RESUMO
A 36-year-old female with serum anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (NMOSD) on carbamazepine (CBZ) therapy for paroxysmal tonic spasms (PTS) developed Type 1 respiratory failure. High-resolution computed tomography chest showed diffuse ground-glass opacities in both lungs predominantly in bilateral perihilar region sparing subpleural regions and predominantly upper lobes with a smooth interlobular septal thickening. A transbronchial lung biopsy was consistent with hypersensitivity pneumonitis and following withdrawal of the CBZ and treatment with steroids her respiratory symptoms resolved. After stopping CBZ, PTS recurred, which was successfully treated with lacosamide. This is the first described biopsy-proven case of CBZ-induced hypersensitivity pneumonitis in the NMOSD patient.
RESUMO
Hirayama disease is a rare restricted form of motor neuron disease. It commonly affects young males. Patients typically presentwith the insidious onset of unilateral weakness and atrophy of the hand muscles that often progresses to the forearm. In somecases, the syndrome is bilateral but often asymmetrical. Of note, the brachioradialis muscle is usually spared. The syndromeaffects C7–C8–T1 muscles with sparing of the C5–6 muscles. We report a case of a 25-year-old male who presented with 2years of history of progressive wasting and weakness of muscles of bilateral hands and forearms. Based on clinical features,electrodiagnostic studies and dynamic magnetic resonance imaging cervical spine diagnosis of Hirayama disease were made.The patient was treated conservatively with a cervical collar. Over a period of 8 months follow-up, no progression was seen.