Thirteen cases of vacuolar myopathy (6 males, 7 females), with age range of 4 months to 22 years and diagnosed over a period from 1986 to 1999, could be categorized into acid maltase deficiency (AMD) (n=6), carnitine deficiency (CD) (n=5), and mitochondria-lipid-glycogen myopathy (MLGM) (n=2), cases of AMD presented as floppy infants with reparatory infection, while cases of carnitine deficiency presented with progressive motor weakness with normal initial milestones. Delayed motor milestones and proximal muscle weakness was the presenting complaints in MLGM. The diagnosis in all these cases was established based on the morphological findings on muscle biopsy, namely demonstration of PAS positive material within the vacuoles in AMD, ragged red fibers, vacuoles containing neutral fats and abnormal mitochondria in CD. MLGM was characterized by the presence of PAS positive material and neutral fat. The diagnosis was confirmed by identification of abnormal mitochondria under electron microscope. The storage product appears to affect not only the muscle metabolism but also the normal structure function relationship. The study highlights the importance of supplementing routine histopathology with muscle histochemistry and election microscopy to delineate the conditions, which look similar in routine histology.

Despite the unquestionable superiority of Transesophageal echocardiography (TEE) over Transthoracic echocardiography (TTE), the impact of TEE on the long term management of cardio-embolic strokes remains uncertain. The purpose of present study was to determine the extent to which TEE, influenced the decision regarding long-term anticoagulant management of patients with ischemic stroke in a University Hospital. Between January 1992 and May 1995, TEE was performed in 122 out of 485 consecutive patients with ischemic stroke enrolled in the Maryland Stroke Data Bank. The indication to perform TEE was unexplained cerebral infarction. Twenty five patients had a possible cardiac source of embolism (CSOE) detected only after a TEE out of whom 17 were placed on anticoagulant therapy. From the remaining 8 patients, 2 expired, 2 were already receiving anticoagulants for different reasons and 4 were managed with antiplatelet agents. To conclude, in 17 of 122 ischemic stroke patients (14%) who underwent TEE, the decision regarding long term anticoagulation was made only because TEE revealed a cardioembolic source.

A six year old boy having complex partial seizures with secondary generalization of four months duration developing isolated expressive dysphasia, later progressing to global aphasia is being reported. His awake EEG showed a left temporal spike wave discharge and sleep EEG showed continuous spike and ware discharges. MR imaging demonstrated focal cortical dysplasia in the left frontal and opercular region, a combination that has not been reported earlier.

A report of patient with nonembolic ischemic cerebral infarction, developing a massive hemorrhagic transformation of the infarct with neurological deterioration on the 15th day after the onset of the ischemic stroke is presented. The hemorrhagic transformation occurred in the absence of hypertension, cardiac source for embolism, coagulation defects, thrombolysis or anticoagulation therapy. It is important to recognize that hemorrhagic transformation can also occur in the natural evolution of an ischemic infarction or with simple therapy like low dose aspirin.

Trigeminal neuropathy is the most common CNS disorder in Sjogren's syndrome. It is believed to be caused by vasculitis. Unless this is recognised, a diagnosis of trigeminal neuralgia is often made. The therapeutic response to steroids is unpredictable. There are two subgroups - those with associated collagen disorders and those only with the sicca syndrome.

Subdural and Intracerebral Hemorrhage, occuring simultaneously in a patient is a very rare condition. The few case reports found in literature occurred in situations of trauma, coagulopathy, CNS malignancy and in dialysis dependant patients. We report one such case where both conditions coexisted, in the background of poorly controlled hypertension. The possible pathogenesis in this case is discussed.