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Most neurological complications of the Klippel-Trénaunay syndrome are due to the presence of cerebral or spinal arteriovenous malformations and their consequences. We report 2 cases of unusual complications of this syndrome. In the first case, where the anomalies were restricted to the left upper extremity, the occurrence of hand numbness led to the discovery of a carpal tunnel syndrome, probably due to the lymphatic abnormal proliferation within the carpal tunnel. In the second case the disease was much more widespread and affecting, among other localizations, the cephalic extremity. The patient presented with intractable left motor seizures that appeared to be due to an occlusion of the right transverse and sigmoid sinuses. Treatment with heparin and anticonvulsants led to rapid recovery.

A 61 year-old man with type 2 diabetes mellitus presented with an extremely rapid and aggressive mononeuritis multiplex. Four months after onset, he had severe postural hypotension and at least 6 cranial nerves and 4 somatic nerves were involved. Extensive work-up failed to discover any etiology for the neuropathy apart from diabetes. Treatment with corticosteroids, i.v. immunoglobulins and cyclosporin was followed by progressive but sustained improvement. This case and few other published ones suggest that some particularly aggressive forms of diabetic neuropathy have an immune mechanism and may be treated favorably with immunosuppressor drugs.

A 30-year-old man was given high doses of phenytoin together with 4 antituberculous drugs for a seizure associated with a probable brain tuberculoma. He developed hepatic toxicity and his serum phenytoin reached the high level of 298 mumol/l (therapeutic range 40-79 mumol/l). All drugs were stopped and the biological parameters returned progressively to normal over the next 15 days. However, he remained with a cerebellar axial syndrome and was still severely ataxic 2 months later. Brain CT and MRI showed mild cerebellar atrophy. This case and the few other published ones, together with some recent experimental data, show that high doses of phenytoin can be toxic to the cerebellar cortical cells. The rarity of similar cases, while millions of epileptics are under phenytoin treatment, would however suggest that individual susceptibility may play a role in this toxicity.