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Publications (4)

  • [Show abstract] [Hide abstract] ABSTRACT: Chorea paralytica (or chorea mollis) is a very rare variant of Sydenham's chorea, characterized by a profound hypotonia, resulting in severe disability. Given the rarity of this condition, data on its prognosis are lacking. Most reports suggest that the delay from onset to recover total autonomy is long, usually several weeks to months which strongly affects the quality of life of these children. We report a videotape case of a 14-year-old girl, who became rapidly bedridden because of severe generalized chorea paralytica. Her clinical picture was totally improved 7 days only after initiation of an "aggressive" treatment, combining steroid pulse, haloperidol and long-term penicillin G, with no relapse after 4-year follow-up. We believe that the best care of this rare and severe form of Sydenham's chorea, should combine pathophysiological treatment with corticosteroids, preferably by pulse-therapy, symptomatic antichoreic treatment by neuroleptics, associated with a long-term antibiotic use to reduce recurrence risk.
    Article · Jun 2013
  • Fettouma Moutaouakil · Hicham El Otmani · Hicham Fadel · [...] · Ilham Slassi
    [Show abstract] [Hide abstract] ABSTRACT: Benign myoclonic epilepsy of infancy is a rare idiopathic generalized epileptic syndrome occurring below the age of 3 years. Although benign outcome is presumed, some recent studies suggest less favorable outcome. A 14-year-old boy had a history of repeated episodes of myoclonic jerks of the shoulders and upper limbs in infancy (age 5 months). An ictal electroencephalogram indicated generalized spike-wave discharges associated with the myoclonic seizures, and the diagnosis of benign myoclonic epilepsy of infancy was made. Valproate treatment resulted in control of the myoclonic seizures, and the drug was withdrawn when the patient was 5 years of age. At the age of 10, he presented with episodes of eyelid jerks associated with brief lapses in concentration triggered by sunlight. Electroencephalography revealed photosensitivity and a pattern of eye-closure sensitivity. These features were compatible with the diagnosis of eyelid myoclonia with absences, or Jeavons syndrome. Lamotrigine eliminated the seizures. The evolution of benign myoclonic epilepsy of infancy to eyelid myoclonia with absences has been reported in one other case. A possible continuum of myoclonic epileptic syndromes, mediated by a common genetic abnormality, suggests the need for longer monitoring of patients with benign myoclonic epilepsy of infancy.
    Article · Sep 2010 · Pediatric Neurology
  • [Show abstract] [Hide abstract] ABSTRACT: Neurological manifestations in Gougerot-Sjogren syndrome (GSS) are valued differently. This is essentially the achievement of the peripheral nervous system. We report 9 cases of neurological manifestation revealing primitive Gougerot-Sjogren syndrome collected over a period of 8 years (1997-2004). GSS diagnosis was retained according to Americano-European group criteria consensus revised on 2002. All our patients were female with an average age of 43 years. Peripheral nervous system manifestation occurred in 78% (Truncal Neuropathy in 44%, anterior horn involvement in 2 cases). Central nervous system involvement was observed in 55.6% (chronic myelopathy and aseptic meningoencephalitis). The analysis of neurological manifestations in GSS encounters three difficulties: the lack in homogeneity of diagnostic criteria (which makes it difficult to compare the frequency of neurological complications in different series), the limited number of large series, and the cases with neurological manifestations revealing this syndrome.
    Article · Mar 2009 · Joint, bone, spine: revue du rhumatisme
  • [Show abstract] [Hide abstract] ABSTRACT: Introduction Les manifestations neurologiques du syndrome de Gougerot-Sjögren (SGS) sont diversement appréciées. Il s’agit essentiellement de l’atteinte du système nerveux périphérique. Méthodes Nous rapportons neuf cas d’atteintes neurologiques révélatrices d’un SGS primitif (SGSP) colligés sur une période de huit ans (1997–2004). Le diagnostic du SGSP a été retenu selon les critères élaborés par le groupe de consensus américano-européen révisé en 2002.Tout nos patients étaient de sexe féminin, avec une moyenne d’âge de 43 ans. L’atteinte neurologique était révélatrice dans tous les cas, il s’agissait d’une atteinte périphérique dans sept cas, isolée dans cinq cas et associée à une atteinte centrale dans deux cas. L’atteinte centrale a été observée dans cinq cas sous forme de myélopathie chronique ou de méningo-encéphalite aseptique. Discussion et conclusions L’étude des manifestations neurologiques du SGS se heurte à trois difficultés : l’absence d’homogénéité des critères diagnostiques utilisés ce qui rend difficile la comparaison des fréquences des complications neurologiques dans les différentes séries, le nombre limité des grandes séries et le fait que l’atteinte neurologique peut être révélatrice posant alors un problème de relation de cause à effet.
    Article · Mar 2009 · Revue du Rhumatisme