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Biomedical subjects

D Nagaraja

Publications and source records attributed to D Nagaraja.

At least 37 records · Page 2Linked to original sources
PubMed1996

Basal ganglionic angioleiomyoma.

A rare case of basal ganglionic angioleiomyoma in a 12-year-old girl is reported. She presented with features of raised intracranial tension, multifocal seizures, left hemidystonia and apraxia of eyelid closure. Cranial CT scan showed a large hypodense lesion with an enhancing mural nodule in the region of head of the caudate nucleus on the right side. The lesion was extending to the posterior limb of internal capsule postero-medially and to globus pallidus laterally. Another small, hyperdense, enhancing lesion was seen in the region of globus pallidus on the left side. The right basal ganglionic mass was excised through frontal craniotomy. Histopathological, Immunocytochemical and Ultrastructural examination of the right basal ganglionic mass showed features characteristic of angioleiomyoma. Following surgery the patient improved initially with respect to dystonia but worsened later on. Repeat cranial CT, 20 months after surgery, showed bilateral frontotemporal atrophy without any tumor recurrence while the lesion on the left side remained quiescent.

Angiomyoma↗
PubMed1996

Case-control study of serum lipoprotein(a) and apolipoproteins A-I and B in stroke in the young.

OBJECTIVES: Abnormalities of lipoprotein(a) and apolipoproteins A-I and B are being recognised as independent risk factors in ischaemic heart disease and atherosclerosis. There are no studies from India where stroke in the young constitutes nearly 20-30% of all strokes. SUBJECTS AND METHODS: Fasting serum lipids, lipoproteins, apolipoproteins A-I and B and lipoprotein(a) were measured in 50 patients aged less than 40 years presenting with completed stroke and 50 normal, age and sex-matched control subjects. Apolipoproteins A-I and B were measured by immunoturbidimetry and lipoprotein(a) by enzyme-linked immunosorbent assay. RESULTS: The serum total cholesterol, HDL-cholesterol, LDL-cholesterol, VLDL-cholesterol, triglycerides and apolipoproteins A-I and B were not significantly different in the test group as compared to the controls. However, serum lipoprotein(a) was significantly higher in the young stroke patients. CONCLUSION: These findings confirm the hypothesis that an elevated serum lipoprotein(a) level is an important risk factor in the development of cerebral ischaemia in patients aged less than 40 years. It may be worthwhile to study whether it is useful in identifying patients most at risk for stroke.

Adolescent↗
PubMed1995

Recurrent Guillain Barre' Syndrome: a clinical, electrophysiological and morphological study.

Of the 220 patients of acute idiopathic demyelinating polyneuritis (AIDP/GBS) seen over a seven year period, 15 patients (M:F:11:4) had a relapsing course (6.8%). Their ages ranged from 8 yrs to 70 yrs. They had 36 episodes at a variable interval of 3 months to 25 yrs. Relapse rate varied from one to four. Antecedent events were noted during 16 episodes in 9 patients but the triggering factors were varied. Clinical features of individual episodes were similar to the acute monophasic illness, although they differed inseverity from one episode to the other. Autonomic disturbances were rare. Albuminocytological dissociation was observed during 19 of the 24 episodes. Electrophysiological abnormalities were observed during 19 of the 24 episodes. Electrophysiological abnormalities were present in all and were comparable with patients of non-recurrent illness. Sural nerve biopsy in 3 patients showed evidence of demyelination, remyelination, Wallerian degeneration and myelin breakdown but none had features of inflammation. With the exception of one death, functional recovery was complete in the majority of patients, irrespective of the type of therapeutic intervention. Acute onset, frequent facial involvement, brief clinical course, near complete recovery and very long asymptomatic periods may distinguish these patients of acute relapsing demyelinating polyneuropathy (ARDP) from chronic relapsing demyelinating polyneuropathy. Relapses in GBS are however unpredictable and recurrent GBS is indistinguishable clinically, electrophysiologically and morphologically from the more frequently seen non-recurrent form of monophasic GB Syndrome. A biochemical or immunological marker may help in this distinction.

Adolescent↗
PubMed1995

Disseminated Burkitt's lymphoma presenting as multiple cranial nerve palsies.

