[Dyke-Davidoff-Masson syndrome: a report of two cases]. / Syndrome de Dyke-Davidoff-Masson: a propos de deux observations.

Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis and mental retardation. We report the clinical and imaging features in two patients with epilepsy revealing a Dyke-Davidoff-Masson syndrome. Brain MRI showed unilateral loss of cerebral volume with hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells. Ipsilateral fronto-parietal polymicrogyria was present in one patient.

[Lateral gaze palsy and progressive scoliosis in 4 Tunisian families]. / Paralysie du regard latéral et scoliose progressive: à propos de 4 familles tunisiennes.

In 1975, Sharpe and Silversides described a neurological entity in a Chinese family. Clinical picture was characterized by paralysis of horizontal gaze, pendular nystagmus and progressive scoliosis. To date, 43 cases have been reported. The pathogenesis remains unclear. The Authors report four Tunisian families with 12 affected individuals. The age of patients ranges from 6 to 34 Years. All examined patients have complete lateral gaze palsy, pendular nystagmus and progressive scoliosis. Blood routine tests, cerebrospinal fluid (CSF), evoked potentials, electromyography (EMG), muscle biopsy, CT scan and cerebral MRI were normal. Autosomal recessive (AR) mode of inheritance is the most probable pattern.

[A new case report of spinal cord compression secondary to beta-thalassemia]. / Un nouveau cas de compression médullaire secondaire à une bêta-thalassémie.

Extramedullary hematopoiesis is an unusual cause of spinal cord compression. We report the case of a 39-year-old man who presented symptoms of progressive dorsal spinal cord compression. Hemogramm showed erythrocytosis with microcytosis. Hemoglobin electrophoresis revealed heterozygous beta-thalassemia. Diagnosis of extramedullary hematopoiesis was based on magnetic resonance imaging findings. Radiological and therapeutic options in this rare condition are briefly discussed.

Childhood diabetic neuropathy: a clinical and electrophysiological study.

Clinical diabetic neuropathy in childhood is rare, but electrophysiological involvement of the peripheral nerve is more frequent. We assessed clinically and electrophysiologically the peripheral nervous system of 69 children and adolescents suffering from diabetes mellitus (DM). The mean age of the patients was 12.8 years and the mean age at onset of DM was 6.8 years with a mean disease duration of 6.3 years. Seven patients (10%) had clinical neuropathy of which ankle jerk reflex abolition was the most frequent sign. Twenty patients (29%) had a neurophysiological neuropathy prevalently affecting the lower limbs. Peripheral neuropathy was correlated with patient age, older age at onset, duration of DM, height and poor glycaemic control.