Fulminant demyelinating diseases.

Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

A rare anterior spinal epidural cyst mimicking Hirayama disease.

INTRODUCTION: Hirayama disease is a rare focal motor neuron disorder that manifests as slowly progressive unilateral or bilateral hand weakness and atrophy. METHODS: The case report of a young man who presented with the phenotype of Hirayama disease indicated an extensive anterior cervical epidural arachnoid cyst. RESULTS: A 34-year-old man presented with a 5-year history of slowly progressive hand and forearm weakness and atrophy. Nerve conduction studies demonstrated low median and ulnar motor amplitudes, and EMG demonstrated fibrillation potentials and long-duration, high-amplitude motor unit potentials in C6-T4-innervated muscles. MRI demonstrated a longitudinally extensive anterior spinal epidural cyst extending from C2 to L1. The patient had improved hand strength after surgery. CONCLUSIONS: Anterior cervical epidural spinal cysts should be considered in the differential diagnosis in patients who present with slowly progressive hand weakness.

Diaphragmatic pacing to facilitate ventilator weaning in neuromyelitis optica-associated respiratory failure.

Patients with neuromyelitis optica may develop neurogenic respiratory failure in the context of severe upper cervical myelitis, which can require prolonged or indefinite mechanical ventilation. Diaphragmatic pacing has FDA approval under a humanitarian device exemption (HDE) to facilitate weaning from mechanical ventilation in the context of cervical spinal cord injury. We describe the use of diaphragmatic pacing in a patient with acute respiratory failure due to a severe attack of neuromyelitis optica on the cervical spinal cord. The device resulted in successful early ventilator weaning.