Prognostic indicators in cerebral venous sinus thrombosis

Cerebral venous sinus thrombosis [CVT] can affect all age groups, particularly women of childbearing age. Overall prognosis for survival and functional independence is better than it was believed. Mortality usually ranges from 6-15% and transtentorial herniation is the major cause of death. Approximately 80% of patients are functionally independent in the long term. Altered mental status and cerebral haemorrhage at presentation are the strongest predictors of death and disability. Patients with CVT related to pregnancy and puerperium generally do better than patients with other causes. Septic CVT carries a worse prognosis than aseptic CVT and of the latter, patients with syndrome of isolated intracranial hypertension have a better prognosis than those with focal deficits or encephalopathy. Anticoagulation is believed to improve outcome in CVT although robust data are lacking. Epilepsy, headaches, visual loss, pyramidal deficits and cognitive impairment are some of the long term sequelae. The risk of recurrence of CVT is low, particularly after the first 12 months of the first episode

Familial homocystinuria

Two cases of siblings diagnosed as cases of familial homocystinuria are reported. Both the cases have classical presentation of familial homocystinuria including history of dislocation of lens of the right eye. Brother had history of psychomotor retardation while sister had a significant history of deep vein thrombosis. Levels of plasma homocysteine were elevated and urinary homocysteine was positive in both the cases

Characteristics of patients with Guillain Barre syndrome at a terrtiary care centre in Pakistan, 1995-2003

To study the clinical presentation, hospital course and outcome of patients admitted with Guillain Barre Syndrome [GBS] to a tertiary care hospital in Karachi, Pakistan. The charts of patients conforming to International Classification of Diseases [ICD] code 9.0, for GBS, from September 1995 to January 2003 were reviewed. Clinical data was recorded on a standardized questionnaire, which included patients' age, sex, antecedent events, neurological signs and symptoms and ventilation requirement. The hospital course was analyzed, including nosocomial infections, therapy given and the functional status of patients, using the Rankin scale [0-6]. Standard SPSS 11.5 software [Windows] was used for data analysis. Thirty-four cases of GBS were admitted to the hospital during the study period, with an age range of 3 to 70 years. The mean age for disease onset was 35.2 years for female patients, compared to 30 years for males; the male/female ratio was 1.6:1.Gastrointestinal infections [12/22, 54.6%] were the most common antecedent event, followed by upper respiratory tract infections [9/22, 40.9%] and skin lesions [1/22, 4.5%]. Most patients developed GBS within one month of the preceding infection. Cranial nerve abnormalities [30/34, 88.2%], autonomic dysfunction [21/34, 61.8%] and respiratory failure requiring intubation [19/34, 55.9%] were also common. The median Rankin score of patients at admission, and at 30 and 60 days thereafter was 5, 4 and 3.5 respectively. The in-patient mortality was 1 of 34 [2.4%]. We found that GBS occurred at all ages and was slightly more common in males. Majority of patients had an antecedent history of infection and had severe disease on presentation. The patients were treated with either plasmapheresis or intravenous immunoglobulins and there was no significant difference in outcome in the two groups. Despite severe persistent disability, in-hospital mortality was low

Spinal cord compression: Need for high index of suspicion

Spinal cord compression in its different forms may be an elusive diagnosis and can result in permanent paraplegia. We present a case of spinal cord compression in a 40 year old female with back pain and progressive gait abnormalities. Patients may present to primary care physicians, who need to maintain a high index of suspicion for spinal cord compression in appropriate settings. History and physical examination can lead to diagnosis and management thus preventing major morbidity. The list of differentials is long and varied, but a judicious history and detailed examination can narrow it down to a few, enabling, appropriate and cost effective investigations and urgent referral to the correct speciality