Parenchymal Neurocutaneous Melanosis in Association with Intraventricular Dermoid and Dandy-Walker Variant: A Case Report

Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

MRI Findings of Intracranial Neurenteric Cyst: Cast Report

Neurenteric cyst is very rare congenital disease and usually presents symptoms in adulthood. It can cause major neurological deficits if detection and treatment are delayed. It is also known to be a source of serious infection of the central nervous system. Neurenteric cyst is usually located in the ventral aspect of the lower cervical or upper thoracic spinal cord. Intracranial neurenteric cyst is very uncommon and less than 35 cases have been reported in the literature. We report MRI findings of two cases of intracranial neurenteric cyst and review the literature.

MR Findings of Kallmann Syndrome

Kallman syndrome is characterised by hypogonadotrophic hypogonadism and anosmia. T1 weighted MR coronal images show the bilateral absence or hypoplasia of olfactory bulbs, tracts, and sulci. We report the MR findings of Kallmann syndrome and revew the literature.

Prominent Extraaxial CSF Space on Cranial Ultrasound in Infants: Correlation with Neuro d evelopmental Outcome

PURPOSE: To determine the clinical significance of prominent extra-axial CSF space (EACSFS) in infants, as seen on cranial ultrasound. MATERIALS AND METHODS: Between March 1996 and November 1997, all infants who had undergone head ultrasound at our institution and were found to have prominent EACSFS were evaluated. The width of the interhemispheric fissure was measured at three locations at the level of the frontal horn, body and atrium of the lateral ventricles. The depth of the CSF space over the convexity was also measured. The average of these measurements was calculated and each patient was assigned to one of three groups: mild, moderate, or marke d. Ultrasound findings were evaluated for other associated abnormalities. Clinical neurodevelopment was evaluated by a pediatric neurologist, and ultrasound and neurodevelopmental findings were correlated. RESULTS: Prominent EAC S FS was found in 153 patients, and neurodevelopmental evaluation up to a corrected age of 9 months was available in 133. One hundred and eight of 117 infants with normal neurodevelopment had no other associated abnormality (n=81), or abnormality associated only with grade I subependymal hemorrhage or cyst (n=27). Twe l ve of 16 infants with an abnormal neurodevelopmental outcome had major abnormalities including PVL, grade IV hemorrhage, and marked ventriculomegaly. CONCLUSION: Prominent EAC S FS alone does not appear to be clinically significant. An abnormal neurodevelopmental outcome is associated with major abnormalities seen on ultrasound. Follow-up examination for prominent EAC S FS is not indicated unless the associated abnormality requires further evaluation.