Dementia in two histologically confirmed cases of multiple sclerosis: one case with isolated dementia and one case associated with psychiatric symptoms.

Abstract

During the past 10 years, considerable attention has been devoted to cognitive impairment in multiple sclerosis. Occasionally this impairment may be so severe that multiple sclerosis presents as a dementia associated with only minor neurological signs and symptoms. The cases of two women affected by multiple sclerosis who presented with a pure dementia are reported. In the first patient, a progressive apragmatic behavioural disturbance with reduced short term memory and learning abilities were the main clinical features. Neuropathological examination of the brain disclosed numerous plaques in the periventricular white matter, with severe atrophy of the corpus callosum. Plaques were also seen in the white matter of both hippocampus and in the columns of the fornix. The impairment of short term memory could be linked to these lesions. Behavioural changes were probably related to the bilateral lesions of the long associative bundles that disconnected the frontal lobes from other parts of the cerebral hemispheres. In the second patient, visual hallucinations were associated with cognitive dysfunction. MRI showed large plaques in the white matter of both left frontal and temporal lobes. Smaller plaques were also present in the periventricular white matter of the occipital lobes, the nature of which were confirmed by a stereotactic biopsy.

Psychiatric manifestations in cerebrotendinous xanthomatosis

“…MS may cause cognitive disturbance or dementia and, less often, organic psychopathology encompassing behavioural/personality disturbance, mood/affective disorders or psychosis. 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, 101, 102, 103, 104 Psychiatric syndromes, such as bipolar disorders and atypical psychosis, often unassociated with cognitive disturbance or dementia, have been described, and euphoria sclerotica is a characteristic psychosyndrome in MS. 83, 84, 85, 89, 91, 94, 95, 96, 99, 104, 105, 106, 107 Pure psychiatric presentation in MS is rare, as is the case for CTX. 83, 89, 91, 106, 107 Major depressive disorder, which often is prodromal leading to diagnostic delay, is overrepresented in MS patients, as are suicidal ideation, anxiety and sleep disorders, and they are mainly believed to be secondary to physical illness and/or disease-modifying treatment.…”

Pain in Parkinson's disease and multiple sclerosis: Its relation to the medial and lateral pain systems

“…WMLs severely affect cortical neuronal functioning by disconnecting association areas (de-afferentiation) in the prefrontal, parietal, and medial temporal cortex (Charil et al, 2003;Fontaine et al, 1994;Rovaris et al, 2002). Cortical neuronal dysfunction arises from a reduction in cortical cerebral glucose metabolism in the prefrontal cortex, thalamus and hippocampus among others (Blinkenberg et al, 2000).…”

Kognitive Teilleistungsstörungen und Demenz bei Patienten mit Multipler Sklerose: Status quo und offene Fragen

“…Für die Autoren kam daher differenzialdiagnostisch auch eine psychotische Erkrankung als Ursache für die Demenz infrage, weshalb eine frontale Biopsie veranlasst wurde. Das Biopsieergebnis erbrachte dann die gesicherte Diagnose einer MS[54].…”