A variant epidemic methicillin resistant Staphylococcus aureus-15 cavernous sinus thrombosis and meningitis: A rare occurrence with unusual presentation.

Septic cavernous sinus thrombosis (CST) is an uncommon clinical syndrome. Although Staphylococcus aureus (S aureus) is the most common bacterial pathogen causing CST, it is infrequent as a cause of meningitis. We report the first case of CST and meningitis from Bengaluru, Karnataka, caused by community-acquired epidemic methicillin resistant Staphylococcus aureus-15 (EMRSA-15), in a previously healthy individual without known risk factors; the patient recovered following treatment with vancomycin. The isolate was genotyped as belonging to staphylococcal cassette chromosome mec type IV and sequence type 22 and carried the panton-valentine leucocidin gene. It is the first Indian EMRSA-15 disease isolate from a case of meningitis. EMRSA-15 has been a major problem in hospitals in UK and it is a cause for great concern in Indian hospitals too.

Feasibility study of stroke surveillance: Data from Bangalore, India.

Background & objectives: Stroke is a leading cause of deaths, and disabilities in India. Reliable and good quality data on epidemiological characteristics of stroke are essential to plan, implement and evaluate stroke prevention and control programmes. A feasibility study was undertaken in Bangalore to examine the possibility of establishing stroke surveillance and to develop methodology for a larger programme. Methods: The study adapted WHO STEPs-STROKE methodology to collect data on hospitalized stroke events and fatal stroke events in the city of Bangalore. In STEP I, Information was collected from 1,174 stroke patients in three large hospitals and were followed till discharge and 28 days; outcome was measured as death or disability. Stroke cases fulfilling diagnostic criteria, evaluated by neurologists and CT/MRI confirmed cases were included. Brief information on major risk factors was collected from all stroke patients / family members and from medical records by trained research officers. In STEP II, death records of Bangalore Mahanagara Palike for 2004 (n=23,312) were analyzed to identify stroke related deaths. Results: Using WHO-STEPs approach, a methodology was developed for stroke surveillance in a geographically defined population. By STEP 1 method - 7 per cent of medical and 45 per cent of neurological admissions were due to stroke with a fatality rate of 9 per cent at hospital discharge and 20 per cent at 28 days. With a mean age of 54.5 (± 17.0) yr and male preponderance, nearly half had one or more risk factors. Weakness or paresis (92%) was the commonest presentation and ischaemic stroke was most frequent (73.8%). One third of total stroke patients were dependent at both discharge and 28 day follow up. By STEP II method the proportional mortality rate for Bangalore city was observed to be 6 per cent and more than 50 per cent of total stroke deaths had occurred in 10 major hospitals. Interpretation & conclusions: The present study has shown that stroke surveillance is possible and feasible. Institution based (hospitals and vital registry data) stroke surveillance supplemented with periodical population based information can provide comprehensive information on vital aspects of stroke like mortality, risk factors, disability and outcome. There is a need to develop stroke surveillance in a phased manner along with mechanisms to apply data for prevention and control programmes.

Spectrum of epilepsy in tuberous sclerosis.

Tuberous sclerosis (TS) is an autosomal dominant disease that affects the brain, skin, eye, heart and kidney. The diagnostic criteria for tuberous sclerosis complex (TSC) have recently been revised. There are relatively few Indian studies on this disorder. Twenty-six patients diagnosed as having TS over a period of 18 years are being reported. The onset of seizures ranged from infancy to adolescence. The patterns of epilepsy encountered were generalized tonic clonic seizures (13), complex partial seizures (10), simple partial seizures (9) and myoclonic jerks (4) including infantile spasms (3). Patients often had more than one seizure type. Nineteen patients were mentally subnormal. Cutaneous manifestations were facial angiofibroma i.e. adenoma sebaceum (20), shagreen patches (7), hypopigmented macules (6), ash leaf spots (4), café-au-lait spots (2), facial hypoplasia (2) and periungual fibromas (1). One patient each had retinal phakoma and renal angiomyolipoma. CT scan revealed sub-ependymal calcifications (12), parenchymal tubers (3), cerebral edema (3) and cortical atrophy (1). One patient had enhancement of peri-ventricular sub-ependymal lesions on MRI. Anticonvulsants prescribed were phenobarbitone (20), diphenyl hydantoin (14), carbamazepine (8), sodium valproate (4), benzodiazepines (4), ACTH (2), prednisone (1), mysoline (1) and vigabatrin (1). Most patients were on combinations of anti-convulsants and response to therapy was usually not very satisfactory. However, the child treated with vigabatrin did well.

Pro-thrombotic States in Stroke.

A large number of prothrombotic states both inherited and acquired, have been linked with ischemic cerebrovascular disease. Inherited deficiencies of the plasma coagulant inhibitory proteins, mainly protein C, protein S, antithrombin III, heparin cofactor-II, and protein Z, defects in the coagulation cascade proteins such as Factor V Leiden and prothrombin gene G20210A mutation, abnormalities of fibrinolysis and hyperhomocystinemia have been associated with stroke. Patients with disorders of the formed blood elements like polycythemia vera, sickle cell anaemia and essential thrombocythemia can also be predisposed to stroke. The most frequently identified acquired states associated with ischemic stroke are the anticardiolipin antibodies and lupus anticoagulants. Screening for occult prothrombotic diathesis is necessary for young patients with stroke of unidentifiable cause, for those with prior venous thrombosis and those with a family history of thrombosis. Knowledge in this area is still incomplete and evolving rapidly.

Ulnar nerve palsy due to axillary crutch.

