Early thalamic and cortical hypometabolism in adult-onset dementia due to metachromatic leukodystrophy.

A case of early-onset adult dementia with family history of dementia is reported, characterised by neuropsychological deficits, suggesting frontal involvement, with mild non specific white matter abnormalities on CT scan. Familial Alzheimer's disease was suspected but the neuropathological diagnosis on brain biopsy was metachromatic leukodystrophy. 18FDG-PET revealed a very peculiar pattern of metabolic impairment in thalamic areas, in medial and frontopolar regions, and in occipital lobes. Neuropsychological follow-up showed relatively stable difficulties of long-term memory and signs of frontal lobe dysfunction, similar to those observed in subcortical dementias. MRI subsequently showed periventricular leukoencephalopathy. The brain metabolic pattern observed in that case of metachromatic leukodystrophy was quite different from that reported in other types of dementia.

[A case of delayed post-anoxic encephalopathy after acute carbon monoxide poisoning]. / A propos d'un cas d'encéphalopathie post-anoxique différée après intoxication aiguë au monoxyde de carbone.

The authors report the case of a young 18 year-old man presenting delayed encephalopathy after severe intoxication with carbon monoxide, and transient cerebral swelling. This condition is characterized by diffuse demyelinating lesions of both cerebral hemispheres, the mechanism of which is unknown.

Giant fusiform aneurysm of the basilar artery. Clinico-pathological report of 3 cases.

From 9000 autopsies, three giant fusiform aneurysms of the basilar artery were found in middle-aged men with arterial hypertension, atherosclerosis, symptoms of encephalic circulatory disturbances, transient then permanent cranial nerves palsy. Neuroradiological investigations suggested a basilar dolichomegartery in one case. Anatomically, these unruptured giant aneurysms (respectively: 2, 2.5 and 4.5 cm largest transversal diameter) compressed the brainstem; a recent softening destroyed the midpons in one case, and both cerebral peduncles in another. A review of the literature underlined the paucity of similar cases, and the difficulty of differential diagnosis of giant basilar aneurysms detected only by neuroradiological investigations without anatomical verifications.

[Progressive multifocal leukoencephalopathy after renal transplantation. Report of two cases]. / Leucoencéphalopathie multifocale progressive après transplantation rénale. A propos de deux observations.

Progressive multifocal leukoencephalopathy (PML) occurred in two patients after kidney transplantation. Two years after such a transplantation associated with immunosuppressive chemotherapy, a 54-year-old male developed polyneuropathy, diffuse alterations of the central nervous system and he died with the suspicion of hypertensive encephalopathy due to progressive renal failure. A 45-year-old female had kidney transplantation first complicated by Listeria monocytogenes meningoencephalitis. She was cured from this disease and had a satisfactory social rehabilitation for two years. Afterwards, she suffered from various neurological ailments, including epilepsy, that were attributed to combined renal failure and developing hydrocephalus. One year after the onset of these neurological symptoms, the grafted kidney was removed and chemotherapy was discontinued but she died a few weeks later. Both patients had typical PML. By electron microscopy, performed on formalin fixed brain tissue, intranuclear round particles (40-50 nm) could be recognized in the first case only. These two cases are confronted with the six published observations of PML following organ transplantation. The frequency of PML has been estimated at 1 for 5000 kidney transplantation, 1 for 2000 chronic lymphoid leukemia and 1 for 10,000 Hodgkin's disease.

Two cases of progressive multifocal leukoencephalopathy after renal transplantation.

Progressive multifocal leukoencephalopathy (PML) occurred in two patients after kidney transplantation. Less than 2 years after such a transplantation associated with immunosuppressive chemotherapy a 54-year-old male developed polyneuropathy then clinical diffuse alteration of the central nervous system. He died three months later with the suspicion of hypertensive encephalopathy due to progressive renal failure. A 45-year-old female had a kidney transplantation first rapidly complicated by Listeria monocytogenes meningoencephalitis. She was cured from this disease and had a satisfactory social rehabilitation during two years. Afterwards, she suffered various neurological troubles, including epilepsy, that were attributed to combined renal failure and developing hydrocephalus. One year after the onset of these neurological symptoms, the grafted kidney was removed and chemotherapy was discontinued. She died three months later. Both patients had typical PML with eosinophilic intranuclear inclusions in presumptive oligodendroglial cells. By electron microscopy, performed on formalin fixed brain tissue, round particles (40-50 nm) could be recognized in some glial cell nuclei. These two cases are confronted with the four published observations of PML following organ transplantation.

[Crossed aphasia in a right-handed patient: a report on one case (author's transl)]. / Aphasie croisée chez une droitière. A propos d'une observation.

The authors report on a 37 year old right-handed patient with a right rolando-parietal infarct which was manifest clinically by not only sensory-motor disorders of the left side of the body, but more especially by an expression aphasia. This observation fulfills the criteria for defining the exceptional case of crossed aphasia in a right-handed patient: aphasic destruction of language engendered by a right unilateral lesion in such a patient, without a previous history of a cerebral lesion or family history of left-handedness. Comparing the aphasic signs in this patient with the rare cases reported in the published literature shows that there is a particular pathognomonic profile, close to the type of aphasic disorganization seen in children and left-handed adults. The authors review the neuropsychopathogenic explanations for this crossed aphasia of right-handed patients.