A case of disseminated Burkitt's lymphoma with nervous system involvement in a HIV negative 35 year old lady is described. She primarily presented with multiple cranial nerve palsies. At autopsy, diffuse involvement of parenchymatous organs and lymphomatous meningitis with conspicuous sparing of gastrointestinal system was observed. In addition, there was an unusual feature of paraneoplastic demyelinating peripheral neuropathy. Incidentally, a large hydatid cyst was also seen in the left lobe in addition to the lymphomatous involvement of the liver.

Adult↗
PubMed1995

Early onset cerebellar ataxia with retained tendon reflexes: a clinical, electrophysiological and computed tomographic study.

Fourteen patients of Early Onset Cerebellar Ataxia with retained tendon reflexes (EOCA) were prospectively evaluated clinically, electorphysiologically and with CT scan. Their age and duration of symptoms were 18.6 +/- 8.3 years and 7.8 +/- 5.1 years respectively. High consanguinity (91.7%) and positive family history (76.9%) suggested autosomal recessive inheritance. Apart from cerebellar signs and brisk knee jerks in all, other important findings were abnormal ocular movements (mostly impaired saccades) in 92.8%, Babinski's sign (78.6%), brisk ankle jerks (64.3%), spasticity in lower limbs (50%) and impairment of proprioceptive sensations (50%). Neuropsychological tests, done in 12 patients, were abnormal in all. Abnormalities of electroneuromyographic studies were universal, motor conduction parameters (85.7%) being more affected than sensory (78.6%). One or more modalities of evoked potentials were abnormal in 71.4%, that of brainstem auditory evoked response being most frequent (50%), followed by posterior tibial somatosensory evoked potential (SSEP) (46.1%) and median SSEP and visual evoked potential (30.8% each). CT scan (n = 12) showed atrophy of brainstem (91.7%), cerebellar hemisphere and/or vermis (83.3%) and cerebral cortex (33.3%). There was no correlation between the duration of disease and degree of disability or abnormalities of nerve conduction and CT parameters. The rationality of the diagnosis of this recently recognised entity of 'EOCA' in Indian context is discussed.

Adolescent↗
PubMed1995

Acute inflammatory demyelinating neuropathy: a critical evaluation of diagnostic criteria for demyelination.

There is agreement on the clinical diagnostic criteria for acute inflammatory demyelinating polyneuropathy (AIDP/GBS) however, there is lack of consensus for detection of demyelination. In order to critically evaluate the prevailing criteria, sixty-six patients who fulfilled NINCDS criteria and had typical features of GBS were studied for electrophysiological abnormalities of peripheral nerves by using standard methods (median, common peroneal, sural and ulnar) between 1 to 12 weeks after the onset of symptoms. The commonest abnormality on motor nerve conduction study was prolonged distal latency (75%-83%) followed by reduction in CMAP amplitude (63%-82%), decreased velocity (48%-62%), conduction block (17%-39%) and f-wave abnormalities (37.8%-59%). Sensory conduction abnormalities were detected in over 20% of median, 25% of ulnar and 33% of sural nerves. All the patients had abnormality of at least two motor conduction parameters in one nerve when values beyond 2 SD of the mean were considered abnormal and over 70% of patients had three abnormalities in two nerves or two abnormalities in three nerves. Comparison with the prevailing criteria for demyelination revealed that the number of patients fulfilling them varied widely: Albers et al. (1985): 74.2%, Albers et al. (1989): 40.9% and Cornblath: 30.3%. We believe that the current criteria for detection of demyelination in acute neuropathy are too strict, underestimate the underlying pathology in GBS and need reassessment.

Adolescent↗
PubMed1994

Acute idiopathic axonal neuropathy (AIAN): a clinical and electrophysiological observation.

