Lydia L. Pauli

BEHAVIOR: Masturbation Simulating Epilepsy

Tlic seven-year-old girl was referred to our center for “better seizure control.” She \\.as tlie product of a nine-month uncomplicated pregnancy, normal delivery, and wiglied 3.7 kg at birth. hlotor and language development Iiacl been normal. She was now in grade 1 and doing average work. T h e father, age 32, is a liigli school teacher; the mother, age 30, is a housewife who had attended college for two years. Both are healthy, with no consanguinity. Two other siblings, a girl age nine years and a boy age four years, are healthy. At 3 years 4 months of age, this patient started to have daily twoto thrce-minute episodes of rocking movements of the body. She would go lo an obscure corner or another room, lie on lier abdomen, stiffen her body, rock back and forth, stare blankly, and then sweat profusely. She would then resume her normal play activities. or might seem drowsy and fall asleep. Two months later, she was hospitalized for investigation of this condition. Urinalyses, blood chemistries and counts, cerebrospinal fluid examination, skull x-ray, and brain scan were all within normal limits. An EEG recording !\.as read as “irregular spike activity in the

Epilepsy in the families of epileptics

Summary Interviews were obtained with members of the families of 669 epileptic subjects with 3,361 close relatives, and 470 control patients with 2,858 close relatives. A small but significant familial aggregation of idiopathic major motor epilepsy was demonstrated. This aggregation appeared to vary with the age of the proband at onset of epilepsy and was highest when the probands epilepsy started between the ages of 0 and 31/2 years and between 91/2 and 151/2 years. No familial aggregation could be demonstrated when the age at onset was over 151/2 years, or in other types of epilepsy. Simple febrile convulsions were not included in the study. Hereditary transmission of epilepsy could not be demonstrated, but it could not be ruled out. Familial aggregation of idiopathic major motor epilepsy may be due to familial aggregation of perinatal brain injury, to inheritance, or to a combination of these factors. Further studies are needed to clarify this. Risk tables are presented, showing the chances of relatives of patients with idiopathic major motor epilepsy of developing epilepsy. The highest risk was found to occur in the close relatives of probands who had developed major motor epilepsy before 31/2 years of age: 7.6 per cent of these relatives may be expected to develop major motor seizures by 191/2 years of age, and 9.4 per cent by 391/2 years of age. Comparable control risks were 1.4 and 2.3 per cent.

No proven relationship of carbamazepine therapy to blood dyscrasias.

To the Editor: We would like to comment on several aspects of the article ‘Carbamazepine: A double-blind cornparison with phenytoin,” by Troupin and co-workers (NEUROLOGY 2751 1-519, June 1977). First. the authors’ statement that, “Although rare, it [the possibility of serious hematologic reactions] has been adequately documented previously.” conveys the definite impression that carbamazepine was unequivocally responsible for the reported cases of hematopoietic disturbances that occurred during therapy with this drug. We are not convinced that such serious hematologic reactions have been adequately documented. In our opinion, the apprehension relative to ”carbainaLepine-induced” blood dyscrasias was initiated largely, if not entirely, by the six cases of aplastic anemia,’-’ five of which terminated reported during the 1960s. We revicwed the case reports of these six patients6 and found that no baseline studies were reported immediately prior to the i n s t i t u t i o n of car b am az e p i n e therapy , nor we re b 1 o od examinations performed frequently, or even regularly, throughout the course of administration of this drug. One can only conclude that aplastic anemia occurred in these six case5 “ i n association with” but not as a direct ”result of” the administration of carbamazepine. In Fellows’ ~ a t i e n t , ~ for example, the autopsy report revealed the cause of death to be hepatitis of disputed origin, and contributing causes of death cited by the author were b r o n c h o p ne it m o n i a , se v e re coronary at hero 5 c 1 e ro s i s , aneurysmal dilation of the right coronary artery, healed myocardial infarctions of the posterior wall and apex of the left ventricle. congestive heart failure. and diabetes mellitus. It is our opinion that a causative role of carbamazepine in the development of aplastic anemia was not conclusively proved in any of the six patients. Among others, Lorge,’ a pioneer in the investigation of carhamazepine as an anticonvulsant, is also unconvinced of such a cause-effect relationship, as attested to by his statement that “over the 12-year period in which Tegretol@ has been in use. nine fatalities have been reported to us in patients who had developed a blood dyscrasia concomitantly with taking Tegretol. Evidence of a causal relationship is nearly always circumstantial, and it is often not justified to assert more than a temporal relationship between treatment with a drug, and coincidental effects.” Second, Troupin and co-workers reported that “significantly fcwer patients had objective side effects while taking carhamazepine” than while taking phenytoin. We do not believe, however, that their comparison was an equitable one, because the mean phenytoin blood level for their study population (31 2 p g per milliliter) was in the range generally classified as “toxic,” whereas the mean carbamazepine blood level (9 .3 p g per milliliter) was in the range usually considered to be “therapeutic.” On this basis, one would certainly expect a higher incidence of side effects in the patients who received phenytoin. It should also be noted that the average phenytoin blood level during the last three months of their investigation was 25 p g per milliliter or higher h 36 of their 47 patients, and 35 p g per milliliter or more in 20 patients. In view of these values, it is indeed startling that so few of their patients experienced symptoms of phenytoin toxicity while none exhibited overall side effects of grades 1V (definite intoxication) or V (encephalopathy). We have not been so fortunate with those of our patients who presented phenytoin blood levels of such magnitude. Third, we were surprised by the authors’ phenytoin dose:blood level ratio; that, for instance, a mean oral dose of 6 .4 mg per kilogram yielded a mean serum concentration o f 3 1.2 pg per milliliter. This is considerably in excess of the generally accepted dose:blood level relationship, in which, 3.5 mg per kilogram of phenytoin results in a blood level of 10 p g per milliliter. Finally, our experience with the carbamazepine treatment of approximately 1.400 patients during the past I2 years has consistently demonstrated that carbamazepine is superior to phenytoin in controlling psychomotor (temporal lobe) seizures, whereas phenytoin is more effective than carbamazepine in controlling major motor (grand mal) convulsions. Our findings, therefore, are in disagreement with the reported results of Troupin and co-workers, that the two drugs are equivalent in controlling both seizure patterns.

OPAQUE AREAS ON CEREBRAL ROENTGENOGRAPHY SIMULATING INTRACRANIAL CALCIFICATIONS CAUSED BY DISEASE.

Fig. 1. This shows the two lesions with the upper lesion showing the three maggots with the breathing pores of the posterior segments unusually distinct. ond was anterior to the first, and just superior to the upper margin of the mandible. The history showed that the child had been placed in the grass to sleep 3 days prior to admission. The next day the two areas in question were swollen and appeared to be ordinary insect bites. The ulcerations were noted just a few hours before he was brought to our care. The photograph clearly demonstrates the obvious diagnosis (Fig. 1). Dr. Leo Saraf of the Department of Surgery was able to remove the maggots after a short pursuit. The first sinus tract near the ear was 10 mm. in diameter and broadened into a cavity 2 or 3 cm. in diameter and about 4~2 mm. in depth. The second sinus tract was about 8 ram. in diameter, 1 cm. in depth and 1~ cm. at the widest point. The necrotic tissue was debrided, the sinuses were irrigated and packed with iodoform gauze. The child made an uneventful recovery. The larvae were identified as members of the family Sarcophagidae but the subtype could not be determined.