SCLEROSI MULTIPLA AD ESORDIO DEMENZIALE: OSSERVAZIONE DI UN CASO CLINICO

Moderate or severe cognitive impairment is rare as the first symptom in multiple sclerosis. We observed a 26 year-old female who had for four months been showing memory and language disturbances, abulia, mood changes. Mild gait ataxia, brisk tendon reflexes at the four limbs, milk weakness of the left leg and bilateral paleness of temporal portion of the optic disk were present at the neurological examination. The patient was anosognosic. She showed disturbances of short and long term memory, anomia, phonemic paraphasias, constructional apraxia, spatial disorientation. MR brain scan showed marked cortical and deep atrophy. Also corpus callosum was atrophic. Multiple, confluent areas of high signal intensity in T2-weighted images were also appreciable in the white matter of centrum semiovale bilaterally, both in periventricular and subcortical location. Another lesion was present in the left middle cerebellar peduncle. HM-PAO SPECT brain scan revealed cortical hypoperfusion in the left frontal and parietal lobes. VEPs, BAEPs and MEPs gave pathological results. Oligoclonal IgG banding and intrathecal IgG synthesis resulted from CSF analysis. According to Poser's criteria the patient was diagnosed as having definite MS (laboratory-supported definite MS B2). In MS patients, cognitive disorders have been found to be associated with atrophy of the corpus callosum, widespread periventricular lesions and derangements of cortical perfusion assessed by SPECT. The observed finding of cortical hypoperfusion in the frontal lobe is in line with previous reports. In this case, the discrepancy between the severe mental impairment and the mild physical disability could be explained by the fact that brainstem and, probably, spinal cord structures were somewhat spared by the demyelinating process.