Benign communicating hydrocephalus in children.
ABSTRACT A review was performed of the computed tomograms (CTs) of 500 children which had been reported as showing widening of the supratentorial subarachnoid spaces with normal cerebral substance. On the basis of this a radiological diagnosis of cerebral atrophy had been made in all but five, who were said to have megalencephaly. From these, the children with large or abnormally enlarging heads, but normal or only slightly enlarged ventricles, were selected; there were 40 such cases (8%). The clinical condition either improved or remained stable over a period of 2 years; in the majority the scan abnormality regressed (22.5%) or remained static (67.5%). In three cases there was slight progression of the CT changes before stabilisation, but only one case developed classical communicating hydrocephalus necessitating a shunt procedure. This condition is a generally benign and mild form of communicating hydrocephalus, for which an aetiological factor was apparent in about two-thirds of the cases studied.
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ABSTRACT: The clinically important neurologic complications in 76 patients with OI seen at the NIH included brainstem compression from basilar in-vagination, skull fracture, and seizure disorders. Neuro imaging studies demonstrated sulcal prominence and ventriculomegaly consistent with communicating hydrocephalus in 17 patients. Basilar invagination was found in 8 individuals, all with clinically severe OI, and caused brain-stem compression in 3 patients. Head circumference growth showed abnormal kinetics with percentile crossing after fontanelle closure in 13 patients and absolute macrocephaly was present in 11 patients. Neurologic evaluation should be part of a team approach in the management of patients with severe OI types. Continued study of the underlying pathophysiology of neurologic features in OI is warranted.07/2009; 31(s4):s23-s26.
Article: Glutaric aciduria type I[Show abstract] [Hide abstract]
ABSTRACT: Serial CT findings in an infant with glutaric aciduria type I (GA-I) are reported. The major CT features were dilatation of the insular cisterns, regression of the temporal lobes, with bat wings dilatation of the Sylvian fissures and hypodensity of the lenticular nuclei. CT changes preceded the onset of symptoms by 3 months. An improvement in the temporal lobe atrophy was seen after a period of treatment, coinciding with marked clinical improvement. A peculiar feature was the presence of external hydrocephalus, which diverted the attention from manifestations of the primary disease and thus consitituted a diagnostic pitfall. The delineation and recognition of the characteristic radiologic manifestations of GA-I are essential for allowing an adequate radiologist/clinican interaction in diagnosing this inborn error of metabolism.Neuroradiology 12/1990; 33(1):75-78. · 2.70 Impact Factor
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ABSTRACT: The computed tomographic scans of the heads of 67 infants and children with frontal subarachnoid space widening seen during a 5-year period were retrospectively studied. Only 22.3% of patients had macrocephaly. Comparison of the clinical and radiologic characteristics of subarachnoid widening in macrocephalic children and in normocephalic or microcephalic children showed no significant differences. It is concluded that undue emphasis has been laid on the association between this radiologic sign and macrocephaly. Widening of the subarachnoid space in children may be a variation of normal development of brain whereby there is a transient accumulation of cerebrospinal fluid in the frontal region. The term external hydrocephalus may therefore be inappropriate in the absence of significant ventricular widening and clinical signs of raised intracranial pressure.Child s Nervous System 01/1992; 8(1):36-39. · 1.24 Impact Factor