Reflex Seizures are Frequent in Patients with Down Syndrome and Epilepsy

Guerrini, Renzo; Genton, Pierre; Bureau, Michelle; Dravet, Charlotte; Roger, J

doi:10.1111/j.1528-1157.1990.tb05496.x

Cited by 76 publications

(37 citation statements)

References 38 publications

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“…9 The first almost always occurs in patients with mental retardation, or in those having severe brain damage in the perinatal or early infantile period with resulting hemiplegia. Otherwise, diseases leading to generalised maldevelopment of the brain, such as Down's syndrome, cause startle epilepsy 10. The induced seizures are almost always generalised tonic, although the tonic phase is at times so brief that only a sudden falling down can be noted.…”

Section: Discussionmentioning

confidence: 99%

Rub epilepsy: a somatosensory evoked reflex epilepsy induced by prolonged cutaneous stimulation

Kanemoto

Watanabe²,

Tsuji³

et al. 2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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To delineate rub epilepsy-a type of reflex epilepsy induced by prolonged or repetitive cutaneous stimulation in a circumscribed area of the body-three cases are presented, as well as one of tooth brushing epilepsy for comparison. In all three cases of rub epilepsy, cutaneous stimuli in a particular body area on the left side initially induced a sensory jacksonian march in the middle of, or in close vicinity to, the trigger zone, which led to subsequent unilateral tonic contractions with intact consciousness. By contrast, a motor jacksonian seizure without sensory aura was induced in the patient with tooth brushing epilepsy. A review of cases with rub epilepsy, including those in this paper, disclosed a striking consistency in clinical manifestations. The symptomatology of the induced seizures indicates a propagation of epileptic discharges from the postcentral gyrus to the supplementary motor area. Rub epilepsy is proposed as a separate clinical entity, clearly demarcated from other somatosensory evoked reflex epilepsies such as startle and tooth brushing epilepsy. (J Neurol Neurosurg Psychiatry 2001;70:541-543) Keywords: somatosensory evoked reflex epilepsy, startle epilepsy, tooth brushing epilepsy As long ago as 1863, John Hughlings Jackson described a case in which touching the thumb would bring on a fit.1 Later, in 1910, Woodcock and Edin described a boy who went into epileptic fits repeatedly when his sister removed the stocking from his right leg. However, until recently, somatosensory evoked epilepsy without an element of surprise has been confused with other types of somatosensory evoked epilepsy such as startle epilepsy. We have named the first type of somatosensory evoked epilepsy as "rub epilepsy", because a long or regular series of tactile contacts are usually the most eVective means of eliciting a seizure. Herein, three cases of rub epilepsy, treated at Kansai Regional Epilepsy Center, are presented, along with a case of tooth brushing epilepsy as a comparison. Written consent was obtained from each patient before the report. Case reports CASE 1A 35 year old woman began to have seizures at 14 years of age. The first attack came suddenly with a sensation of pressure in the left shoulder, immediately followed by secondary generalisation. Since that incident, paroxysmal dysaesthesia has occurred daily in that area. At the age of 17, the patient noticed that she could trigger seizures by repeatedly patting her left shoulder. After the stimulation, she would feel pressure in this region that would then extend down the left arm to the tip of her fingers. This was followed by either a tonic contraction of the left arm with a simultaneous leftward movement of the head, or by prolonged localised jerks of the fingers of the left hand. At the age of 35, she was transferred to a university hospital, because a generalised seizure had recurred after a 21 year interval. Occasionally, paroxysms of sudden atonia of the left limbs were also noted. She was diagnosed as having psychogenic seizures, because...

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Section: Discussionmentioning

confidence: 99%

Rub epilepsy: a somatosensory evoked reflex epilepsy induced by prolonged cutaneous stimulation

Kanemoto

Watanabe²,

Tsuji³

et al. 2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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“…8 The patients described mostly had severe epilepsy, often associated with moderate to severe fixed neurological handicaps, representing diffuse neurological damage. The purpose of this paper is to present the clinical and investigational findings of a further 19 patients. Only some of the current series were affected by fixed neurological deficit, others had no neurological signs and were of normal intelligence.…”

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confidence: 99%

Startle provoked epileptic seizures:features in 19 patients.

Manford

Fish

Shorvon

1996

Journal of Neurology, Neurosurgery & Psychiatry

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Objectives-To define the clinical characteristics of a group of patients with startle provoked epileptic seizures (SPES). Methods-Nineteen patients were identified during the course of a larger study of clinical seizure patterns. A witnessed seizure account was obtained in all patients; interictal EEG in 18, video-EEG-telemetry in eight, CT in 18, and high resolution MRI in eight. Results-The onset of SPES was in childhood or adolescence in 14 of 19 patients. It was preceded by exclusively spontaneous seizures in nine patients and SPES had been replaced by exclusively spontaneous seizures in two patients. Sudden noise was the main triggering stimulus and somatosensory and visual stimuli were also effective in some patients. The clinical seizure pattern involved asymmetric tonic posturing in 16 of 19 patients. Focal neurological signs were present in nine patients, mental retardation in six, and 10 were clinically normal. Ictal scalp EEG showed a clear seizure discharge in only one patient with a tonic seizure pattern; over the lateral frontal electrodes contralateral to the posturing limbs. Brain CT showed a porencephalic cyst in three patients, focal frontal atrophy in one, and generalised atrophy in one. Brain MRI was undertaken in five normal subjects and three neurologically impaired patients, six with normal CT. It showed a porencephalic cyst in one patient. In six patients, there were dysplastic lesions. They affected the lateral premotor cortex in three patients and the perisylvian cortex in three patients, one with bilateral perisylvian abnormality. Conclusions-SPES are more frequent than is generally appreciated. They may be transient and occur relatively commonly without fixed deficit, by contrast with previous reports. The imaging abnormalities identified in those without diffuse cerebral damage suggest that SPES are often due to occult congenital lesions and that the lateral premotor and perisylvian cortices are important in this phenomenon. (3 NeurolNeorosurg Psychiatry 1996;61:151-156)

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“…Startle-provoked seizures typically arise between the ages of 10 months and 14 years and are seldom diagnosed in adults 1–3. The presumed insults resulting in startle epilepsy (including hypoxic-ischaemic injury, central nervous system infection and traumatic brain injury) often occur in the perinatal or postnatal period 1–4. Although the exact cerebral structures responsible for the generation of startle-provoked seizures have not been fully elucidated, mesial structures, including the supplementary motor area (SMA), precuneus and primary motor/somatosensory areas, have been suggested by some 1.…”

Section: Introductionmentioning

confidence: 99%