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Biomedical subjects

A B Taly

Publications and source records attributed to A B Taly.

At least 19 recordsLinked to original sources

Obsessive-compulsive disorder and rheumatic chorea: is there a connection?

There is increasing evidence to suggest basal ganglion involvement in the aetiopathogenesis of obsessive-compulsive disorder (OCD). Twenty subjects with rheumatic chorea were assessed for presence of OCD and evaluated on Leyton's Obsessional Inventory. Four subjects had OCD. The study group had markedly higher scores on all the scales of Leyton's Obsessional Inventory. The findings implicate that caudate dysfunction is not sufficient on its own to explain the presence of obsessive-compulsive behaviour and that additional basal ganglion regions/rostral connections also have to be involved to produce co-morbidity.

Adolescent

Sympathetic skin response in Guillain-Barré syndrome.

Dysautonomia is a common feature of Guillain-Barré (GB) syndrome and is sometimes the cause of significant morbidity and death. Changes in sympathetic skin response (SSR) may be one of the accompaniments of dysautonomia. An attempt was made to correlate SSR changes with clinical and electrophysiologic features in a group of 24 patients with GB syndrome fulfilling NINDS (National Institute of Neurological Disorders and Stroke) criteria. A total of nine patients had absent SSR. Thirteen patients had clinical dysautonomia, of whom five had absent SSR. Five patients had features of predominant axonal damage and preserved SSR. A trend towards correlation of SSR abnormalities with common peroneal nerve conduction parameters (velocity and compound muscle action potential amplitude) was noted. We conclude that SSR abnormalities are common in GB syndrome and may be complementary to bed-side tests for autonomic dysfunction.

Action Potentials

Sympathetic skin response: a decade later.

Sympathetic skin response (SSR) is a simple, reproducible test of function of a polysynaptic reflex having diverse afferents, a common efferent pathway through the spinal cord, pre and post-ganglionic sympathetic fibers and with sweat glands as effectors. The reflex is co-ordinated in the posterior hypothalamus or upper brainstem reticular formation. It has been used in a variety of disorders of peripheral and central nervous system. Methodology, possible anatomic substrates, changes in SSR in various diseases and their correlation with clinical features of dysautonomia, bed side tests for dysautonomia and other electrophysiological parameters are critically evaluated. Almost a decade after the start of its widespread clinical utilization, several aspects of SSR remain inconclusive. A consensus as to what change in SSR to consider abnormal is yet to be reached. Though its ease of application supersedes a variety of other autonomic function tests, relying only on SSR changes for prognostication or therapeutic decisions appears impracticable. A battery of tests is thus a necessity.

Axons

Sympathetic skin response in acute sensory ataxic neuropathy.

Sympathetic skin response (SSR) is a recently described objective method of studying sudomotor sympathetic nerve function and has been studied in a variety of peripheral neuropathies. We report SSR changes in nine patients with acute sensory ataxic neuropathy (ASAN). All had severe sensory and mild motor nerve conduction abnormalities; five had dysautonomia. SSR, elicited by electric shock and cough stimuli, was absent in three patients. Latency was normal in all when SSR was present. Two patients had SSR amplitude of 0.2 mV or less. Absence of SSR did not correlate with dysautonomia, absence of sensory nerve action potential or motor nerve conduction abnormalities. Follow up SSR studies revealed return of absent SSR in one patient over a period of 3 months, despite persistence of ataxia. To our knowledge, this is the first report of SSR changes in ASAN.

Action Potentials

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

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

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

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

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

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

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

Limb girdle myasthenia: a study of familial and sporadic cases.

Chronic limb girdle myasthenia gravis (MG) is a rare entity. We describe six such patients (F:M 4:2) who constituted 5% of 120 MG cases in a seven year study. The disease was familial in four and sporadic in two. No patient had ocular muscle weakness either at presentation (mean of 18.2 months after onset of illness) or during a mean follow up period of 36 months. Diagnosis was established by a positive decremental response on repetitive stimulation of a proximal muscle. Muscle biopsy was essentially normal in all five patients. All patients responded to acetylcholinesterase inhibitors, although to varying degrees. Four patients also received steroids. One patient with sporadic MG had transient worsening but others showed partial improvement. It is noteworthy that the initial diagnosis in these patient was other than MG. Diagnosis of limb girdle myasthenia needs to have a strong index of suspicion as it has therapeutic implications.

Adult

Involvement of peripheral nervous system in juvenile Parkinson's disease.

We evaluated, by using electrophysiological techniques, 29 patients with juvenile Parkinson's disease (JP), who had no known causes or clinical signs of neuropathy. Electromyographic evidence of chronic partial denervation with reinnervation was observed in nine patients (34.6%). Abnormalities of motor conduction in the common peroneal nerve were present in four (13.8%), Sural sensory conduction in nine (31.9%) and sympathetic skin response (SSR) in eleven (37.9%) patients. The symptoms of dysautonomia correlated poorly with changes in SSR. These abnormalities were independent of age at onset, duration or severity of the disease and antiparkinsonian drugs used. This study suggests that the peripheral nervous system is involved in JP in more than 50% of patients. The commonly observed symptoms of dysautonomia in Parkinson's disease may have a peripheral origin.

Adult

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

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