A young lady with residual polio, using axillary crutch since early childhood, presented with tingling, numbness and weakness in ulnar nerve distribution of five months duration. Ulnar motor conduction study revealed proximal conduction block near the axilla, at the point of pressure by the crutch while walking. Distal ulnar sensory conduction studies were normal but proximal ulnar sensory conduction studies showed absence of Erb's point potential. These findings suggested the presence of conduction block in sensory fibers as well. Proper use and change of axillary crutch resulted in clinical recovery and resolution of motor and sensory conduction block.

Early onset cerebellar ataxia with retained tendon reflexes (EOCA) and olivopontocerebellar atrophy (OPCA): a computed tomographic study.

Computed tomographic (CT) studies in olivopontocerebellar atrophies (OPCA) and 'early onset cerebellar ataxia with retained tendon reflexes (EOCA)' are few and vary widely in methodology and criteria for cerebellar and brainstem atrophy. In this prospective study, CT scan observations on 26 patients (EOCA-11, OPCA-15) were compared with 31 controls using qualitative and quantitative assessment of cisterns, ventricles and atrophy of brain. Vermian and/or cerebellar hemispheric (predominantly anterior) atrophy was present in 80.8% and both were equally common. Cerebral cortical atrophy (26.9%) and leukoariosis (15.4%) were less frequently seen. Statistically significant atrophy of pons, brachium pontis, cerebellum and midbrain was noted in patient group. No significant differences were observed between EOCA and OPCA groups. Evidence of atrophy did not correlate with either the duration of illness or the severity of cerebellar ataxia in both the groups. The severity of brainstem atrophy in 14 patients with and 12 patients without abnormal brainstem auditory evoked response did not differ significantly. This study highlights the methodology of CT evaluation for brainstem and cerebellar atrophy, draws attention to cerebral atrophy and emphasizes the lack of significant differences in CT morphology between OPCA and EOCA patients.

Preceding infection as a risk factor of stroke in the young.

INTRODUCTION: The cause of stroke in the young remains unknown in 20-50% of the patients. Infections preceding stroke have been recently recognised to be an independent risk factor of stroke. MATERIAL AND METHODS: Sixty consecutive patients aged 40 years or less presenting with ischaemic completed stroke are taken up for the study. Patients with neurological deficit of less than 24 hours, evidence of haemorrhage on CT scan, infection occurring after the onset of stroke were excluded. Controls consisted of age and sex matched persons residing in the same area. Both the groups were enquired about preceding fever and infections and were examined for evidence of infections. Serum was examined for antibodies against measles, herpes simplex, and Japanese B encephalitis viruses. Cultures were put up from appropriate samples and CSF examined in patients only. RESULTS: Evidence of infection was noted in 26 (43.3%) of patients and 6 controls (p < 0.001). History of fever was elicited in 23 patients and 3 controls while 15 patients were febrile on examination at admission. Signs of local infection was observed in 14 patients and one control. The commonest site of infection was respiratory tract. Cultures were positive in 11 patients, commonest being beta haemolytic streptococci in six from throat. Conventional risk factors were identical in both groups of patients with and without evidence of preceding infection. Smoking and alcoholism were significantly higher in patients with preceding infection. CONCLUSION: Preceding infection is an important risk factor of stroke in the young. Smoking and alcoholism are more frequent in patients with preceding infection. Whether they predispose the individual for infection or infection increases the stroke risk in them needs to be examined.

Plasma antithrombin III deficiency in ischaemic stroke in the young.

A deficiency of plasma antithrombin III has been identified as a potential risk factor for thrombosis. In a pilot study of 56 patients aged less than 40 years who presented with ischaemic stroke of unknown etiology, we detected only one case of plasma antithrombin III deficiency. Antithrombin III activity was estimated by a chromogenic assay. Hence, antithrombin III deficiency, though rare, should be considered while evaluating young patients with stroke of unknown etiology.

Puerperal cerebral venous thrombosis: therapeutic benefit of low dose heparin.

Advances in imageology have improved the diagnostic yield of cerebral venous/sinus thrombosis (CVT). However, its management remains a challenge. The present study was carried out to study the role of heparin in CVT. Therapeutic outcome of 150 patients of puerperal CVT manifesting within one month of delivery or abortion, was analyzed. The diagnosis was supported by cranial computed tomography and/or angiography whenever required. Seventy three patients, 46 with non-haemorrhagic infarction and 27 with haemorrhagic infarction, received 2500 units of subcutaneous heparin, three times a day within 24 hours of hospitalization till 30th post partum day or symptomatic relief. Seventy seven patients during the same period, 50 with non-haemorrhagic infarction and 27 with haemorrhagic infarction, who did not receive heparin formed the control group. Repeat CT scans were done when indicated. Among the heparin group, 34 patients made full recovery. There were eight deaths, all among the patients with haemorrhagic lesion. In control group, only 14 patients recovered completely (P=<0.001) and 18 died (P=<0.001). There were no adverse effects of heparin. Low dose heparin is safe and efficacious in puerperal CVT, even in patients with haemorrhagic infarction.

Heparin in haemorrhagic infarction in cerebral venous sinus thrombosis.

Morbidity and mortality in puerperal cerebral venous thrombosis (CVT) can be reduced by arresting the progression of thrombosis using heparin. However, conventional dose of heparin requires monitoring of coagulation parameters and carries a risk of haemorrhage. The present study involved 56 patients of puerperal CVT with CT evidence of haemorrhagic infarction. Twenty nine of these patients received low dose heparin till 30th post-partum day or symptomatic relief. Their clinical features and severity were similar to 27 patients who did not receive heparin. The mortality and morbidity at discharge was significantly less (P < 0.001) in heparin treated group. There were no haemorrhagic complications. Low dose heparin is safe and effective in cerebral venous thrombosis, even with haemorrhagic infarction.

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.

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.

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.

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.