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Objective: Morvan syndrome is characterized by central, autonomic, and peripheral hyperexcitability due to contactin-associated protein 2 (CASPR2) antibody. Our objective was to study the clinical spectrum, electrophysiologic, autonomic, polysomnographic, and neuropsychological profile in patients with CASPR2-related Morvan syndrome., Methods: Serum and CSF samples that were CASPR2 antibody positive from 2016 to 2019 were assessed. Among them, patients with Morvan syndrome diagnosed based on clinical and electrophysiologic basis were included., Results: Fourteen (M:F = 10:4) patients with Morvan syndrome were included with age at onset of 37.1 ± 17.5 years. The clinical features were muscle twitching (12), insomnia (12), pain (11), paresthesias (9), hyperhidrosis (7), hypersalivation (6), double incontinence (3), spastic speech (2), dysphagia (2), behavioral disturbances (2), seizures (1), and cold intolerance (1). Neurologic examination revealed myokymia (12), hyperactive tendon reflexes (10), and tremor (6). EMG revealed neuromyotonia (12) and increased spontaneous activity (7). Autonomic function tests conducted in 8 patients revealed definite autonomic dysfunction (4), orthostatic hypotension (2), early dysfunction (1), and postural orthostatic tachycardia syndrome (1). Polysomnography findings in 6 patients revealed insomnia (3), absence of deep sleep (1), high-frequency beta activity (1), REM behavior disorder (1), and periodic leg movements (1). Neuropsychological evaluation showed subtle involvement of the left frontal and temporal lobe. Malignancy workup was negative. All patients were treated with steroids. There was complete neurologic resolution in follow-up with persistent neuropathic pain in 5 patients., Conclusions: This study has contributed to the growing knowledge on CASPR2-related Morvan syndrome. It is important for an increased awareness and early recognition as it is potentially treatable by immunotherapy., (© 2021 American Academy of Neurology.)

Background and Aims: Myelin oligodendrocyte glycoprotein (MOG) is an oligodendrocytopathy resulting in demyelination. We aimed to determine the frequency of MOG-associated disorders (MOGAD), its various clinical phenotypes, and imaging characteristics., Methods: All patients with MOGAD were included. Description of the various clinical phenotypes, investigation profile, therapeutic response, differences between pediatric and adult-onset neurological disorders, determination of poor prognostic factors was done., Results: The study population consisted of 93 (M:F = 45:48) (Pediatric:40, Adult-onset:47, Late-onset:7) patients with a median age of 21 years. Among the 263 demyelinating episodes; 45.8% were optic neuritis (ON), 22.8% were myelopathy, 17.1% were brainstem, 7.6% were acute demyelinating encephalomyelitis(ADEM), 4.2% were opticomyelopathy and 2.3% with cerebral manifestations. There was exclusive vomiting in 24.7% prior to onset of clinical syndrome, none of them had area postrema involvement. ADEM was exclusively seen in pediatric patients. Poor prognostic indicators included: (i) incomplete recovery from an acute attack, (b) brainstem syndrome, (c) ADEM with incomplete recovery, (d) MRI suggestive of leukodystrophy pattern, (e) severe ON, (f) ADEMON., Conclusions: The Spectrum of MOG-associated disorders is wider affecting the brain (grey and white matter) and the meninges. There are various clinical phenotypes and MRI patterns, recognition of which may help in the determination of therapeutic strategies, and long-term prognosis.