Neuromyelitis optica--report of an autopsy proven case from South India.
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Biomedical subjects
Publications and source records attributed to D Nagaraja.
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This prospective study was undertaken to characterize better electrophysiologically the newly described clinical entity of "Early onset cerebellar ataxia with retained tendon reflexes (EOCA)" and compare it with Olivopontocerebellar atrophy (OPCA) and Friedreich's ataxia (FA). Concentric needle electromyography and motor (median, common peroneal and posterior tibial) and sensory (median, sural and posterior tibial) nerve conduction studies were carried out in 14 patients of EOCA. The results were compared with those of 10 and 16 patients of FA and OPCA respectively. All patients of EOCA had either motor or sensory conduction abnormalities, motor being slightly more frequent than sensory (87.7% versus 78.6%). The neuropathy was distal and symmetrical, lacked correlation with duration or clinical stage of the disease, even between patients of the same family. Electrophysiological studies helped to detect subclinical motor and sensory neuropathy in most of the patients. No characteristic electrophysiologic abnormalities separated patients of EOCA from those of OPCA or FA, though the overall incidence of abnormalities was higher in the latter two groups. It is concluded that subclinical peripheral neuropathy is often present in patients of EOCA. The impaired proprioceptive sensation noted among these patients may be due to large fiber neuropathy rather than posterior column involvement alone. A subgroup of them, who have severe sensory neuropathy, may be difficult to differentiate clinically from patients of FA. The clinical entity of EOCA is indistinguishable electrophysiologically from FA and OPCA.
Studies of sensory system involvement in Guillain Barre' (GB) Syndrome are sparse in the literature. This communication presents the clinical and electrophysiological data of 100 patients of GB Syndrome evaluated over 5 years at NIMHANS, Bangalore, India. Sensory symptoms or signs were present in 45% and 59% of patients in upper and lower limbs respectively and were distal and symmetrical. Impairment of joint position and vibration sense was the commonest finding and was associated with a greater need for ventilatory support and autonomic disturbances. Sensory nerve conduction studies involved median, ulnar and sural nerves and electrophysiological evidence of abnormality was present in at least one sensory nerve in 80% of cases. These were: absent sensory nerve action potential (SNAP) in 19%-41%, reduced SNAP amplitude in 28-34% and reduced conduction velocity in 8%-15%. "Abnormal Median and normal Sural response", the pattern characteristic of demyelinating neuropathy, was noted in 29% of the cases. While sensory symptoms and signs were more frequent in lower limbs, electrophysiological abnormalities were more frequent in median and ulnar nerves. There was good association between motor and sensory conduction parameters in median and ulnar nerves. The time of performance of nerve conduction studies did not influence the results. Electro-clinical study of sensory system adds to our understanding of GB Syndrome.
A patient with SSPE who had prominent diffuse white matter hypodensities on CT scan indicating central white matter demyelination and nerve conduction abnormalities suggestive of peripheral neuropathy is reported. Diagnosis was established by demonstration of elevated CSF measles antibody titers and presence of virus antigen and nucleocapsid in peripheral nerve. To our knowledge, this is the first report of demonstration of measles virus nucleocapsides and antigen in the peripheral nerve in SSPE.