Encefalopatía aguda conmioclonías por fenitoína. A propósito de un caso / Acute myoclonic encephalopathy due to phenytoin. A case report

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[Epileptic seizures as the first sign of fibrous bone dysplasia]. / Crisis epilépticas como primera manifestación de displasia ósea fibrosa.

INTRODUCTION: Fibrous bone dysplasia is an unusual disorder of the maturation of bone, seen as hyperostosis of the craniofacial bones and the diaphyses of long bones. Monostotic and polyostotic forms occur, depending on whether one or more bones are affected. The diagnosis is radiological (cranial CT or MR) or on morbid anatomy. The etiopathogenesis is not known. The association of epilepsy and monostotic fibrous bone dysplasia is rare. We present a case of monostotic fibrous bone dysplasia which presented with epileptic seizures. CLINICAL CASE: A 28 year-old-woman had had undiagnosed epilepsy for 12 years. Neurological examination was normal and EEG findings non-specific. Cranial CT and MR suggested fibrous bone dysplasia. Since there was bilateral reduction of the visual fields, due to compression of both optic nerves, the affected bones were removed surgically. Anatomopathological study confirmed the diagnosis of fibrous bone dysplasia. CONCLUSIONS: The association between epilepsy and monostotic fibrous bone dysplasia is unusual. Epilepsy may be an initial symptom in asymptomatic fibrous bone dysplasia. The mechanism for the production of epileptic seizures may not be related to compression phenomena or local ischemia, but be secondary to alteration in the mechanism of cAMP as the second messenger of the cerebral cortex. Patients with fibrous bone dysplasia should undergo neurological examination to rule out local compression with minimal clinical findings.

[Trigeminal neuralgia in children as the only manifestation of Chiari I malformation]. / Neuralgia trigeminal infantil como única manifestación de una malformación de Chiari tipo I.

OBJECTIVE: The appearance of trigeminal neuralgia in infancy is exceptional. Trigeminal neuralgia as the only manifestation of a Chiari malformation is also infrequent. We present the case of a patient with a Chiari type I malformation in which the only symptom was the presence of trigeminal neuralgia since infancy. We have found no similar cases in the literature. CLINICAL CASE: A 39 year old man with no significant clinical history complained of brief, recurrent episodes of pain in the right maxillary region, compatible with trigeminal neuralgia since the age of eight. The frequency, duration and intensity of these episodes had progressively increased. The only unusual finding on physical examination was a sluggish right corneal reflex. Cranial MR showed descent of the cerebellar tonsils to below the level of the posterior arch of the atlas, together with slight descent of the brainstem. These findings are characteristic of a Chiari type I malformation. The symptoms rapidly disappeared when treatment was started with carbamazepine. CONCLUSION: In exceptional cases, trigeminal neuralgia may present in infancy and be the only manifestation of a Chiari malformation.

[Early and late epileptic crisis following cerebral hemisphere ischemia]. / Crisis epilépticas precoces y tardías tras isquemia cerebral hemisférica.

INTRODUCTION: There is no agreement as to the frequency and characteristics of epileptic seizures (ES) associated with cerebrovascular disease (CVD). OBJECTIVE: To analyze the presence of early (Se) and late (SI) ES, factors related to these, prognosis and conversion to epilepsy. PATIENTS AND METHODS: We made a retrospective study of 386 patients with cerebral hemisphere ischemia (100 TIA and 286 infarcts). Follow-up was 29.69 +/- 13.92 months. Two groups were defined: a) Patients without ES, and b) patients with ES. Those occurring within 15 days of the episode of ischemia were considered to be Se and the others Sl. We studied the prognosis (modified Rankin scale on discharge from hospital) and mortality. RESULTS: Twenty three patients (6%) had crises (18 Se and 5 Sl). The commonest type was simple partial motor crises (13 or 56.5%) followed by generalized tonic-clonic crises (9 or 39.1%) which predominated in the Se group. There were 6 solitary crises (Se) and 17 (73.9%) recurrences (66.6% of the Se and 100% of the Sl) (p = 0.049). There was an association between the presence of a family history of epilepsy (p < 0.001) and increased risk factors for epilepsy and CVD, particularly previous ictus and cardiopathy respectively (p = 0.034). No patient with TIA or lacunar infarct had ES. Extensive infarcts showed and increased tendency to the associated with hemorrhagic transformation, involvement of the cortex (p < 0.001) and cardioembolic incidents (p = 0.025). They had a worse prognosis, led to more disability (p < 0.001) and earlier mortality (38.4%) (during the first three months) (p = 0.015). These factors were associated (p < 0.001). CONCLUSIONS: Postischemic cerebral seizures are frequent and have a high recurrence rate, especially in patients with extensive infarcts involving the cortex and these of embolic origin. The prognosis is worse when crises occur early.

