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Biomedical subjects

A S Girija

Publications and source records attributed to A S Girija.

At least 19 recordsLinked to original sources

Childhood acute disseminated encephalomyelitis presenting as Foix-Chavany-Marie syndrome.

A 10-year-old boy presented with anarthria and bilateral central faciolinguovelopharyngeomasticatory paralysis with "automatic voluntary dissociation" suggestive of Foix-Chavany-Marie syndrome or anterior opercular syndrome following a nonspecific upper respiratory infection. Brain magnetic resonance imaging revealed bilateral subcortical perisylvian demyelination in addition to multiple subcortical white-matter demyelination. The clinicoradiologic picture suggested acute disseminated encephalomyelitis. The child recovered completely following intravenous methylprednisolone and intravenous immunoglobulin therapy. This is the first report of anterior opercular syndrome in childhood acute disseminated encephalomyelitis.

Child↗

Plasmapheresis in childhood acute disseminated encephalomyelitis.

Accepted modes of therapy in acute disseminated encephalomyelitis include intravenous methylprednisolone, intravenous immunoglobulin or a combination of both. Effectiveness of plasmapheresis has been demonstrated by many case reports. We used plasmapheresis to treat steroid non-responsive acute disseminated encephalomyelitis in two children. There was complete clinical and radiological recovery in both the cases.

Adolescent↗

Acute disseminated encephalomyelitis treated with plasmapheresis.

Accepted modes of therapy in acute disseminated encephalomyelitis include intravenous methyl prednisolone, intravenous immunoglobulin or a combination of both. Effectiveness of plasmapheresis has been demonstrated by previous case reports. We report two patients with steroid non-responsive acute disseminated encephalomyelitis in which plasmapheresis resulted in complete clinical and radiological recovery, though the therapy was initiated in the fifth week of illness. A total of 45-50 ml/kg body weight of plasma was removed in six equal exchanges over a period of two weeks. This report highlights that plasmapheresis could be of use even in the early second month of illness.

Acute Disease↗

Invasive aspergillosis producing painful ophthalmoplegia.

Painful ophthalmoplegia is caused by the lesions of orbital apex and anterior cavernous sinus. Cavernous sinus syndrome can be produced by intracranial invasive aspergillosis. A case of painful ophthalmoplegia due to invasive aspergillosis caused by Aspergillus niger in a diabetic patient is presented.

Diabetes Mellitus, Type 2↗

Anterior inferior cerebellar artery infarct with unilateral deafness.

We report the case of a young man with anterior inferior cerebellar artery infarct causing unilateral deafness. Clinical features and audiometry suggested cochlear localization for deafness. MRI brain showed an infarct in the right AICA territory with involvement of pons. Involvement of the internal auditory artery explains the cochlear deafness.

Adult↗

Genetic association analysis of KCNQ3 and juvenile myoclonic epilepsy in a South Indian population.

Juvenile myoclonic epilepsy (JME) is a common subtype of idiopathic generalized epilepsy that shows a complex pattern of inheritance. We have tested the association between JME phenotype and an intragenic marker in KCNQ3 by using the transmission disequilibrium test in 119 probands and their parents. Mutations in KCNQ3 are known to cause benign familial neonatal convulsions and are involved in the physiologically important M current in neurons. Our results provide suggestive evidence of allelic association between JME and KCNQ3 ( P-value=0.008) and raise an interesting possibility of a genetic contribution to JME, viz., of a gene that causes a monogenic form of human epilepsy.

Brain↗

Pyridoxine-dependent seizures: a review.

Pyridoxine-dependent seizure is a rare autosomal recessive disorder that usually presents with neonatal intractable seizures. This syndrome results from an inborn abnormality of the enzyme glutamic acid decarboxylase, which results in reduced pyridazine-dependent synthesis of the inhibitory neurotransmitter gamma amino butyric acid. The full range of symptomatology is unknown; but can be associated with autism, breath holding and severe mental retardation, bilious vomiting, transient visual agnosia, severe articulatory apraxia motor dyspraxia, microcephaly and intrauterine seizures. Parenteral pyridine injection test is a highly effective and reproducible test in confirming the diagnosis. Pyridoxine should be administered as a diagnostic test in all cases of convulsive disorders of infancy in which no other diagnosis is evident. Epileptic seizure discharges subside within 2-6 minutes after the intravenous injection of 50-100 mg of pyridaoxine. Once the diagnosis is confirmed, maintenance therapy should be continued indefinitely and doses increased with age or intercurrent illnesses. The maintenance dose of Bg needed is still not clear. There is a relatively wide range for the daily B6 dose necessary to control the seizure i.e., 10-200 mg/day.

