Sample records for polyradiculopathy

  1. Polyradiculopathy secondary to severe hypertriglyceridemia.

    PubMed

    Nesbitt, Cassie; Wong, Daniel; Batchelor, Peter

    2015-05-08

    A 74-year-old man presented with a subacute severe thoracic polyradiculopathy affecting the T4-T8 dermatomes bilaterally. Extensive investigation demonstrated markedly raised triglyceride levels of 44 mmol/L (<1.7). The patient's unique presentation is discussed alongside a review of triglyceride-induced neurotoxicity and therapeutic management. 2015 BMJ Publishing Group Ltd.

  2. Motor polyradiculopathy during pembrolizumab treatment of metastatic melanoma.

    PubMed

    Sepúlveda, Maria; Martinez-Hernandez, Eugenia; Gaba, Lydia; Victoria, Ivan; Sola-Valls, Nuria; Falgàs, Neus; Casanova-Molla, Jordi; Graus, Francesc

    2017-12-01

    Pembrolizumab, a monoclonal antibody directed against the immune checkpoint programmed cell death-1 receptor (PD-1), has improved survival in patients with advanced melanoma. Neuromuscular immune-mediated side effects have been rarely reported. We describe a 44-year-old man with metastatic melanoma who presented with progressive muscle weakness after 23 doses of pembrolizumab. The patient developed asymmetric, proximal muscle weakness and atrophy in all four limbs. Cerebrospinal fluid examination showed albuminocytologic dissociation. MRI revealed contrast enhancement of the lumbosacral roots. Electrodiagnostic studies demonstrated widespread fibrillation potentials in all four limbs, suggesting a generalized motor polyradiculopathy. Despite pembrolizumab discontinuation and treatment with steroids and intravenous immunoglobulin, limb weakness worsened. Electrodiagnostic studies were repeated, and showed marked and diffuse axonal motor damage. Seven weeks after clinical onset the patient was treated with plasma exchanges. He showed no further deterioration. We report a severe motor polyradiculopathy associated with an anti-PD-1 agent that expands the spectrum of neuromuscular complications of this class of drugs. Muscle Nerve 56: E162-E167, 2017. © 2017 Wiley Periodicals, Inc.

  3. Neuromuscular Diseases Associated with HIV-1 Infection

    PubMed Central

    Robinson-Papp, Jessica; Simpson, David M.

    2010-01-01

    Neuromuscular disorders are common in HIV, occurring at all stages of disease and affecting all parts of the peripheral nervous system. These disorders have diverse etiologies including HIV itself, immune suppression and dysregulation, co-morbid illnesses and infections, and side effects of medications. In this article, we review the following HIV-associated conditions: distal symmetric polyneuropathy, inflammatory demyelinating polyneuropathy, mononeuropathy, mononeuropathy multiplex, autonomic neuropathy, progressive polyradiculopathy due to cytomegalovirus, herpes zoster, myopathy and other rarer disorders. PMID:19771594

  4. Acute lumbosacral polyradiculopathy due to cytomegalovirus in advanced HIV disease: CSF findings in 17 patients.

    PubMed Central

    Miller, R F; Fox, J D; Thomas, P; Waite, J C; Sharvell, Y; Gazzard, B G; Harrison, M J; Brink, N S

    1996-01-01

    OBJECTIVES: To describe the abnormalities in CSF from HIV infected patients with acute lumbosacral polyradiculopathy (ALP) caused by cytomegalovirus (CMV) infection. METHODS: Retrospective case notes and laboratory records were reviewed for 17 consecutive patients with CMV associated ALP admitted to specialist HIV/AIDS units at UCL Hospitals and Chelsea and Westminster Hospital. RESULTS: Infection with CMV was confirmed by detection of CMV DNA by polymerase chain reaction amplification in 15 patients (all of whom were negative by culture), by culture in one patient, and by objective clinical response to anti-CMV treatment in one patient. Only nine patients had a CSF pleocytosis 28-1142 (median 150) cells/mm3; in seven there was a polymorphonuclear (PMN) leucocyte preponderance. Protein concentrations in CSF were moderately or considerably raised in 13 patients; CSF: plasma glucose ratios were < or = 50% in five patients. Two patients had no pleocytosis, normal CSF: plasma glucose, and normal or near normal protein values. CONCLUSIONS: Abnormalities in CSF in CMV associated ALP are varied: only 50% of patients have a "typical" PMN preponderant pleocytosis. The diagnosis of this condition should not rely on demonstration of a PMN preponderant pleocytosis, but on identification of CMV DNA in CSF and the exclusion of other opportunistic infections and lymphoma in order that specific anti-CMV treatment may be instituted. PMID:8937337