Twenty patients (M:F 15:5) with electrophysiological evidence of predominant axonal lesion and fulfilling clinical criteria for Guillain Barré Syndrome were observed during a period of 6 years (1985-1990). Their mean age was 27.5 years (range 5-55). Seven patients had antecedent febrile illness. Peak motor deficit was reached at a mean period of 6.5 days (range 2-21 days). All the patients had distal muscle weakness out of proportion to proximal muscle weakness. Facial paresis (13 patients), bulbar palsy (2), respiratory failure (1), sensory deficits (7) and dysautonomia (1) were other salient features. CSF analysis revealed albumino-cytological dissociation in 12 patients. One patient died and in the remaining patients the recovery was delayed and incomplete. Presence of predominant distal muscle wasting and weakness, low amplitude CMAP or inexcitable nerves, absence of conduction block or significant temporal dispersion, normal or only slightly reduced conduction velocity and evidence of poor recovery suggest that the primary pathology in these patients may be axonal degeneration. These cases may represent a distinct entity and need to be differentiated from the more commonly observed acute idiopathic demyelinating neuropathy.

Acute Disease↗
PubMed1994

Association of Japanese encephalitis virus infection with Guillain-Barré syndrome in endemic areas of south India.

This study is a report of 34 cases of Guillain-Barré syndrome (GBS) observed in Bangalore (South India), an endemic area for Japanese encephalitis virus (JEV) infection. Virological and immunological findings suggested an antecedent and recent JEV infection in 21/34 patients. Nineteen patients among them showed high levels of JEV-specific IgM antibodies in serum and/or CSF, while the viral antigen could be demonstrated in one case and virus isolation from the CSF was successful in one patient. EMG studies revealed features of predominantly demyelinating neuropathy in 18/25 cases. Comparison of clinical findings, duration of illness and outcome in GBS patients with evidence of JEV infection and those without did not reveal any differences. Pathological findings in one patient corroborated the association of JEV with GBS. We conclude that, JEV infection may predispose to Guillain-Barré syndrome in endemic areas.

Adolescent↗
PubMed1994

Cerebrovascular disease in children.

Stroke although rare in children, is an important cause of morbidity in the paediatric age group. Over a period of 8 years, 43 children (17 boys and 26 girls) in the age groups of 1-16 years (mean 8.02 yrs) presented with stroke which constituted 10% of all strokes in the young and 0.7% of all paediatric admissions. The chief clinical features were hemiplegia (86%), convulsions (27%), fever (23%), dysphasia (23%), headache (11%) and altered level of consciousness (11%). Routine laboratory tests were non-contributory. Cranial computerized tomography (CCT) on 21 patients was abnormal in 95% and was useful in revealing the extent of infarction. Infarction was confined to middle cerebral artery territory, often involving basal ganglionic structures and was associated with focal or diffuse atrophy. Angiograms were abnormal in 78% of the patients (18/23) and were complimentary to the CCT. Etiological factors identified were: Moya-moya disease 6, arteritis 5, fibromuscular dysplasia 2, scorpion sting 2, and venous sinus thrombosis and small vessel occlusion one each. Though 23% of the patients had fever at onset, no obvious evidence of systemic or CNS infection was noticed. Stroke in children continues to pose a diagnostic challenge.

Adolescent↗
PubMed1994

Critically ill Guillain Barre' syndrome.

Among the 153 patients fulfilling NINDS criteria for Guillain Barre' Syndrome (GBS) seen over 5.5 yrs, there were 47 (M:F 38.9) critically ill patients (age range 4 to 60 years). Antecedent event was recorded in 25 patients and the peak deficit was attained over a mean period of 9.5 days. Besides severe motor paralysis other salient features were: bulbar paralysis--42, sensory symptoms or signs--21, dysautonomia 31 and requirement for ventilatory assistance 45. CSF protein was raised in 63% cases. All the 17 patients who underwent electromyography had abnormalities of nerve conduction paramentes. Mean stay on the ventilator was 29.6 days and was not influenced by corticosteroid. Complications were frequent: pulmonary and urinary tract infection, dysautonomia, electrolyte disturbances, haemetmesis, bleeding from tracheostomy site and hepatic and renal failure. Mortality in steroids treated group (13/27) and the conservatively managed group (5/20) did not differ significantly. No discriminant factor emerged between survivors and non-survivors. Age and sex of the patients, presence of antecedent event, onset to peak interval and CSF protein level did not predict the need for ventilatory assistance, although these patients at admission had more frequent weakness of facial, bulbar, trunk, neck and proximal muscles of upper limbs and autonomic disturbances. Course of GBS remains unpredictable at the onset of the disease, warrants close supervision and meticulous supportive care and remains a therapeutic challenge.

