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Biomedical subjects

J B Stephenson

Publications and source records attributed to J B Stephenson.

107 records · Page 6Linked to original sources

Reflex anoxic seizures ('white breath-holding'): nonepileptic vagal attacks.

From clinical history 58 children were diagnosed as having reflex anoxic seizures secondary to provoked cardioinhibition (also known as white breath-holding attacks). Before referral, these seizures were commonly misdiagnosed as epileptic either because the provocation was ignored, not recognised, or was a febrile illness, or because there was no crying, no obvious breath-holding, little cyanosis, and often no pallor to suggest syncope and cerebral ischaemia. The duration of cardiac asystole after ocular compression was measured in these children and in 60 additional children with other paroxysmal disorders. In 45 (78%) of the 58 with reflex anoxic seizures asystole was 2 seconds or over, and in 32 (55%) it was 4 seconds or greater, an abnormal response. Review of the literature supports the concept that these seizures result from vagal-mediated reflex cardiac arrest which can if necessary be prevented by atropine. The simple name 'vagal attack' is proposed. Ocular compression under EEG and ECG control supports the clinical diagnosis if asystole and/or an anoxic seizure is induced; the procedure described is safe and should be routine in seizure or syncope evaluation, when a meticulous history still leaves room for doubt.

Adolescent↗

Hereditary coproporphyria and epilepsy.

A 9-year-old boy with mental deterioration and epilepsy suffered an acute attack of hereditary coproporphyria associated with worsening of seizure control. Leucocyte coproporphyrinogen oxidase activity was undetectable in the patient during this attack, and was reduced in his mother, a latent case. The complex relationship between porphyria, epilepsy, and anticonvulsant drugs is discussed.

Aminolevulinic Acid↗

Epilepsy: a neurological complication of thalidomide embryopathy.

The possibility of epilepsy resulting from maternal thalidomide ingestion was investigated by obtaining a telephoned neurological history from all suspect families in the United Kingdom in which the specific diagnosis was uncertain. The annual incidence in the first seven years of life was found to be five times the figure for the general population, and the prevalence of active epilepsy is significantly increased in the teenage thalidomide population. That this increased incidence and prevalence of epilepsy is not a chance observation is supported by published clinical and experimental evidence of central nervous system abnormalities in thalidomide embryopathy, in addition to the known neurological effects of the drug in the adult.

Adolescent↗

Clumsy children.

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Cerebral Palsy↗

Familial dysequilibrium-diplegia with T-lymphocyte deficiency.

A second family is described with a combination of defective thymus-dependent immunity and cerebral palsy. The cerebral palsy comprised nonprogressive dysequilibrium and mild spastic diplegia without limb ataxia. This genetic entity of presumed autosomal recessive inheritance is clearly distinguished from ataxia-telangiectasia. Immunological abnormalities should be sought in other familial or unexplained cerebral palsy syndromes.

Agglutinins↗

Anoxic seizures: self-terminating syncopes.

This review focuses on anoxic seizures induced by self terminating syncopes in the young. Anoxic seizures are nonepileptic events consequent upon abrupt interruption of the energy supply to metabolically active cerebral neurones. Anoxic seizures are the most common paroxysmal events misdiagnosed as epilepsy. Neurally mediated syncopes have numerous appellations, especially in the young. This proliferation of terminology likely results from uncertainty regarding pathophysiology. The most important type of self-limiting syncope from the point of view of diagnostic difficulty has been called neurocardiogenic or vasovagal syncope and reflex anoxic seizure, amongst other names: this review includes a video clip of such a child with prolonged asystole. It also includes a detailed case history emphasising the feelings of a patient with this type of syncope who was misdiagnosed as having epilepsy for many years. The second class of self-terminating syncope discussed and illustrated on video is the so-called breath-holding spell of young children. The third example illustrated is the compulsive Valsalva manoeuvre of individuals with autistic spectrum disorder, in which anoxic seizures - as shown on the video clips - are easily misdiagnosed as epileptic seizures, with unfortunate consequences.

Child↗