxin-A. However, treatment response is highly variable. Part of this variability may be due to the inability of clinical tests to differentiate between the neural (e.g., spasticity) and nonneural (e.g., soft tissue properties) contributions to hypertonia, leading to the terms “spasticity” and “hypertonia” often being used interchangeably. Recent advancements in instrumented spasticity assessments offer objective measurement methods for distinction and quantification of hypertonia components. These methods can be applied in clinical settings and their results used to fine-tune and improve treatment. We reviewed current advancements and new insights with respect to quantifying spasticity and its contribution to muscle hypertonia in children with CP. First, we revisit what is known about spasticity in children with CP, including the various definitions and its pathophysiology. Second, we summarize the state of the art on instrumented spasticity assessment in CP and review the parameters developed to quantify the neural and nonneural components of hypertonia. Lastly, the impact these quantitative parameters have on clinical decision-making is considered and recommendations for future clinical and research investigations are discussed. PMID:25649546

  15. Bell's palsy: a summary of current evidence and referral algorithm.

    PubMed

    Glass, Graeme E; Tzafetta, Kallirroi

    2014-12-01

    Spontaneous idiopathic facial nerve (Bell's) palsy leaves residual hemifacial weakness in 29% which is severe and disfiguring in over half of these cases. Acute medical management remains the best way to improve outcomes. Reconstructive surgery can improve long term disfigurement. However, acute and surgical options are time-dependent. As family practitioners see, on average, one case every 2 years, a summary of this condition based on common clinical questions may improve acute management and guide referral for those who need specialist input. We formulated a series of clinical questions likely to be of use to family practitioners on encountering this condition and sought evidence from the literature to answer them. The lifetime risk is 1 in 60, and is more common in pregnancy and diabetes mellitus. Patients often present with facial pain or paraesthesia, altered taste and intolerance to loud noise in addition to facial droop. It is probably caused by ischaemic compression of the facial nerve within the meatal segment of the facial canal probably as a result of viral inflammation. When given early, high dose corticosteroids can improve outcomes. Neither antiviral therapy nor other adjuvant therapies are supported by evidence. As the facial muscles remain viable re-innervation targets for up to 2 years, late referrals require more complex reconstructions. Early recognition, steroid therapy and early referral for facial reanimation (when the diagnosis is secure) are important features of good management when encountering these complex cases. PMID:25208543

  16. Thermograpic study of upper extremities in patients with cerebral palsy

    NASA Astrophysics Data System (ADS)

    Lampe, R.; Kawelke, S.; Mitternacht, J.; Turova, V.; Blumenstein, T.; Alves-Pinto, A.

    2015-03-01

    Trophic disorders like reduced skin blood circulation are well-known epiphenomenon of cerebral palsy (CP). They can influence quality of life and can lead to skin damages and, as a consequence, to decubitus. Therefore, it is important to analyse temperature regulation in patients with CP. Thermal imaging camera FLIR BCAM SD was used to study the dependency of skin blood circulation in upper extremities of patients with CP on hand dominance, hand force and hand volume. The hand force was evaluated using a conventional dynamometer. The hand volume was measured with a volumeter. A cold stress test for hands was applied in 22 patients with CP and 6 healthy subjects. The warming up process after the test was recorded with the thermal camera. It was confirmed that the hands of patients warm up slower comparing to healthy persons. The patients' working hands warm up faster than non-working ones. A slight correlation was established between the hand grip force of the working hands and their warm up time. No correlation was found between the warming up time and the volume of the hand. The results confirm our assumption that there is a connection of peripheral blood circulation to upper limb motor functions.

