Reflex seizures induced by calculation, card or board games, and spatial tasks: a review of 25 patients and delineation of the epileptic syndrome.

Nine patients had reflex activation of seizures by calculation, card and board games, or spatial tasks. The common denominator for these and the 16 others reported in the literature appears to be activity related to function of the parietal lobe. The clinical and EEG findings in all 25 patients support the diagnosis of primary generalized epilepsy. Seizures usually start during adolescence and consist of myoclonus, absences, and generalized convulsions. Specific inquiry about reflex activation should be carried out in patients with generalized epilepsy since this is rarely provided spontaneously. Attacks could be controlled satisfactorily in 89% of our patients. The genetic features are those of a primary generalized epileptic disorder without evidence for a specific inheritance of reflex sensitivity. Neuropsychological analysis of the stimuli points to parietal cortical dysfunction. These stimuli lead to activation of a generalized epileptic process analogous to the occipital cortical participation in the activation of generalized epileptic abnormality occurring in patients with photosensitive epilepsy.

Facial asymmetry in patients with temporal lobe epilepsy. A clinical sign useful in the lateralization of temporal epileptogenic foci.

While a clinical history is frequently sufficient for a localizing diagnosis of temporal lobe epilepsy, lateralization of the epileptogenic abnormality often is impossible on clinical grounds alone. Since we have noted facial asymmetry in such patients, 50 individuals with temporal lobe epilepsy were studied. In patients with unilateral foci, we found contralateral lower facial weakness of mild to severe degree in 73 percent while 13 percent had ipsilateral weakness, and in 13 percent the face was symmetrical. The facial weakness usually was more striking on emotional movement. In the group of patients with bitemporal independent discharges, 61 percent had some asymmetry, often facial weakness on the side opposite the major focus. Of 25 control individuals, only one third had a facial asymmetry and this was slight. Facial asymmetry in temporal lobe epilepsy is a useful though not an absolute clinical lateralizing sign. It is a factor to be considered in addition to other clinical, radiologic, neuropsychologic, and electroencephalographic findings in the evaluation of patients with temporal lobe seizures.

Water-drinking as ictal behavior in complex partial seizures.

The urge to demand, pour, and drink water at the time of an attack was encountered in 20 patients who had seizures with complex partial symptomatology. Two patients were studied with bitemporal stereotaxically implanted depth electrodes. Drinking was associated with electrographic and clinical seizures starting in the amygdala, hippocampus, and parahippocampal gyrus. Sometimes, this was the only clinical manifestation of an attack, and its significance would not have been recognized without depth recording. Ictal drinking was never encountered in patients without electroencephalographic evidence of temporal epileptic abnormality, and therefore seems to have localizing significance.

Genetic and family studies in Friedreich's ataxia.

This study consists of two parts: 1. A detailed genetic analysis of 35 sibships in which 58 individuals were affected with Friedreich's ataxia; and 2. Clinical and laboratory examinations of parents and siblings, in an attempt at carrier detection and diagnosis of the pre-clinical state. The increased parental consanguinity, the lack of affected individuals in other generations, and the lack of significance of extrinsic etiological variables, all suggested an autosomal recessive mode of inheritance, and this was confirmed by formal genetic analyses, employing several different methods. Associated abnormalities in our series of 58 patients included cardiomyopathy (51.7%), diabetes mellitus (19.0%), optic atrophy (5.2%), nerve deafness (5.2%) and congenital malformations (6.9%). The incidence of diabetes mellitus, congenital malformations, and epilepsy and/or febrile convulsions was elevated in first degree relatives of patients with Friedreich's ataxia.

[Chronic pain and syringomyelic slit of the posterior horns of the spinal cord]. / Douleur chronique et fente syringomyélique des cornes postérieures de la moelle épinière.

