[Neonatal myasthenia gravis]. / Myasthénie et grossesse: l'atteinte du nouveau-né peut être révélatrice.

BACKGROUND: Myasthenia gravis, an autoimmune disease of young women, is due to the dysfunction of neuromuscular transmission. The newborn of a myasthenic mother inconstantly presents a transitory neonatal myasthenic syndrome. Maternal aggravation, or even myasthenic crisis with respiratory failure, can occur in the first three months post-partum. CASE REPORT: Mrs. S., para two without appreciable medical history, delivered normally a boy weighing 4 kg with an Apgar score of 10/10. At 3 h of life the newborn was admitted to the neonatal care unit for grunting and axial hypotonia. Diagnoses of maternal-fetal infection and fetal distress were excluded. The dissociated pattern of neurological disorders (refusal to drink, axial hypotonia, hypomimia, but good contact and normal alertness) led to search for neuromuscular causes or poison. Myasthenia gravis was then considered and confirmed by maternal electromyography, allowing the diagnosis of transient neonatal myasthenia gravis and early diagnosis and treatment of the maternal myasthenic crisis in a specialized care unit. The outcome of both mother and child was favorable under treatment. CONCLUSION: Lack of maternal myasthenia gravis history should not result in excluding the diagnosis of transitory neonatal myasthenia gravis when evocative neonatal neurological signs are present. The symptomatology in the newborn may indeed reveal maternal myasthenia gravis, allowing an early diagnosis in both the mother and the newborn.

[Pneumococcal meningitis revealing dysplasia of the bony labyrinth in an infant]. / Méningite à pneumocoque révélatrice d'une dysplasie du labyrinthe osseux chez un nourrisson.

BACKGROUND: Dysplasias of the bony labyrinth are frequently associated with cerebrospinal fluid fistula and are usually discovered because of recurrent meningitis. CASE REPORT: A 1 year-old infant was admitted for a pneumococcal meningitis which appeared 2 days after the occurrence of a clear otorrhea from the right ear. The same organism was isolated from the otorrhea fluid, which also contained cerebrospinal fluid as confirmed cytochemically. The meningitis rapidly resolved with antibiotic treatment. Auditory brain stem responses were abolished from the right ear. CT of the temporal bones showed a pseudo-Mondini type labyrinth dysplasia at the right ear and Mondini type dysplasia at the left one. A translabyrinthine cerebrospinal fluid fistula was discovered by surgical exploration of the right ear, occurring through a perforation in the stapedial foot plate. The leak was cured by packing the vestibule and obturating both oval and round windows. Three years after the operation, the child did not experience any further episode of otorrhea or meningitis. CONCLUSIONS: Features suggesting a translabyrinthine fistula, especially otorrhea and deafness, should be systematically searched in any child with bacterial meningitis. Closure of these fistulas can prevent severe infectious recurrences.

[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent]. / Déficit homozygote en C8 révélé par des infections récidivantes à Neisseria meningitidis chez un adolescent.

BACKGROUND: Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adolescence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. CASE REPORT: A 14 year-old gypsy boy was admitted because of a febrile meningococcal meningitis that was complicated by a rapidly extensive and necrotic purpura, obnubilation and clotting abnormalities without hemodynamic anomalies. The patient was given symptomatic therapy and a 12-day course of antibiotics that resulted in rapid and complete recovery. Medical history of this patient showed that he had been admitted at the age of 3 years for a severe febrile purpura with septic shock and clotting abnormalities followed by rapid and complete recovery after symptomatic and antibiotic therapy. No germ had been then isolated. The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examination of the terminal pathway-revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin prophylaxis. He remained healthy during the ensuing 4 years. CONCLUSIONS: Meningococcal infections associated with late complement component deficiency are generally uncomplicated but they remain potentially severe. Early screening for this late complement component deficiency should be considered after severe clinical manifestations.

[Hyperglycemia revealing neonatal infection]. / Hyperglycémie révélatrice d'une infection néonatale.

BACKGROUND: Hyperglycemia as the first manifestation of neonatal sepsis is rare. CASE REPORT: A breast-fed neonate was admitted at the age of 6 days because of vomiting. Group B streptococci had been isolated in secretions of the ear at birth but the neonate had not been treated. At admission, physical examination was normal but glucosuria, without ketonuria, and hyperglycemia (9.7 mmol/l) were noted. Because hyperglycemia was not explained by usual causes, the baby was systemically given antibiotics. The next day, blood, spinal fluid and urine cultures taken on admission were positive for group B streptococci while blood fibrinogen and C-reactive protein were increased. Hyperglycemia and glycosuria were normal after 24 hours of antibiotic therapy and follow-up was uneventful. Subsequently, the same bacteria was isolated from the mother's milk. CONCLUSION: Isolated hyperglycemia may reveal an infection; therefore its discovery might contribute to early diagnosis and treatment.