Familial amyotrophic lateral sclerosis: first report from India.

We report two patients diagnosed to have familial amyotrophic lateral sclerosis (FALS). A 40 year old lady had progressive weakness and atrophy of the limbs and bulbar palsy from the age of 39 years and with electrophysiological evaluation was confirmed as definite ALS. Her mother had presented in 1978 at the age of 42 years with symptoms and signs of ALS. The other patient was a 43 year old male with rapidly progressive weakness, wasting and spasticity of the limbs and bulbar palsy of 4 months duration and with electrophysiological evidence of diffuse anterior horn cell involvement. His father also had onset of illness at 43 years of age with gradually progressive spasticity and atrophy of the extremities followed by bulbar palsy. In the first instance the mother had a duration of illness of 8 years while in the second the father lived for 15 years after the onset of illness.

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.

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.

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.