A 30 month-old girl, child of non-consanguineous parents with no family history of neurometabolic or neurodegenerative disorders, presented with gait disturbance with toe-walking of two weeks’ duration; neurologically she showed the picture of a mild spastic diplegia (increased muscle tone and deep tendon reflexes predominantly in the lower extremities, clonus, Babinski’s sign, Rossolimo’s reflex) with an ataxic component. Brainstem auditory (BAEP), visual (VEP) and somatosensory (SEP) evoked potentials demonstrated cortical latency delays. SE, axial T2-weighted MRI images (TR 2100 msec, TE 90 msec) demonstrated extensive symmetrical high signals in the deep white matter of both cerebral hemispheres without involvement of the arcuate fibers (fig. 1), while there was no abnormal signal from the cerebellar white matter (fig. 2). Nerve conduction velocity (NCV) studies were consistent with a severe demyelinating peripheral neuropathy.

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