Adolescent↗
PubMed1992

Epidemic of acute inflammatory myopathy in Karnataka, south India: 30 cases.

Thirty patients of acute inflammatory myopathy were seen over a short period of 11 months (February to December 1986) at NIMHANS, Bangalore, South India. The characteristic features were: short febrile illness followed a few days later by myalgia, edema of extremities, severe motor weakness and involvement of multiple other systems. Their mean age was 32.3 years and M:F ratio was 4:1. CK levels were increased in all. EMG done in 23 patients showed spontaneous activity in 13 and myopathic pattern in all. Nerve conduction studies revealed abnormalities in 12 cases. Muscle biopsy done in 21 patients showed varying degree of myophagocytosis and inflammatory infiltrates. All patients received steroids for only 6-8 weeks. Twenty-two patients recovered, one developed residual disability and 7 patients died during the acute phase. None of the survivors has developed relapse so far. Such cases with monophasic illness in clusters have not been reported earlier.

Adolescent↗
PubMed1991

Angiographic profile of ischaemic stroke in the young--study of 143 cases.

The incidence of stroke in the young is higher in the Indian subcontinent than in the West, but the precise cause is not known. Previous angiographic studies in cases of "young stroke" have yielded variable results. The present angiographic study of 143 cases of young stroke (under 40 years of age) showed a high incidence of abnormality (71%). The lesions included atherosclerotic disease of the internal carotid artery (68%), stenosis/occlusion of the major intracranial vessels (37%) and small-vessel disease. Tandem lesions were common (26%). Primary atherosclerosis is thought to be the cause of young stroke in the Indian population.

Adult↗
PubMed1991

Acute ataxic neuropathy: a clinical, electrophysiological and morphological study.

Sensory ataxia as the chief manifestation of acute neuropathy is rather rare. Of the 224 cases of acute polyneuropathy seen over 6 years (1984-1990) only 10 patients (M:F 3:7) had disabling ataxia as the presenting feature. Their ages ranged from 14-61 years. Antecedent febrile illness was present in 6 patients and the peak deficit evolved over 2-25 days. Severe ataxia, paresthesia, distal areflexia and predominant joint sense loss were common to all, motor weakness was either absent or insignificant. CSF was acellular and revealed elevated protein in 3 subjects. All patients had electrophysiological evidence of severe sensory neuropathy with mild or no motor neuropathy. Sural nerve biopsy in one patient showed loss of large, as well as small, diameter myelinated fibres, secondary demyelination, but no evidence of inflammation. At follow up marginal to moderate improvement in ataxia was noted in only 5 patients. Absence of ophthalmoplegia and motor weakness, poor prognosis and characteristic electrophysiological and histopathological observations suggest that acute ataxic neuropathy may be a distinct entity.

Adolescent↗
PubMed1990

Vertebro-basilar insufficiency due to carotid stenosis.

Two patients with internal carotid disease and normal/hypoplastic vertebro-basilar system presenting with symptoms of vertebrobasilar insufficiency are presented. The role of associated anomalies of the circle of Willis or major vessels in the pathogenesis of symptoms is highlighted.

Carotid Artery Diseases↗
PubMed1990

Hypothermia: an unusual manifestation of Reye's syndrome.

A four year old girl manifested with seizures, raised intracranial tension and altered consciousness. Laboratory investigations suggested Reye's syndrome. Later she developed fatal hypothermia. Rarity of such a case is highlighted. Probable pathogenesis of hypothermia in Reye's syndrome is discussed.

Biopsy↗
PubMed1990

Simultaneous cerebral arterial and venous thrombosis.

Simultaneous thrombotic occlusion of cerebral arterial and venous systems in the same individual is rare. Three patients of stroke in whom autopsy revealed co-existing arterial and venous infarctions, major arterial occlusion (vertebral 1, internal carotid 1) and dural sinus and cortical vein thrombosis are reported. Role of infarcted brain, haemorrheological factors and alcohol in the pathogenesis of this rare event is highlighted.

Adult↗