  5. Epidemiology and Clinical Features of Guillain-Barre Syndrome in Isfahan, Iran.

    PubMed

    Ansari, Behnaz; Basiri, Keivan; Derakhshan, Yeganeh; Kadkhodaei, Farzaneh; Okhovat, Ali Asghar

    2018-01-01

    Guillain-Barre syndrome (GBS) is an immune-mediated peripheral neuropathy. We compared clinical, laboratory characteristics, and disease course of GBS subtypes in a large group of Iranian patients in Isfahan. We collected data from patients who were admitted to Alzahra referral university Hospital, Isfahan, Iran with a diagnosis of GBS. In this population-based cross-sectional research, characteristic of 388 cases with GBS between 2010 and 2015 were studied. The current study recruited 388 patients with GBS including 241 males (62.1%) and 147 females (37.9%) with a mean age of 42.78 ± 21.34. Patients with polyradiculopathy had the highest mean age of 55.12 ± 20.59 years, whereas the least age was seen in acute motor axonal neuropathy (AMAN) with the mean of 36.30 ± 18.71 years. The frequency of GBS witnessed the highest frequency in spring with 113 cases (29.1%) and winter with 101 cases (26%). Patients' electrodiagnostic findings indicated that the highest frequency pertained to AMSAN with 93 cases (24%), whereas the least frequent diagnosis was acute Polyradiculopathy with 8 cases (2.1%). Most of the patients did not have any infections (53.6%) and among patients with infections, AMSAN had the highest frequency (22.9%) and finally, patients with AMSAN and AMAN had a higher length of stay. The study demonstrated incidence, sex distribution, preceding infection, and surgery similar to previous studies. However, our data differs from a study in Tehran that showed acute inflammatory demyelinating polyradiculoneuropathy is more prevalent than other types and we found a seasonal preponderance in cold months, particularly in axonal types.

  6. Steroid-responsive polyradiculopathy in association with focal segmental glomerulosclerosis

    PubMed Central

    Chapman, Andrew R.; Gamble, Paul; Pollock, Anne Marie; Joss, Nicola

    2013-01-01

    An 80-year-old woman presented with simultaneous increasing muscle weakness and nephrotic syndrome. A renal biopsy confirmed focal segmental glomerulosclerosis (FSGS). Her neurological diagnosis best fitted with a Guillain–Barre-like syndrome. There have been several cases of FSGS in combination with both conventional and atypical Guillain–Barre syndrome (GBS). Our patient was treated with high-dose steroids and resolution of both nephrotic syndrome and neurological symptoms occurred over 6 months. This article reviews all previously published presentations of this nature and discusses putative mechanisms for the development of concurrent FSGS and GBS. PMID:26069832

  7. Polyradiculopathies from schwannomatosis.

    PubMed

    Jia, Yuxia; Kraus, James A; Reddy, Hasini; Groff, Michael; Wong, Eric T

    2011-01-01

    We describe a case of schwannomatosis presenting as radicular pain and numbness in multiple radicular nerve distributions. There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi. Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels. The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining. The findings are consistent with sporadic form of schwannomatosis. This case highlights the importance of using MRI and INI1 immunohistochemistry to differentiate familial schwannomatosis, neurofibromatosis 2 (NF2)-associated schwannomatosis, and sporadic schwannomatosis.

  8. Post chicken pox neurological sequelae: Three distinct presentations.

    PubMed

    Paul, Rudrajit; Singhania, Pankaj; Hashmi, Ma; Bandyopadhyay, Ramtanu; Banerjee, Amit Kumar

    2010-07-01

    Varicella zoster infection is known to cause neurological involvement. The infection is usually self-limiting and resolves without sequelae. We present a series of three cases with neurological presentations following chicken pox infection. The first case is a case of meningitis, cerebellitis and polyradiculopathy, the second is a florid case of acute infective demyelinating polyradiculoneuropathy (Guillian-Barré syndrome) in a middle-aged female and the third case is a young man in whom we diagnosed acute transverse myelitis. All these cases presented with distinct neurological diagnoses and the etiology was established on the basis of history and serological tests confirmatory for chicken pox. The cases responded differently to treatment and the patients were left with minimum disability.

