Search PubMed⌕ Search

Biomedical subjects

M F Ben Dridi

Publications and source records attributed to M F Ben Dridi.

At least 19 recordsLinked to original sources
PubMed1994

[Neurologic Behçet disease].

BACKGROUND: Behçet's disease is very occasionally revealed by neurological abnormalities. This report describes such a case. CASE REPORT: A 13 year-old girl was admitted because of status epilepticus and fever. Clinical examination showed horizontal nystagmus, cerebellar syndrome, right hemiplegia, ophthalmoplegia and meningitis. Her CSF contained 80 lymphocytes/mm3, 0.75 milligram proteins and 0.5 milligram glucose. CT scan showed a hypodense area in the internal capsule. Two similar episodes occurred 4 and 6 months later, but all attempts at determining the etiology were negative. A diagnosis of neurological Behçet's disease was considered 10 months after the first episode when the patient developed oral ulcers followed by pseudofolliculitis of the scalp. The disease recurred several times despite treatment with prednisone, colchicine and antiaggregant drugs. CONCLUSION: Recurrent aseptic meningitis is a classic manifestation of neurological Behçet's disease. It usually appears 2 months to 27 years after the first extraneurological signs and not, as in this case, several months before.

Adolescent↗
PubMed1993

[Robinow's syndrome associated with deafness].

BACKGROUND: Robinow mesomelic dysplasia is an autosomal dominant or recessive condition that results is a flat facial profile, mesomelic shortening and frequent genital hypoplasia. This report describes a case with bilateral deafness. CASE REPORT: A boy was born at term to consanguineous parents who have no morphological abnormalities. The child's older brother and sister are normal. The patient was admitted at the age of 5 months because of abnormal features: enlarged skull with prominent forehead, flat facial profile, hypertelorism, small chin, shortening in the mesomelic segments of the limbs and genital hypoplasia without cryptorchidism. X-rays confirmed the mesomelic shortening and showed radial head dislocation and vertebral abnormalities. These abnormalities were more evident at the age of 2 1/2 years, when a bilateral sensorineural hearing loss was detected. CONCLUSION: This case of Robinow has all the characteristic findings plus deafness. The parental consanguinity suggests that its inheritance is autosomal recessive.

Abnormalities, Multiple↗
PubMed1993

[Stark-Kaeser type scapulo-peroneal amyotrophy of neurogenic origin. Study of a familial case].

BACKGROUND: There are several types of the rare syndrome, scapuloperoneal atrophy; the report deals with a familial case of the Stark-Kaeser type. CASE REPORT: A 16 year-old Tunisian boy was admitted for "steppage" gait. His mother had distal myoatrophy of four limbs and pes cavus. His sister, aged 8 years, also had pes cavus. She had no tendon reflexes and electromyography showed evidence of muscular denervation with normal nerve conduction velocity. The "steppage" gait in our patient began when he was 6 years old. When he was examined at 16 years, there were bilateral atrophy of the sternocleidomastoid muscle, and atrophy of the scapular girdle and the peroneal muscles with loss of tendon reflexes; there was no sensory involvement. Nerve conduction velocity was normal and electromyography showed fibrillation and pseudomyotonia potentials. Creatine kinase activities were moderately increased. Muscle biopsy showed significant neurogenic type changes. CONCLUSION: This patient has a Stark-Kaeser type spinal amyotrophy, a well-individualized entity due to progressive degeneration of the anterior horn cells.

Adolescent↗
PubMed1993

[Pulmonary sequestration in an infant].

A case of intralobar pulmonary sequestration in an infant is reported. The lesion presented as recurrent bronchopulmonary infections. The chest film showed a density in the right lower lobe, in which air-filled images and air-fluid levels developed rapidly. Aortography demonstrated systemic blood supply to the lesion from three arteries stemming from the thoracic aorta. The pathological study of the operative specimen confirmed the diagnosis. Postoperative outcome was satisfactory.

Aortography↗
PubMed1992

[Imerslund-Najman-Gräsbeck anemia. Apropos of a case].

Imerslund-Najman-Gräsbeck anemia is an infrequent disease with an autosomal recessive pattern of inheritance. The characteristic anomaly is selective malabsorption of vitamin B12 by the ileal mucosa. Diagnosis rests on a positive family history and on the demonstration of megaloblastic anemia with proteinuria. The proteinuria is due to glomerular dysfunction with mesangial proliferation. Management rests on lifelong parenteral administration of vitamin B12. A case of Imerslund anemia with a favorable outcome under vitamin B12 treatment is reported.

Anemia↗
PubMed1991

[Erythermalgia: a rare vascular acrosyndrome].

A case of erythermalgia with arterial hypertension that appeared in a 13 year-old boy is described. This condition led to a loss of weight of 10 kg within one month. None of the diseases known as a cause of this rare condition was found. Clinical manifestations were only improved when extremities were placed in cold water: treatment with pizotifene was also effective suggesting the role of serotonine in the mechanism of the crises.

Adolescent↗
PubMed1991

[Zinc].

Explore the source record for details and available documents.

Biological Availability↗
PubMed1990

[Nesidioblastosis. Apropos of 12 new cases].

Two new cases of diffuse hyperplasia of the pancreas are reported. This infrequent condition is caused by intermittent and variable insulin hypersecretion. The hyperinsulinism is responsible for severe, lasting and intractable hypoglycemia that causes seizures and mental retardation. Onset usually occurs in the neonatal period. The diagnosis of hyperinsulinism rests on four criteria: the presence of increased insulin levels in the face of hypoglycemia, the low urinary excretion of ketone bodies during hypoglycemic episodes, the need for more than 15/mg/kg/min glucose to maintain the serum glucose level above 2 mmol/l, and a positive response to glucagon. The topographic diagnosis is often disappointing. Medical treatment of the hypoglycemia with diazoxide is a transient measure. Subtotal pancreatectomy is indispensable. Postoperative results are variable. Insulin deficiency diabetes mellitus is common and unusual in that insulin induces an exaggerated response. Recovery can be observed. If hypoglycemia recurs, diazoxide is often effective.

Female↗
PubMed1989

[Early infantile form of Niemann-Pick disease type C. Apropos of 2 siblings].

We report two cases of Niemann-Pick disease in a sister and brother. Early jaundice was the first manifestation in both cases and was followed by cachexia and a rapidly fatal outcome. Neurologic involvement was obvious in both patients. Biologic phenotype was consistent with a diagnosis of type C sphingomyelinase, although clinical expression was different. These two cases should be classified within the infantile and early forms of Niemann-Pick disease type C. Antenatal diagnosis was performed during a third pregnancy. Enzyme activity assays on a specimen of trophoblast taken at the tenth week of gestation showed the fetus was not affected. This diagnosis was confirmed by a normal clinical evaluation at two months of life, and normal sphingomyelinase activity of cultured skin fibroblasts.

Age Factors↗