Clinical Neuroscience

[Diffusion weighted magnetic resonance imaging of metachromatic leukodystrophy - a case report]

PINTÉR Nándor1, KOLLÁR Katalin2, RUDAS Gábor3, BARSI Péter3

MARCH 25, 2009

Clinical Neuroscience - 2009;62(03-04)

[We present the case of a 7 years old boy with the juvenile form of metachromatic leukodystrophy. Beside conventional sequences, a diffusion-weighted magnetic resonance study was performed. On T2-weighted images a diffuse hyperintensity of the cerebral white matter with a typical tigroid pattern was seen as a well-known characteristics of the disease. No difference was detectable on T2-weighted images between the different cerebral white matter regions. Diffusion study showed low apparent diffusion coefficient values in the temporal and orbitofrontal regions and normal values in other cerebral regions compared to the normal appearing cerebellar white matter, wich is partly consistent with the previously reported data. The results of early diffusion studies of the disease are not straightforward and the physical and biochemical backgrounds of the diffusion properties still remain unknown. The more data we collect, the closer we get to the understaning of the diffusion changes in metachromatic leukodystrophy.]


  1. Semmelweis Egyetem, Kardiológiai Központ, Képalkotó Diagnosztikai Részleg, Budapest
  2. Heim Pál Gyermekkórház, Neurológiai Osztály, Budapest
  3. Semmelweis Egyetem, MR Kutatóközpont, Budapest



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[Aims - This review is a summary of four studies investigating the effect of epileptic disturbance and other functional factors on the brain lateralization. Methods - Patients were investigated by functional MRI by using visual, sensorimotor, memory, and speech paradigms. Results - 1. Malformations of cortical development localized within speech centers induce contralateral speech reorganization in only 50% of cases. 2. Epileptic disturbance can induce a reorganization in language lateralization independent of the effect of lesion. 3. Bilateral epileptic activity can prevent a reorganization of memory functions contralateral to the seizure onset region. 4. Injury of the right upper limb can induce a left-to-right shift of language lateralization. Conclusions - Our results may have implication during neurorehabilitation process and neurosurgical interventions. Participation of malformations of cortical development in cognitive functions should keep in mind during surgery. Reorganization of speech- and memory functions should also be considered during operations of central nervous system.]

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[We previously reported a male patient with an 18q21.3 deletion, hyperuricemia and typical symptoms of the Lesch- Nyhan syndrome who lacked hypoxanthine-guanine-phosphoribosyl- transferase (HGPRT) deficiency. The patient developed progressive peripheral neuropathy in additon to his profound mental retardation and self-injurious behavior. At the age of 23 years MR imaging revealed globally delayed myelination with relative sparing of the corpus callosum and frontal lobes. They were focal hyperintensities suggestive of gliosis. Multimodality evoked potentials found evidence of impaired central and peripheral conduction. Single photon emission computed tomographic (SPECT) imaging demonstrated left frontal hyperperfusion and under it a temporoparietal hypoperfusion.]

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