  9. Polyradiculopathies from Schwannomatosis

    PubMed Central

    Jia, Yuxia; Kraus, James A.; Reddy, Hasini; Groff, Michael; Wong, Eric T

    2011-01-01

    We describe a case of schwannomatosis presenting as radicular pain and numbness in multiple radicular nerve distributions. There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi. Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels. The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining. The findings are consistent with sporadic form of schwannomatosis. This case highlights the importance of using MRI and INI1 immunohistochemistry to differentiate familial schwannomatosis, neurofibromatosis 2 (NF2)-associated schwannomatosis, and sporadic schwannomatosis. PMID:21643503

  10. Severe Acute Axonal Neuropathy Induced by Ciprofloxacin: A Case Report.

    PubMed

    Popescu, Cyprian

    2018-01-01

    Fluoroquinolones increase the risk of peripheral neuropathy. The present work aims to report a case of fluoroquinolone-related severe axonal neuropathy. The subject of this study was a 62-year-old man who exhibited generalized sensory disturbances 4 days after treatment by ciprofloxacin prescribed for urinary infection. Electrodiagnostic studies revealed severe motor-sensory axonal neuropathy with widespread fibrillation potentials in support of generalized motor polyradiculopathy. There was no evidence of conduction blocks or albuminocytologic dissociation in favor of an autoimmune inflammatory reaction. The only pathological biomarker was the reduction of serum folate. According to this case, we suggest that folate level could be routinely measured and supplementation should be performed in patients with fluoroquinolone-induced neuropathy.

  11. Pelvic plexus compression due to a uterine leiomyoma in a woman with acute urinary retention: a new hypothesis.

    PubMed

    Andrada, Andrea Orosa; De Vicente, José Miguel Gómez; Cidre, Miguel Angel Jiménez

    2014-03-01

    Acute urinary retention (AUR) in women is an uncommon occurrence described by the International Continence Society (ICS) as a painful, palpable, or perceptible bladder when the patient is unable to pass urine. Contrarily to men, AUR in women is not usually due to any obstructive process. Neurologic causes are the most common reason for AUR in reproductive-age women. A few case reports have been published concerning women suffering from gynecological pathology and AUR, and they propose extrinsic compression of the urinary tract. In the case we report, AUR pathophysiology was compression of the pelvic plexus by a giant uterine leiomyoma. An electromyogram displayed motor polyradiculopathy of S1 and S2 nerve roots, and the patient was unable to urinate due to an uncontractible bladder.

  12. Persistent cauda equina syndrome after caudal epidural injection under severe spinal stenosis: a case report

    PubMed Central

    Seo, Young Tak; Kong, Hyun Ho; Lee, Goo Joo; Bang, Heui Je

    2017-01-01

    Caudal epidural injection (CEI) is one of the most common treatments for low-back pain with sciatica. CEI rarely leads to neurologic complications. We report a case of persistent cauda equina syndrome after CEI. A 44-year-old male patient with severe L4 and L5 spinal ste-nosis underwent CEI for low-back pain and sciatica. The CEI solution consisted of bupivacaine, hyaluronidase, triamcinolone acetonide, and normal saline. He experienced motor weakness and sensory loss in both lower extremities and neurogenic bladder for more than 1 year after the procedure. His ankle dorsiflexors, big-toe extensors, and ankle plantar flexors on both sides were checked and categorized as motor-power Medical Research Council grade 0. His bilateral ankle-jerk reflection was absent. An electrophysiological study showed lumbosacral polyradiculopathy affecting both sides of the L5 and S1 nerve roots. A urodynamic study revealed hypoactive neurogenic bladder affecting both sacral roots. PMID:28652808

