Risk of seizure recurrence after a first unprovoked seizure: a prospective study among Jordanian children.

PURPOSE: There is wide variation in the reported recurrence rate after a first unprovoked seizure in children. We investigated the risk of recurrence after a first unprovoked seizure in Jordanian children and the risk factors associated with increased recurrence rate. METHODS: All consecutive patients aged 3 months-14 years who presented with their first unprovoked seizures between January 1997 and 2000, were included in a prospective study and followed up for 3 years for possible recurrence. Of the patients studied, there was slight male predominance (56.6%) and 55% of them were 2-9 years of age. Generalised seizures were reported in 75% and the remaining 25% had partial seizures. The duration of seizure was 1-4 minutes in 59%. Family history of epilepsy was positive in 31% and parental consanguinity in 32%. The role of these factors in increasing the risk of recurrence was also investigated. RESULTS: Two hundred sixty-five patients were included in the study and continued follow up for 3 years. Ninety-eight (37%) of them experienced seizure recurrence. Among the predictor factors for recurrence, partial seizure (P = 0.003) and positive family history (P = 0.000) were associated with a statistically significant increased risk. Sex, age, duration of seizure and consanguinity were not associated with increased risk of recurrence. CONCLUSION: Thirty-seven percent of the children studied experienced a second attack after a first unprovoked seizure over the 3 years follows up period. The risk of recurrence was significantly higher in children with a partial seizure (55%) and among those with a positive family history of epilepsy (59%). Age at first seizure, sex, duration of seizure and consanguinity were not significantly related to the risk of recurrence.

A novel form of distal hereditary motor neuronopathy maps to chromosome 9p21.1-p12.

Distal hereditary motor neuronopathies (dHMNs) form a heterogeneous group of rare disorders characterized by distal weakness and wasting in the limbs with no significant sensory involvement. Harding has classified dHMNs into seven categories based on clinical and genetic criteria. We report a novel form of autosomal recessive dHMN in 7 consanguineous families located in the Jerash region of Jordan. Onset of the disease is between 6 and 10 years of age and is characterized by weakness and atrophy of the lower limbs associated with pyramidal features. Within 2 years, symptoms progress to the upper limbs. Neurophysiological studies typically show normal conduction velocities, reduced compound motor action potential amplitudes, normal sensory nerve action potentials, and chronic neurogenic changes on needle electromyography. No significant abnormalities are seen on sural nerve biopsy. We call this novel form of dHMN Jerash hereditary motor neuronopathy. We studied the families at the molecular genetic level and mapped the Jerash hereditary motor neuronopathy gene to an approximately 0.54-cM region on chromosome 9p21.1-p12, flanked by microsatellite polymorphic marker loci D9S1845 and D9S1791. A maximum LOD score of 19.80 at theta = 0.001 was obtained between the disease and locus D9S1878.