Biomedical subjects
Publications and source records attributed to A B Taly.
A patient with Sturge-Weber-Dimitri disease presented with intractable seizures and progressive intellectual deterioration. There was no facial nevus or focal neurologic abnormality. CT disclosed bilateral calcification in a parieto-occipital gyral pattern. Histopathology of the brain revealed extensive calcification of vessel wall in parieto-occipital cortices.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Computerized tomography (CT) is widely used in the diagnostic work-up of epilepsy. However, there are only scant reports of transient postictal CT abnormalities. Three patients who presented with elementary partial seizures and manifested transient CT findings are presented. The need for an adequate ictus-free period for CT evaluation of seizure disorders is emphasised.
Explore the source record for details and available documents.
This article presents a study of a family in which association of hyperuricaemia and more interestingly increased C.S.F. uric acid was noticed in two members who had classical dystonia musculorum deformans (DMD). Other members of the family had no clinical evidence of DMD but had increased serum and C.S.F. uric acid. Investigations did not reveal any of the common disorders of the purine metabolism in any of the family members. An attempt has been made to discuss the aetiopathological correlation.
Clinical, histological, immunohistochemical and ultrastructural features of 5 cases of inclusion body myositis -4 sporadic (s-IBM) and one hereditary (h-IBM) form are described. These patients (3 men, 2 women) had chronic progressive weakness of varying severity in all 4 extremities with sparing of cranial muscles. Elevation of CPK was noted in 2 patients. Electromyography revealed features of myopathy in 4 and additional neurogenic changes in 2 subjects. Clinical diagnosis was often other than inclusion body myositis. Presence of characteristic eosinophilic inclusions within the vacuoles established the diagnosis. The inclusions were congophilic and showed positivity to ubiquitin, beta-amyloid and SMI-31 in the sporadic cases while congophila was absent in the hereditary form. Immunostaining to hyperphosphorylated-tau was negative in both s-IBM and h-IBM. Membraneous whorls were observed at ultrastructural level. None of the patients improved with steroids and trial with other immunosuppressants was unsuccessful.
We report a patient with glioblastoma multiforme who was subsequently diagnosed to have Wilson's disease. Immunohistochemical studies of the tumor revealed high (> 60%) labeling index for p53 and Rb retinoblastoma protein. Whether this association is like the co-occurrence of retinoblastoma and Wilson's disease due to possible somatic mutation in chromosome 13 needs to be explored.
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.