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We report two cases of central pontine myelinolysis (CPM) following liver transplantation. The incidence may well be underestimated as in the past the diagnosis of CPM was based on postmortem findings. Malnutrition, poor clinical condition, encephalopathy are common features of transplanted patients developing CPM. The clinical course is characterized by a biphasic pattern; after normal recovery from anesthesia, there is a subsequent and gradual deterioration in the neurological state. The complex syndrome associates loss of consciousness, flaccid quadriplegia and pseudobulbar palsy. Among the many factors suspected of inducing CPM, a rapid correction of natremia (> 12 mmole/l/day) seems most probable. With regards to liver transplantation, CPM presents rather specific problems. Delaying transplantation to correct hyponatremia carries a risk of severe hepatic encephalopathy. On the other hand, the intraoperative compensation of blood losses with high sodium content blood products tends to induce an abrupt rise in sodium serum concentration. Moreover, renal capacity to excrete sodium is often impaired, due to hepatic insufficiency and surgical procedure. Transplantation should not be delayed, but as infusion of large amounts of sodium cannot be avoided (fresh frozen plasma, human albumin, red blood cells), natremia may be controlled by continuous veno-venous hemofiltration with low sodium content substitution fluids.

A 37-year-old woman presented with epileptic fits and monoclonal IgG lambda type on CSF examination. A stereotactic brain biopsy disclosed a lymphoplasmacytic lymphoma with local amyloid deposits. The pathogenesis of this unusual localized amyloidosis is discussed.

An epidemiologic survey began on March 1, 1984, and ended on February 28, 1985. During this period, all neurologists and electroencephalographers of the department of Gironde, an administrative district of the French Southwest (1,128,164 residents in 1982) obtained information by questionnaire from all persons who had experienced an epileptic seizure for the first time in their lives. Recurrent, isolated, and situation-related seizures were included. Febrile convulsions and neonatal seizures were excluded. The global incidence rate of diagnosed epileptic seizures was 71.3/100,000. The incidence rates per year and per 100,000 persons by type of epileptic syndrome were 1.7 for idiopathic and 13.6 for symptomatic localization-related epilepsies, 5.6 for idiopathic and 1.1 for symptomatic generalized epilepsies, 1.9 for undermined epilepsies, 29.0 for situation-related seizures, 18.3 for isolated seizures, and 0.3 for television epilepsies. Other epileptic syndromes were not represented. Using a classification of epileptic syndromes and not of epileptic seizures reduces difficulties in an epidemiologic survey. Diagnosis of an epileptic syndrome is time dependent, however, and at follow-up some patients shift from one group to another.

A 81 year-old female, weighing 56 kgs, had taken carbamazepine (10.2 mg/kg/day) for simple partial motor seizures of recent onset during 15 days. EEG recording showed slow waves on the left hemisphere. CT was normal. The seizures were controlled for a few days and then reappeared beginning as simple partial seizures and progressing to impairment of consciousness. The patient became confused. On admission she was severely confused. Neurological examination was otherwise normal. Plasma sodium level was 134 meq/l and CBZ plasma level was 4.3 micrograms/ml. EEG showed high voltage bilateral delta waves and a left focus. The drug was withdrawn with relief of clinical symptoms and to a lesser extent, of EEG abnormalities. Phenytoin encephalopathy is a well known pathological entity but to our knowledge there has been no report of carbamazepine encephalopathy with neither excessive plasma level of the drug nor water intoxication. An idiosyncratic reaction is considered likely.