Functional neuroradiologic investigations in band heterotopia.
Band heterotopias are an example of genetic generalized neuronal migration disorders that may be present in patients with mild epilepsy and normal or slightly impaired intellect, as well as in patients with intractable epilepsy and mental retardation. The case of a 17-year-old left-handed female patient with epilepsy and normal cognitive development is reported in whom single-photon emission computed tomography (SPECT), proton magnetic resonance spectroscopy, and functional magnetic resonance imaging (fMRI) were performed. MRI revealed the presence of bilateral asymmetric band heterotopia. SPECT revealed a left frontoparietal and occipital hypoperfusion, demonstrating a good correlation with the electroencephalogram abnormalities. Because of the appearance of new types of seizures, the patient underwent a second MRI investigation together with a proton magnetic resonance spectroscopy (MRS) study. MRI confirmed bilateral band heterotopia characterized by greater thickness in the left hemisphere at the frontal and occipital level. MRI and SPECT findings were in agreement with left occipital electroencephalogram abnormalities and with occipital seizure type. Qualitative results of proton MRS revealed normal spectra profiles in the examined left frontal and occipital heterotopic area and in the normal overlying cortex. Later, fMRI was performed. The finger-tapping test of the right hand yielded the activation of both normal left sensory-motor cortex and the facing band heterotopia. In the right hemisphere, only the activation of the sensory-motor neocortex was observed; no involvement of the right misplaced brain tissue was present. This functional behavior could be considered the consequence of poor neuronal representation. On the contrary, the involvement of both band heterotopia and normal cortex observed in the left hemisphere could be the result of many synaptic interconnections. Functional investigations may have an important role in defining the activity of band heterotopia per se and in relation to the overlying neocortex.
Division of Pediatric Neurology, Pediatrics Department, "La Sapienza" University, Roma, Italy.
P
P
Iannetti
A
A
Spalice
U
U
Raucci
F M
FM
Perla
D000293:Adolescent; D001921:Brain / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D002465:Cell Movement / Q000235:genetics; D002828:Choristoma / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D004292:Dominance, Cerebral; D004827:Epilepsy / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D007360:Intelligence; D008279:Magnetic Resonance Imaging; D020125:Mutation, Missense; D019275:Radiopharmaceuticals / Q000627:therapeutic use; D019690:Technetium Tc 99m Exametazime; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods
D019275:Radiopharmaceuticals; D019690:Technetium Tc 99m Exametazime