  13. Persistent cauda equina syndrome after caudal epidural injection under severe spinal stenosis: a case report.

    PubMed

    Seo, Young Tak; Kong, Hyun Ho; Lee, Goo Joo; Bang, Heui Je

    2017-01-01

    Caudal epidural injection (CEI) is one of the most common treatments for low-back pain with sciatica. CEI rarely leads to neurologic complications. We report a case of persistent cauda equina syndrome after CEI. A 44-year-old male patient with severe L4 and L5 spinal ste-nosis underwent CEI for low-back pain and sciatica. The CEI solution consisted of bupivacaine, hyaluronidase, triamcinolone acetonide, and normal saline. He experienced motor weakness and sensory loss in both lower extremities and neurogenic bladder for more than 1 year after the procedure. His ankle dorsiflexors, big-toe extensors, and ankle plantar flexors on both sides were checked and categorized as motor-power Medical Research Council grade 0. His bilateral ankle-jerk reflection was absent. An electrophysiological study showed lumbosacral polyradiculopathy affecting both sides of the L5 and S1 nerve roots. A urodynamic study revealed hypoactive neurogenic bladder affecting both sacral roots.

  14. Paralytic squint due to abducens nerve palsy : a rare consequence of dengue fever

    PubMed Central

    2012-01-01

    Background Dengue fever is an endemic illness in the tropics with early and post infectious complications affecting multiple systems. Though neurological sequelae including mononeuropathy, encephalopathy, transverse myelitis, polyradiculopathy, Guillain-Barre syndrome , optic neuropathy and oculomotor neuropathy have been reported in medical literature, the abducens nerve despite its notoriety in cranial neuropathies in a multitude of condition due to its long intracranial course had not been to date reported to manifest with lateral rectus paralysis following dengue. Case presentation A previously well 29 year old male with serologically confirmed dengue hemorrhagic fever developed symptomatic right lateral rectus palsy during the critical phase of the illness, which persisted into convalescence and post convalescence with proven deficit on Hess screen. Alternate etiologies were excluded by imaging, serology and electrophysiology. Conclusions The authors detail the first reported case of abducens nerve palsy complicating dengue fever in a previously healthy male from Sri Lanka. In a tropical country with endemic dengue infections, dengue related abducens neuropathy may be considered as a differential diagnosis in cases of acquired lateral rectus palsy after dengue fever. PMID:22799448

  15. Decreased Axon Flare Reaction to Electrical Stimulation in Patients With Chronic Demyelinating Inflammatory Polyneuropathy.

    PubMed

    Kokotis, Panagiotis; Schmelz, Martin; Papagianni, Aikaterini E; Zambelis, Thomas; Karandreas, Nikos

    2017-03-01

    In chronic inflammatory demyelinating polyradiculopathy (CIDP), the impairment of unmyelinated nerve fibers appears unexpected. The measurement of the electrically induced axon flare reflex is a clinical test to assess the peripheral C-nociceptor function. In this study, we compared the flare area in patients suffering from CIDP with healthy subjects. We examined 18 patients fulfilling the criteria for CIDP (11 men, mean age 51.8 years, SD 15.1) and 18 age-matched adult healthy volunteers (control group) (11 men, mean age 51.9 years, SD 15.8). The flare responses were elicited by transcutaneous electrical stimulation and recorded by laser Doppler imaging. There was a significant reduction of electrically induced maximum flare area in the foot dorsum of patients with CIDP (t-value 2.08, P = 0.04) which proved to be length-dependent measured by a numerical index comparing the results with the forearm and thigh. The repeatedmeasures ANOVA revealed statistically significant smaller flare areas in all body regions for the CIDP group (P < 0.001). The axon flare reaction to electrical stimulation was decreased in patients with chronic demyelinating inflammatory polyneuropathy. The evaluation of the axon flare response can be proposed as a noninvasive objective functional test to detect an impaired C-fiber function in CIDP patients with the advantages of simplicity of the procedure, time economy, and objectivity.

