Oromandibular dystonia and persistent psychiatric symptoms in extra-pontine myelinolysis.

A 32 years female presented with gradually progressive dysarthria, dysphagia, oromandibular dystonia and mild generalized weakness. She had several episodes of acute psychotic behavior. She had abnormal saccadic eye movements, generalized hypertonia and exaggerated jerks in upper limbs. She was previously treated in a peripheral hospital for severe vomiting and diarrhea. MRI of brain revealed symmetrical T-2 weighted hyperintensities in bilateral putaminal and caudate region along with pons and midbrain suggesting demyelination due to a metabolic insult. Her power improved gradually over days and the dysarthria, dysphagia and oromandibular dystonia improved gradually over several weeks with supportive measures but the psychiatric manifestations are still persisting.

Wilson's disease--unusual features.

Two cases of Wilson's disease with unusual features are reported. In one case neurological abnormality was the presenting feature without any clinical involvement of the liver. In the other case, neurologic manifestations were associated with rickets and cholelithiasis, a result of chronic hemolytic state. Apart from clinical profile both the cases were diagnosed by grossly reduced serum ceruloplasmin level. However, Kayser-Fleischer rings were found in each case.

The lateralizing significance of version of head and dystonic limb posturing in epileptic seizures.

Several clinical signs have been described for lateralization and localization of seizure focus in complex partial seizures; however, the specificity of each of these has been widely debated upon. The present study was done to evaluate 'dystonic unilateral limb posturing' and 'versive head movements' for lateralization and localization of epileptic foci in patients with intractable partial complex seizures, being investigated with long term Video-EEG monitoring. Fifteen patients with 46 seizures, studied with long term Video EEG, had either one or both of these signs. The video recordings of the clinical behaviour were noted and later compared with the corresponding EEG. Unilateral dystonic limb posturing and versive head movements had good lateralising value in complex partial seizures, suggestive of temporal lobe origin, contralateral to the seizure focus, with a specificity of 87.5% and 86% respectively. In addition, turning of the whole body to one side was associated with a contralateral seizure focus in 100% cases. Appendicular automatisms were found to be of no lateralising significance, as they occurred on either side with equal frequency. These motor phenomena should, therefore, be enquired about in detail, during history taking for lateralization or localization of seizure focus.