Biomedical subjects
Publications and source records attributed to J P Viana.
Myoclonus can present itself in various distinct clinical contexts. The authors review the possible different types of myoclonus, as a single manifestation or included in a syndrome, based on a semiological and aetiological classification. Special emphasis is given to the group of progressive myoclonus epilepsies.
The authors describe a case of hereditary neuropathy with liability to pressure palsies (entrapment), and compare it to reports from literature. The main characteristics are: autosomal dominant inheritance, recurrent mononeuropathies (ulnar, median, peroneal, brachial plexus), and specific features at nerve biopsy. The sensory nerve reveals predominantly demyelinating alterations, having the remaining myelin fibres focal thickenings, the so called tomaculous, and shows numerous subperineural structures named Renaut Bodies. The EMG findings show a slowing of the nerve conduction velocities and an increase of distal latencies.
Status Epilepticus can assume as many forms as there are types of epileptic seizures, and its clinical outcome largely depends on the etiology. Convulsive Status Epilepticus is a common emergency associated with high morbidity and mortality, but prompt and appropriate pharmacological therapy can reduce both. The authors present a rational treatment protocol for this syndrome.