  16. Epidemiology of Peripheral Neuropathy: An Indian Perspective

    PubMed Central

    Trivedi, Sweety; Pandit, Alak; Ganguly, Goutam; Das, Shyamal Kumar

    2017-01-01

    Peripheral neuropathy (PN) is a common disorder and presents as diagnostic and therapeutic challenge to physicians and neurologists. In epidemiological studies from India from various regions the overall prevalence of PN varied from 5 to 2400 per 10,000 population in various community studies. India is composed of a multiethnic, multicultural population who are exposed to different adverse environmental factors such as arsenic and lead. Use of different chemotherapeutic agents with propensity to affect peripheral nerves, increasing methods of diagnosis of connective tissue disorders and use of immunomodulating drugs, growing aging population is expected to change the spectrum and burden of peripheral neuropathy in the community. The other important aspect of peripheral neuropathies is in terms of the geographical and occupational distribution especially of toxic neuropathies like arsenic which is common in eastern belt; lead, mercury and organo-phosphorous compounds where occupational exposures are major sources. Inflammatory neuropathies either due to vasculitis or G B Syndrome, chronic inflammatory polyradiculopathies are another major group of neuropathies which is increasing due to increase longevity of Indian subjects and immunological impairment, also adds to morbidity of the patients and are potentially treatable. Leprous neuropathy is common in India and although its frequency is significantly decreasing because of national control program yet pure neuritic form still remains a cause of concern and similar is the case with another infective cause like diptheric neurpathy. Thus this article is an attempt to cover major categories and also highlight the areas where further studies are needed. PMID:28904445

  17. The Importance of Rare Subtypes in Diagnosis and Treatment of Peripheral Neuropathy: A Review.

    PubMed

    Callaghan, Brian C; Price, Raymond S; Chen, Kevin S; Feldman, Eva L

    2015-12-01

    Peripheral neuropathy is a prevalent condition that usually warrants a thorough history and examination but has limited diagnostic evaluation. However, rare localizations of peripheral neuropathy often require more extensive diagnostic testing and different treatments. To describe rare localizations of peripheral neuropathy, including the appropriate diagnostic evaluation and available treatments. References were identified from PubMed searches conducted on May 29, 2015, with an emphasis on systematic reviews and randomized clinical trials. Articles were also identified through the use of the authors' own files. Search terms included common rare neuropathy localizations and their causes, as well as epidemiology, pathophysiology, diagnosis, and treatment. Diffuse, nonlength-dependent neuropathies, multiple mononeuropathies, polyradiculopathies, plexopathies, and radiculoplexus neuropathies are rare peripheral neuropathy localizations that often require extensive diagnostic testing. Atypical neuropathy features, such as acute/subacute onset, asymmetry, and/or motor predominant signs, are frequently present. The most common diffuse, nonlength-dependent neuropathies are Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and amyotrophic lateral sclerosis. Effective disease-modifying therapies exist for many diffuse, nonlength-dependent neuropathies including Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and some paraprotein-associated demyelinating neuropathies. Vasculitic neuropathy (multiple mononeuropathy) also has efficacious treatment options, but definitive evidence of a treatment effect for IgM anti-MAG neuropathy and diabetic amyotrophy (radiculoplexus neuropathy) is lacking. Recognition of rare localizations of peripheral neuropathy is essential given the implications for diagnostic testing and treatment. Electrodiagnostic studies are an important early step in the diagnostic evaluation and provide information on the localization and pathophysiology of nerve injury.

  18. [Atypical Guillain-Barre syndrome clustering: is it necessary to reconsider the diagnostic criteria and microbiological protocol?

    PubMed

    Dominguez-Mayoral, A; Gutierrez, C; Lopez-Dominguez, J M; Eichau, S; Abril, J; Navarro-Mascarell, G; Quesada-Garcia, M A; Ramos, M; Alvarez-Lopez, M; Menendez-De Leon, C; Izquierdo, G

    2017-05-01

    Guillain-Barre syndrome is classically defined as a symmetrical ascending acute polyradiculoneuropathy, although there are atypical variants that make diagnosis difficult. The medical data of six patients in our hospital area are collected during the first quarter of 2013. Lumbar punctures, imaging, neurophysiological studies, ganglioside antibodies and serologies have been proposed in all cases. We focus on the atypical features as late hyporeflexia, increased frequency of asymmetry and distal paresis and initial fever. From a neurophysiological point of view, all patients presented sensorimotor axonal forms. The most consistent datas in early studies is the F wave's alteration. A Miller Fisher variant associated with faciocervicobraquial paresis and cerebral reversible vasoconstriction syndrome has been detected. A bilateral brachial paresis and lumbar polyradiculopathy in the context of influenza A infection is other interesting case. The saltatory variant with cranial nerve involvement and lower limbs paresis has been demonstrated in one patient. Bands in cerebrospinal fluid are positive in three cases and anti-ganglioside antibodies in one patient. The syndrome of inappropriate secretion of antidiuretic hormone may explain some of the hyponatremias registered. The first line of treatment are inmunoglobulins in all patients. Plasmapheresis exchanges has been used as an additional therapy in four cases. These clusters of six axonal cases with atypical clinical features justifies the need for knowledge of these variants in order to achieve an early treatment. Late hyporeflexia and brachialfaciocervico, saltatory and lumbar forms should be considered in the spectrum of Guillain-Barre syndrome. The etiological study should rule out a lots of pathogens as influenza A.

