Progressive respiratory depression and bulbar palsy: Question

Congenital oculo-bulbar palsy.

A girl developed progressive weakness of bulbar and ocular muscles starting before the age of two years. Electromyography revealed a widespread subclinical myopathy. An intercostal muscle biopsy showed complex abnormalities including occasional neurofilamentous accumulations and honeycomb-like membranous material in terminal axons. Endplates were small and some secondary synaptic clefts were ab...

On the Etiology of Myasthenic Bulbar Palsy

Thyrotoxicosis presenting as acute bulbar palsy.

Myopathy chiefly affecting the proximal muscles of the limbs is frequently seen in hyperthyroidism. But isolated acute bulbar palsy without skeletal muscle involvement is rare in thyrotoxicosis. We report the case of a 52 year old man who presented with severe dysphagia, dysphonia and bouts of aspiration. Laboratory testing revealed an underlying Graves' thyrotoxicosis. His symptoms recovered d...

Familial progressive supranuclear palsy.

A progressive extrapyramidal syndrome and dementia occurred in three members of one family. The age of onset was in the seventh decade and the affected individuals showed many of the clinical features of progressive supranuclear palsy (PSP). Necropsy of one individual revealed the neuropathological features of PSP. We propose that this family has a familial form of PSP and review the evidence i...

Progressive Supranuclear Palsy (PSP)

Introduction: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder producing characteristic eye movement abnormalities in the classic presentation. The disease pathologically is marked by accumulation of abnormal tau proteins. There are useful MRI features, however the diagnosis remains clinical in the majority of cases, and no objective diagnostic test exists. Treatment at...