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Thursday, February 20, 2014

Microcephaly its causes and newest line of treatment

Definition
•            It is defined as a small brain resulting in a head circumference below the mean for age by more than three standard deviations.
Etiology
Microcephaly results from failure of brain growth. It may be divided into two main groups:
1- Primary microcephaly: familial, or with chromosomal aberration syndromes (e.g. Down syndrome).
2- Secondary microcephaly (non genetic): this group results from a noxious insult affecting the fetus in utero, at birth of the infant, e.g. asphyxia or during the first 2 years of life which is the period of rapid brain growth e.g. radiation, congenital infections (toxoplasmosis, rubella, cytomegalo virus), drugs (hydantoin), meningitis and encephalitis.
Clinical picture
•          There is receding forehead and the ears are prominent.
•          The fontanel closes earlier than expected.
•          Manifestations of the etiological condition may be apparent.
•          Mental retardation is present in all cases of microcephaly.
•          Seizures and spasticity may be associated.
Diagnosis
•          Serial measurement of head circumference is important to demonstrate the limited or even arrested growth of the head.
•          Skull x-ray .
•          chromosomal analysis.
•          CT scanning, MRI.
Differential diagnosis
•          It should be differentiated from craniostenosis.
 Management
•           advice to parents about recurrence risk in future pregnancies General management.



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