Biomedical subjects
F Andermann
Publications and source records attributed to F Andermann.
Occult encephaloceles and temporal lobe epilepsy: developmental and acquired lesions in the middle fossa.
Three patients with medically refractory partial complex seizures were shown to have small and initially unsuspected encephaloceles of the middle fossa. All had bitemporal, independent, interictal epileptogenic abnormalities, and the side of origin of the seizures could not be determined by surface EEG alone. The encephaloceles were missed on the initial radiologic studies and were found retrospectively in two patients; the third was found at operation and was not demonstrable radiologically, even in retrospect. One was the sequel of remote mastoid surgery, another was neoplasm-related, and the third, developmental. In all, bony defects of the middle cranial fossa were associated with temporal lobe herniation. The patients were treated surgically and have had no further partial complex seizures since operation.
The indications for and the role of depth electrode recording in epilepsy.
A series of 47 patients studied with stereotactically implanted depth electrodes is analyzed. Indications and grouping of the patients fall into three main categories: (a) the bitemporal series (35 patients) where there is ambiguity as to the lateralization of the focus; (b) the unilateral series (6 patients) where the problem is one of localization within one hemisphere, and (c) the generalized series where one tries to determine the primary focus in secondary generalized seizures (6 patients). The best indication appears to be the bitemporal cases, in which a large number of attacks can be recorded by computer. Most of them tend to have a well-lateralized onset in one of the temporal lobes; 32 of the 35 implanted patients were operated upon (91.4%). The results on the seizure tendency in these patients is comparable, if not superior, to those obtained in the so-called unilateral cases. Correlation studies indicate that interictal activity is a good indicator of the predominance of seizures.
Sexual ictal manifestations predominate in women with temporal lobe epilepsy: a finding suggesting sexual dimorphism in the human brain.
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Occipitotemporal epilepsy studied with stereotaxically implanted depth electrodes and successfully treated by temporal resection.
A young woman had seizures suggestive of temporal lobe origin with some clinical features indicative of occipital onset. Electroencephalograms recorded through scalp and sphenoidal electrodes showed predominantly right posterior temporooccipital epileptogenic discharges. Stereotaxic depth electrode recordings from the right temporal and occipital lobes demonstrated that some seizures arose independently from either the temporal or occipital lobes. Most arose within the occipital lobe, but, to become clinically evident, the seizure discharge had to spread to the temporal lobe. Following temporal lobe resection, including excision of limbic structures, the patient has remained seizure free for four years except for a few generalized seizures before and after delivery of her child.
Seizures induced by thinking.
A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arithmetic with a significantly high incidence. Tasks involving multiplication, division, and manipulation of spatial information were significantly associated with discharges, but few, if any, discharges appeared when addition and subtraction tasks of equivalent difficulty were performed. Tasks involving the retention of numerical information in short-term memory (e.g., immediate repetition of a series of 8 digits) were never associated with paroxysmal EEG activity. It is argued that generalized epilepsy of this kind may be related to focal dysfunction in a manner analogous to the involvement of the occipital lobe in cases of pattern-sensitive epilepsy.
The role of the limbic system in experiential phenomena of temporal lobe epilepsy.
Experiential phenomena occurring in spontaneous seizures or evoked by brain stimulation were reported by 18 of 29 patients with medically intractable temporal lobe epilepsy who were investigated with chronic, stereotaxically implanted intracerebral electrodes. The phenomena mainly consisted of perceptual (visual or auditory) hallucinations or illusions, memory flashbacks, illusions of familiarity, forced thinking, or emotions. Experiential phenomena did not occur unless a seizure discharge or electrical stimulation involved limbic structures. For such phenomena to occur, seizure discharge or electrical stimulation did not have to implicate temporal neocortex. This was true even for perceptual experiential phenomena. Many experiential responses elicited by electrical stimulation, particularly when applied to the amygdala, were not associated with electrical afterdischarge. Limbic activation by seizure discharge or electrical stimulation may add an affective dimension to perceptual and mnemonic data processed by the temporal neocortex, which may be required for endowing them with experiential immediacy.
Anoxic tonic seizures due to asthma; a serious complication in adults.
Anoxic tonic seizures are reported as a complication of severe asthma in two adults. The nature of these attacks can be misinterpreted. They do not indicate the presence of a recurrent seizure problem unconnected to the episodes of anoxia caused by asthma. These attacks do not respond to antiepileptic medication, but cease if the asthma can be controlled. One of our patients developed an anoxic encephalopathy and the other died. The need for prompt diagnosis and appropriate treatment is emphasized.
Dopaminergic mechanism in generalized photosensitive epilepsy.
Apomorphine, a dopamine receptor agonist, blocked epileptic photosensitivity in patients with primary corticoreticular epilepsy. This effect was not modified by naloxone, a narcotic antagonist, suggesting that apomorphine acts on cerebral dopaminergic receptors. Apomorphine did not block spontaneous spike-and-wave discharges in patients with nonphotosensitive primary corticoreticular epilepsy. The different actions of apomorphine on spontaneous and photically induced spike-and-wave activity suggest that there is a selective dopaminergic mechanism in human epileptic photosensitivity.
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.
An autosomal dominant syndrome of hemiplegic migraine, nystagmus, and tremor.