  17. Changes in masticatory muscle activity in children with cerebral palsy.

    PubMed

    Briesemeister, Marianne; Schmidt, Kelly Cristine; Ries, Lilian Gerdi Kittel

    2013-02-01

    The objective of the study was to determine whether children with cerebral palsy (CP) have abnormal bilateral masseter and temporal muscle activation during mastication. The muscular activity of 32 children aged between 7 and 13 years was assessed during the task of non-habitual mastication by means of surface electromyograms. During non-habitual mastication, the amplitude of all assessed muscles in the inactive period and the amplitude of the Right Masseter and Left Temporal muscles in the active period of children with CP was greater (p < 0.05) in relation to the group of children with Typical Development (TD). Considering each muscle individually, only the duration of the active period of Right Masseter and Right Temporal muscles in children with CP was lower (p < 0.05) than in the TD children. Considering the four analyzed muscles, the duration of time of general active period, when at least one muscle should be activated, was higher in children with CP (p < 0.05) than in children with TD showing greater time variation in inactivation (p < 0.05). The higher muscle activity during the phases of the masticatory cycle, with longer duration of the active period and with greater variability between the muscles to inhibit this activity show greater difficulty in coordinating the muscles of mastication in children with CP compared to children with TD. PMID:23063911

  18. Heterotopic ossification after hip surgery in cerebral palsy.

    PubMed

    Tirelli, A; Artiaco, S; Zanchini, F; Di Martino, A

    2004-01-01

    Heterotopic ossification is a post-surgery complication occasionally observed in patients with infantile cerebral palsy. In the majority of the cases such lesions are found at the hip after surgery on the skeleton and the soft tissues. At our Institute in the last five years, this complication has been observed four times, in 39 patients, who underwent mainly soft tissue releases because of flexion and adduction contracture of the hip. In these cases, as in a further patient with myelomeningocele, the periarticular heterotopic ossification appeared in the hip after tenotomy of the ilio-psoas at the lesser tronchater associated to other surgical procedures. Conversely, no cases of ossification have been found after tenotomy of the adductors or the gracilis or selective tenotomy of the psoas at the pelvic brim. The exact causes of this complication are still unclear, but after an analysis of the literature and patient history it can be hypothesised that it may be related to the surgical procedures carried out. PMID:15751594

  19. Early numeracy in cerebral palsy: review and future research.

    PubMed

    Van Rooijen, Maaike; Verhoeven, Ludo; Steenbergen, Bert

    2011-03-01

    Children with cerebral palsy (CP) often have problems with arithmetic, but the development of numerical abilities in these children has received only minor attention. In comparison, detailed accounts have been written on the arithmetic abilities of typically developing children, but a theoretical framework is still lacking. A promising perspective is the embodied cognition framework, which focuses on the influence of perception and action behaviours on cognition. We searched the literature to find the available studies on the early numeracy capacities of children with CP. We reviewed eight studies in which primary school-aged children with CP with a verbal IQ of at least 70 participated. The selected studies showed that these children are regularly delayed in performing simple arithmetic operations compared with their typically developing peers. However, owing to the limited number of studies no definite conclusions can be drawn regarding the precursors and developmental trajectories of arithmetic abilities in children with CP. We argue that the embodied cognition framework is well suited to scrutinize the arithmetic abilities of children with CP and provide future directions for research. PMID:21087241

  20. Congenital anomalies in cerebral palsy: where to from here?

    PubMed

    McIntyre, Sarah; Blair, Eve; Goldsmith, Shona; Badawi, Nadia; Gibson, Catherine; Scott, Heather; Smithers-Sheedy, Hayley

    2016-02-01

    Proportions of cases of cerebral palsy (CP) with congenital anomalies recorded in Australian CP registers range from 15% to 40%. The anomalies seen in CP are extremely variable. We have identified that CP registers often do not have quality data on congenital anomalies, necessitating linkage with congenital anomaly registers. However, a lack of unified processes and definitions in congenital anomaly registers and data collections means that linkages are complex, need to be carefully planned, and limitations acknowledged. Historically in CP research, congenital anomalies have been classified by International Classification of Disease codes, then combined into brain and other major and minor anomalies. Systems have been developed to classify congenital anomalies into aetiologically related groups, but such a classification has yet to be trialled in CP. It is anticipated that primary prevention of a small proportion of cases of CP is possible through the primary prevention of congenital anomalies, especially those due to teratogens. Owing to the anticipated low prevalence of each subgroup, global collaboration will be required to further these lines of enquiry. PMID:26762782