Hypnic headache syndrome: a case with good response to indomethacin.

Hypnic headache syndrome is a rare benign disorder which occurs in the elderly. It presents as a holocephalic or, less frequently, hemicranial headache during night sleep, lasting between 20 and 180 min; it is not associated with autonomic signs and responds well to lithium carbonate. We report a new case which responded well to indomethacin, and suggest that indomethacin may be used as an alternative treatment in this rare headache syndrome of the elderly.

[Acute cervical ischemic myelopathy: anterior spinal artery syndrome]. / Mielopatía isquémica aguda cervical: síndrome de la arteria espinal anterior.

Acute ischemic myelopathy is an uncommon cause of spinal lesion. Its most common location is the thoracic-lumbar region in the territory of the anterior spinal artery. The involvement of the cervical segment is occasional and with certain etiologic features that differentiates it from the involvement of other spinal segments. We report here two patients with no vascular risk factors who had an infarction in the territory of the cervical anterior spinal artery after physical exertion in one of the patients. We suggest the compression of anterior radicular-medullary arteries or the cervical anterior spinal artery against the anterior bone wall of the spinal canal as possible pathogenic mechanism.

[The concept and treatment of drug-resistant intractable epilepsy]. / Epilepsia intratable o farmacorresistente. Concepto y tratamiento.

Epilepsy is considered as being untreatable when attacks present unsatisfactory control or else when brought under control only with doses of anti-epileptic drugs which produce severe secondary effects. There has to be provable certainty that the anti-epileptic drugs were used correctly. Untreatable epilepsy is an essential condition for surgical intervention; consequently rigorous therapeutic and diagnostic criteria are needed which may also improve control of the attacks. About 20% of epilepsies are untreatable. The basic mechanisms of untreatable epilepsy are not well known and may be untreatable either from onset or as a consequence in the progression of the epileptogenetic process. Various factors may be involved in a bad prognosis. Spontaneous improvement is rare from the therapeutic point of view and before considering surgery at least two anti-epileptic drugs should be tried monotherapeutically prior to their use in combination. It is well known that bitherapy brings about an important improvement in a few cases. Anti-epileptic drugs should be used at their maximum tolerable dosage independently of theoretical plasmatic levels.

Electro-oculogram in multiple system and late onset cerebellar atrophies.

The present investigation uses electrooculogram to evaluate multiple system atrophy (MSA) and late onset cerebellar atrophies (LOCAs), both idiopathic (ILOCA) and late onset autosomal dominant cerebellar ataxia (ADCA). Forty cases were clinically examined using scales for cerebellar, pyramidal, parkinsonian, mental status and neuroimaging quantitative evaluations. The patients were classified into three groups: olivopontocerebellar atrophy (OPCA), striatonigral degeneration (SND), Shy-Drager syndrome (SDS), and LOCA. We have used direct current electro-oculography in order to establish their validity in making the diagnosis. Cerebellar signs were significantly correlated with impaired VOR-fix gain and OKN, abnormalities of saccades, and reduced smooth pursuit gain (p < 0.05). Pons atrophy was significantly correlated with impaired VOR-fix gain (p < 0.01), abnormalities of saccades (p < 0.01), and reduced smooth pursuit gain (p < 0.05). Cerebellar hemisphere atrophy was significantly correlated only with impaired VOR-fix gain (p < 0.05), and medulla oblongata atrophy only with abnormalities of saccades (p < 0.05). Gaze-evoked nystagmus was found in 42.8% of patients with OPCA, and only in 14.2% with SND, but was not found in LOCA patients (t test, p < 0.05). In patients with OPCA, the combination of gaze-evoked nystagmus, abnormalities of sinusoidal VOR and reduced OKN gain measurements was very frequent, while infrequent in both LOCA (Fisher's exact test, p < 0.05) and SND subjects (p < 0.01). SDS also showed abnormalities of the oculomotor system.

[Usefulness of plasmapheresis before thymectomy in the management of myasthenia gravis]. / Utilidad de la plasmaféresis pretimectomía en el tratamiento de la miastenia grave.

Plasmapheresis (PMP) is effective in some clinical situations of myasthenia gravis (MG). To evaluate its usefulness before thymectomy in patients with moderate generalized MG with no respiratory involvement, we studied 24 patients with MG divided into 2 groups of 12. PMP was performed before thymectomy in one group. The course of disease and mean doses of drugs required were recorded over a follow-up period of 12 months. There were no significant differences between the 2 groups in clinical condition or mean dose of anticholinesterase agents before or after thymectomy. Significantly higher doses of corticosteroids were used after the third month of follow-up in the group treated with PMP. We conclude that PMP confers no benefit when applied before thymectomy in patients without severe MG.