Four children with epilepsy, ranging in age from 8 to 15 years, and diagnosed with Chiari type I malformation by brain magnetic resonance imaging (MRI), are reported from La Sapienza University, Rome, Italy. Seizures were complex partial, and the EEG showed temporal and parieto-occipital sharp waves or spikes. Headaches occurred in one child. The MRI showed no cortical structural involvement compatible with epileptogenic dysgenesis. An interictal SPECT showed cortical areas of hypoperfusion that correlated with the EEG focal abnormalities. A cerebellar hypoperfusion was also shown in 2 of the patients, suggesting an associated functional or structural lesion. [1]

COMMENT. Headache and neck pain are the most common symptoms associated with Chiari I malformation in childhood,. and seizures are infrequently reported. The MRI of the cerebrum is usually normal, and seizures are assumed to be cryptogenic. Brain SPECT, revealing a functional or structural lesion, appears to offer a cortical and/or cerebellar explanation for the seizures in some cases, when the MRI evidence for microdysgenesis is lacking.