[Canavan disease or n-acetyl aspartic aciduria [one case]]

Canavan's disease [CD] or N-Acetylaspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N- acetylaspartic acid in the brain. The authors report a case in a ten-month old boy who presented with developmental delay and megalencephaly noticeable afterfour months of age. Magnetic resonance imaging of the brain show diffuse white matter degeneration. The diagnosis of CD was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry

[Glucose transporteur type 1 deficiency: a mild form]

Glucose transporter type 1 deficiency is a rare but curable disease whose real frequency is probably under estimated. This defect is responsible df reduced glucose brain supply across the blood-brain barrier. We report a mild form of GLUT1 deficiency suspected in al 9 months old boy when low cerebrospinal fluid rate glucose had persisted several months after recovery from presumed viral meningitis. GLUT 1 deficiency should be evoked in any child with delayed milestones development or seizures of unclear cause. The sole effective treatment is based on ketogenic diet

[Listeria monocytogenes meningitis in immunocompetent children. [a case report]]

Introduction: Listeria monocytogenes is, increasingly, recognised as a cause of life-threatening disease mainly in immunocompromised persons, but it is an uncommon cause of bacterial meningitis beyond the newborn period in healthy subjects

Report: We present the case of a 10-year-old previously healthy child who was admitted at a general pediatric unit for high fever, impaired consciousness and intra cranial hypertension syndrome. Cerebrospinal fluid examination revealed 980 cells/mm3 in mixed formula [neutrophils = 50 per cent, lymphocytes = 50 per cent], decreased glycorrachia [1.5 mmol/l], increased albuminorrachia [2g/l] and normal chlorurachia. Gram's stain was negative. initial parenteral antibiotics combining cefotaxime and vancomycin didn't lead to clinical improvement. In fact, after 48 hours, the temperature was usually high and the patient remained obnubilated and, also, developed bilateral convergent strabismus. The second cerebrospinal fluid sample showed lymphocytic meningitis [125 cells/mm3, neutrophils = 5 per cent, lymphocytes = 95 per cent], decreased glycorrachia [0.5 mmol/l], increased albuminorrachia [1.2 g/l], normal chlorurachia and negative soluble antigens. Gram's stain showed Gram positive bacilli. Subsequent culture yielded Listeria monocytogenes. The patient was so treated with parenteral ampicillin for fifteen days and showed uneventful recovery. No immunocompromised conditions were identified in this patient

Discussion: In its most severe form, listeriosis is an invasive disease that affects not only immunocompromised but also immunocompetent persons in whom the neuromeningeal form is the most frequent

Conclusion: L. monocytogenes should be considered among the aetiologies of bacterial meningitis in children who do not respond initially to conventional antimicrobial treatment or who deteriorate rapidly even if they are immunocompetent and non-neonates