A mother and son suffer from hemiplegic migraine with onset in childhood. Both have nystagmus which has not changed for many years, but the date of onset is uncertain. They have an asymmetrical tremor, clinically indistinguishable from essential tremor. Neuroophthalmological examination revealed inability to produce smooth pursuit, gaze-paretic nystagmus, rebound nystagmus, failure of fixation suppression of the vestibuloocular reflex both horizontally and vertically, and low gain of the optokinetic system. These abnormalities, confirmed by electrooculography, are commonly seen in disease of the cerebellum and brainstem. Treatment with propranolol and pizotyline lessened the number of episodes of hemiplegia and improved the tremor. Hemiplegic migraine has been reported in association with nystagmus, retinal degeneration, deafness, and ataxia in varying combinations in three other families with autosomal dominant inheritance. These associated neurological manifestations likely represent system degenerations rather than the effect of repeated ischemia imputable to the migraine itself. The syndrome of hemiplegic migraine, tremor, and ocular smooth pursuit system disorder seen in this family appears to be inherited as a single autosomal dominant trait, although more than one autosomal dominant gene may be involved.
Friedreich's ataxia and oral glucose tolerance: II. The effect of ingested glucose on serum growth hormone in homozygotes, obligate heterozygotes and potential carriers of the Friedreich's ataxia gene.
Homozygotes, obligatory heterozygotes and potential carriers of the Friedreich's Ataxia gene were tested with an oral glucose tolerance in order to assess a. the prevalence of abnormalities in glucose handling, b. the secretory pattern of two "diabetogenic" hormones, growth hormones and prolactin and c. to evaluate the possibility for detection of an abnormal trend in these hormones to be used as a genetic marker. Despite the high prevalence of glucose abnormalities horizontally and vertically in these families, the basal output and responses of these metabolic hormones to a glucose challenge were appropriate and thus not characteristic of any of the above groups.
Startle disease or hyperekplexia: further delineation of the syndrome.
Startle disease is an autosomal dominant disorder with two phenotypic expressions. In the major form, there is hypertonia in infancy, and later an insecure gait. The patients have falling attacks without unconsciousness and in these, they are often injured or suffer concussions. Episodes of shaking of the limbs lasting for several minutes and resembling generalized clonus or repetitive myoclonus occur. These are most often nocturnal and are also unaccompanied by loss of consciousness. the patients are hyperreflexic and show an increased incidence of associated neurological and electroencephalographic abnormalities. The minor form of startle disease is only manifested by excessive startle and this is inconstant. In infancy it is brought out by febrile illness and in adult life by emotional stress. Gastaut and Villeneuve postulated the existence of a sporadic form of hyperekplexia different from the disorder described by Suhren et al. Review of their report and comparison with the cases of Suhren et al, and our own patients leads us to believe that the sporadic and familial forms of startle disease are the same. The disorder is rare, probably misdiagnosed initially as spastic quadriplegia, and later as epilepsy. Clonazepam appears to be the treatment of choice and its effect is sustained.
Marriage and fertility in epileptic patients.
Marital status and fertility were compared for 100 epileptic females and 100 epileptic males. The marriage rate among male patients was 59% of expected (p less than 0.001) as compared to 83% for female patients (p less than 0.10). Patients with early onset of seizures, particularly males, had lower marriage rates than expected, unlike patients with onset after age 20. Married female patients had only 69% of the expected number of live-born children (p less than 0.001) as compared to 100% for married male patients. Marriage and fertility rates of epileptic females have improved in the last generation, while those of epileptic males have remained constant.
Transient abolition of generalized photosensitive epileptic discharge in humans by apomorphine, a dopamine-receptor agonist.
Apomorphine, an agonist of dopamine receptors, blocks or significantly reduces photically induced seizures in the baboon (Papio papio). We therefore studied the effect of subcutaneously administered apomorphine in 11 patients with generalized photosensitive epilepsy. Visual evoked potentials were not altered by apomorphine, but in nine patients apomorphine transiently blocked the epileptic photosensitivity for an average of 45 minutes. Therefore, dopaminergic mechanisms play a role in the pathophysiology of human generalized photosensitive epilepsy.
Cerebromacular degeneration: progress and prospects in the search for the elusive biochemical defect.
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Pathological involvement of primary sensory neurons in Werdnig-Hoffmann disease.
Seven patients with the typical clinical picture and muscle biopsy findings of classical Werdnig-Hoffmann disease showed Wallerian degeneration in their biopsied sural nerves. In dorsal root ganglia of one patient there were residual nodules and several chromatolytic neurons. By electron microscopy the changes of chromatolysis were confirmed and found to be consistent with an axonal reaction. Involvement of the primary sensory neuron is probably a regular pathologic feature of Werdnig-Hoffmann disease. The type of abnormality suggests in initial failure of the axon distal to the nerve roots.
Basilar migraine, seizures, and severe epileptiform EEG abnormalities.
Four adolescents had basilar migraine, infrequent cerebral seizures, and severe EEG abnormalities. The clinical course was benign, with normal personality, mentation, and neurologic examination. Almost continuous EEG abnormalities, consisting of rhythmic temporo-occipital sharp and slow wave discharges, or generalized spike and wave complexes, were seen in serial recordings. The rare seizures, either focal or generalized, usually followed a migrainous aura and seemed to be readily controlled with anticonvulsant medication. The complex relationship between classical migraine and epilepsy is illustrated by this syndrome; recognition of its relatively benign course may prevent unnecessary investigation and undue alarm.