Child↗

Primary antiphospholipid antibody syndrome presenting as venous infarct and deep vein thrombosis.

We report the case of a young lady with primary antiphospholipid antibody syndrome, who had two spontaneous abortions and cerebral venous thrombosis and subsequently deep vein thrombosis of the leg veins. Three classes of antiphospholipid antibodies (IgG, IgM, IgA) were elevated. There was no clinical or laboratory evidence for other autoimmune or systemic illnesses. We are presenting the case due to the rarity of the same.

Abortion, Spontaneous↗

Social factors and psychopathology in epilepsy.

One hundred and six epilepsy patients were assessed over a period of 6 months for psychiatric morbidity, social support, stressful life events in previous year and disability. 45 patients (42.45%) had a psychiatric diagnosis. Organic depressive disorder headed the list (16.98%) followed by mild cognitive disorder (11.32%) and tobacco dependence (8.49%). There was no significant difference in the mean age, sex, mean education, age at onset of epilepsy, duration of epilepsy, psychiatric diagnosis, mean scores on social support scale, presumptive stressful life event scale and disability assessment schedule between different types of epilepsy. The difference in mean scores of presumptive stressful life events scale and disability assessment schedule between epileptics with and without psychiatric diagnosis was not statistically significant.

Adult↗

A case of hemeplegic migraine with leptomeningeal angiomatosis.

A 43 year old man with thirty years history of recurrent hemiplegic migraine, consistently occurring on one side (left sided paresthesia and weakness of less than forty five minutes followed by right sided headache) and lately increasing to once in three days was investigated. CT head and carotid angiogram showed gyral calcification with prominent medullary and subependymal veins confirming the diagnosis of leptomeningeal angiomatosis. Management and followup is discussed.

Adult↗

Seizures in hyperglycemic patients.

AIM OF STUDY: To study the pattern of seizures in hyperglycemic patients and its correlation with serum osmolality, blood sugar and serum sodium. MATERIALS AND METHODS: (Study period June 1999-June 2000) Forty patients who presented with first time seizures and who were detected to be diabetic were included in the study. All patients had detailed history, clinical examination blood sugar, BUN, serum electrolytes, serum osmolality, serum calcium estimation. EEG and CT head scan was also done. The seizures were classified as per ILAE classification (1981). The study group was further divided into Group A--those with normal CT (n 24) and Group B-- those showing infarcts in CT scan (n 16). The control group (n 40) were matched for age and had first occurrence of seizures and CT showed infarct and were euglycemic. All the investigations were done for the control group as well. Statistical analysis was done using ANOVA. RESULTS: None of the patient had diabetic ketoacidosis. Patients in study group had statistically significant incidence of (1) Focal seizures with or without generalisation (100%); (2) Visual hallucination (25%); (3) Epilepsia partialis continua (20%). Comparing the subgroups (A and B) complex partial seizures and visual hallucination was significant at 5% level in the group A (NKH with normal CT). Ninety percent in the study group had increased serum osmolality (Vs 22.5% in the control group). Duration (eight days Vs 1) and frequency (15 per day vs 1.45) were positively correlated with RBS > S.osmolality > S.sodium. CONCLUSION: 1. Long duration and frequent focal motor seizures with or without secondary generalisation occurs in NKH which at times may be the first manifestation of DM. 2. Complex partial seizures, visual hallucination and epilepsia partialis continua are more common in NKH patients than in euglycemic patients. 3. There is positive correlation of blood sugar, S.osmolality and S.sodium with frequency and duration of seizures. RBS > S.osmolality > S.Na. 4. Correction of hyperglycemia is the main stay of management of seizures. 5. Movement induced seizures which is specific for NKH is a rarity.

Adult↗

Neuro-Behçet's disease.

Behçet's disease (BD) is a multifocal disorder with an immunogenetic basis, which persists over many years. Initial descriptions mentioned oral and genital ulcers with uveitis. Later a number of other manifestations were added, like skin, joint and neurological. The involvement of nervous system (Neuro-Behçet's) is reportedly uncommon. We hereby report four cases of Neuro-Behçet's, i.e.; two cases of strokes involving multiple areas of the central nervous system and two cases had features of benign intracranial hypertension. All cases had mucocutaneous lesions or other system involvement. Cases satisfied the international study group criteria for diagnosis of BD. All cases were pathergy test positive. In comparison with the literature from Turkey and Greece, which reports a high pathergy positivity, reports from India have shown only few cases to be positive. The prognosis of Neuro-Behçet used to be poor but has recently been improved with reduced mortality, although whether this can be attributed to treatment with steroids and/or cytotoxic agents remains uncertain.

Adolescent↗