Two male patients 46 and 44 y.o. respectively, were admitted for a syndrome of chronic pain characterized by: sudden onset, spontaneous or following spasmodic coughing, of an anterior hemithoracic pain slowly progressing to involve several unilateral cervicothoracic dermatomes, a continuous burning sensation made worse by light touch, limb movements and cold water, and partially relieved by warm water or deep palpation. On examination, patient 1 revealed no sensorimotor deficit after repeated observations during 8 years. At autopsy, a syrinx localized at the cord segments corresponding to the symptoms was found without documentation of specific causal factors. It involved the posterior horn of the cord selectively. In patient 2, pain was associated with slight hypesthesia to pinprick and heat from C2 to T5 on the left without motor deficit since 18 months. A high resolution C.A.T. scan showed an intramedullary cavity 0.3 cm from the midline in the projection of the posterior horn without anomalies at the cervicomedullary junction. These observations link chronic pain syndromes with predominantly posterior horn lesions, which so far have failed to respond to conventional therapeutic measures.

Postictal psychosis after partial complex seizures: a multiple case study.

Bouts of unusually frequent partial complex seizures originating in the temporal lobe, that sometimes became secondarily generalized, induced psychotic episodes in 9 patients. In 7, the increase in seizures occurred at a time when antiepileptic drugs (AEDs) were being reduced during intensive EEG monitoring with a view to surgical treatment of intractable epilepsy. According to DSM-III-R criteria, the postictal psychosis resembled an organic delusional syndrome which was paranoid in 7 and schizophreniform in 1 and an organic mood syndrome in 1. A high incidence of ictal fear, of bilateral independent epileptogenic discharge, and of small foreign tissue lesion were unexpected findings and appeared to represent risk factors, especially in patients otherwise handicapped by an epileptic personality disorder. Recognition of postictal psychosis in this setting and in others is important both prognostically and therapeutically. Postictal psychosis does not constitute a contraindication to surgical treatment of epilepsy.

On the relation of dystonic movements to serum thyroxine levels.

A patient with psychomotor retardation secondary to delayed treatment of cretinism developed abnormal dystonic movements in the absence of other signs of toxicity during levothyroxine replacement therapy at a serum thyroxine level of 16 mug./100 ml. The dystonic movements disappeared when the serum thyroxine level fell. The abnormal movements were considered to be related to high thyroxine levels in this patient with pre-existing central nervous system dysfunction.

Timing of seizure recurrence in adult epileptic patients: a statistical analysis.

Seizure diaries were maintained prospectively in 24 epileptic patients (19 with partial complex, three with partial simple, and three with primary generalized seizures) who were selected consecutively, had stable seizure patterns, were reliable historians, and were known to be compliant with medications. Diaries were maintained for an average of 237 days (range, 61-365), and an average of 18 seizures were recorded per patient (range, 5-76). Seizure patterns were analyzed by using the methods appropriate for a time series of events (point process). Two patients had a decreasing trend in seizure frequency. For 12 patients, seizure occurrence was indistinguishable from that of a Poisson process. The remaining 10 patients had an exponential distribution of seizure intervals, but did not fit other criteria for a Poisson process; 3 of these showed evidence for seizure clustering; none showed evidence for a seizure cycle. It is concluded that the pattern of seizure occurrence in most epileptic people is random, but in approximately 50%, it is not occurring according to a Poisson process. These observations indicate that seizure cycling and/or clustering are not common in epileptic patients, but do not exclude the possibility that seizures have been precipitated by some randomly occurring event, such as sleep deprivation or increased stress.

Intractable partial epilepsy following low-dose scalp irradiation in infancy.

We report the development of intractable epilepsy in 3 patients treated with irradiation to "strawberry" scalp nevi in infancy. Low-dose radiation was used (12 and 13 Gy in 2 of the patients). The clinical evolution suggested a recognizable and distinctive postradiation syndrome. There was concordance between the site of radiation as shown by localized alopecia, the clinical features of the partial seizures, and electrographic abnormalities. The clinical picture was unlike delayed cerebral radiation necrosis of adulthood, which is not thought to occur at doses below 50 Gy, in 2-Gy fractions. Neurological deficits were not progressive and in 2 patients there was no evidence of parenchymal injury on cranial magnetic resonance imaging scanning. These differences suggest pathogenetic differences to cerebral radiation injury of adulthood, probably relating to the interaction between nervous system development, individual susceptibility, and the low doses of radiation employed.