  19. An ayurvedic approach in the management of Guillain-Barre syndrome: A case study.

    PubMed

    Nakanekar, Amit; Bhople, Sunanda; Gulhane, Harshad; Rathod, Suraj; Gulhane, Jayant; Bonde, Pravin

    2015-01-01

    Guillain-Barre syndrome is an acute, frequently severe and fulminant polyradiculopathy that is autoimmune in nature. Guillain Barre syndrome is a rare disorder that causes immune systems to attack peripheral nervous system (PNS). A 46 year old male patient, presenting with sudden onset, complete paralysis of all four limbs (quadriplegia), unable to walk, stand, sit, difficulty in deglutition (dysphagia) and dysarthia, was having foley's catheter and Ryle's Tube brought by relative to Out Door Patient Department (OPD) of Government Ayurvedic Hospital, Nagpur; He was provisionally diagnosed as subacute sensory motor paraplegia. Previously patient admitted and treated in Government Medical College (GMC) Nagpur but did not show any sign of improvement so patient was admitted and treated with Ayurvedic treatment for about 50 days. As per Ayurvedic classics, this condition can be correlated with sarvāṅ gagatavātavyādhi (~vāta disorder affecting all parts of the body), which is apatarpaṇa in nature (~diseases with deprived nourishment of body tissue) preceded by jvara (~(H/O fever before onset of GBS). Hence, the principle of treatment is santarpaṇa cikitsā (~nourishing treatment). Santarpaṇa (~nourishing treatment) includes bahyopakramas (~nourishing external treatment modalities), such as abhyaṅga (~oleation therapy) and ṣaṣṭikaśālipiṇḍasveda (~sudation using of hot and processed ṣaṣṭika rice), karmabasti (~medicated enema) śirodhārā (gentle pouring of medicated liquid over forehead) and jvaraghna cikitsā (~treatment of fever) using various Ayurvedic herbomineral compounds. Remarkable results were observed in the form of improvement in the muscle power from zero to five of all four limbs with improvement in speech. There was no difficulty post treatment in deglutition, sitting, standing and walking; and now patient has near to normal movements.

  20. Voltage-gated potassium channel-complex autoimmunity and associated clinical syndromes.

    PubMed

    Irani, Sarosh R; Vincent, Angela

    2016-01-01

    Voltage-gated potassium channel (VGKC)-complex antibodies are defined by the radioimmunoprecipitation of Kv1 potassium channel subunits from brain tissue extracts and were initially discovered in patients with peripheral nerve hyperexcitability (PNH). Subsequently, they were found in patients with PNH plus psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) with prominent amnesia and frequent seizures. Most recently, they have been described in patients with pure epilepsies, especially in patients with the novel and distinctive semiology termed faciobrachial dystonic seizures (FBDS). In each of these conditions, there is a close correlation between clinical measures and antibody levels. The VGKC-complex is a group of proteins that are strongly associated in situ and after extraction in mild detergent. Two major targets of the autoantibodies are leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2). The patients with PNH or MoS are most likely to have CASPR2 antibodies, whereas LGI1 antibodies are found characteristically in patients with FBDS and LE. Crucially, each of these conditions has a good response to immunotherapies, often corticosteroids and plasma exchange, although optimal regimes require further study. VGKC-complex antibodies have also been described in neuropathic pain syndromes, chronic epilepsies, a polyradiculopathy in porcine abattoir workers, and some children with status epilepticus. Increasingly, however, the antigenic targets in these patients are not defined and in some cases the antibodies may be secondary rather than the primary cause. Future serologic studies should define all the antigenic components of the VGKC-complex, and further inform mechanisms of antibody pathogenicity and related inflammation. © 2016 Elsevier B.V. All rights reserved.

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