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In almost all of the earlier reported cases of Kufs’ disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination revealed an apparent poor diffusion of specific lipofuscinic deposits, the finger print profiles (FPs). We report the ultrastructural data from skin, muscle(More)
The powers of the theta (3.5-7 Hz), alpha (7.4-12.3 Hz) and beta 1 (12.9-14.8 Hz) rhythms were analysed in symmetrical derivations. Sixteen right-handed young male adults were observed: at rest, with eyes closed (EC) (4 sequences) and eyes open (EO) (4 sequences); during right then left tonic alternate or sequential movements of the hand and while gazing to(More)
Kufs disease is the adult form of ceroid neurolipofuscinosis, and an uncommon cause of degenerative nervous system disease affecting young adults. We present here 4 cases of family form revealed by a demential syndrome. In all 4 patients MRI showed diffuse cortical atrophy predominant in the parietal regions. In 3 of these 4 patients MRI also exhibited a(More)
Circumduction-related nystagmus is an extremely rare and complex oculomotor anomaly. A case observed in a patient with multiple sclerosis was monocular. The lack of any motor paralysis sign demonstrated that central oculomotor pathway dysfunction can affect only one eye. This would only be possible if the central oculomotor pathways were at least in part(More)
Musical dichotic listening by musicians and non-musicians free from nervous affections was investigated under different situations which provoke modification of activity of each cerebral hemisphere. The melodic stimuli produced a greater response in the left hemisphere in musicians and in the right hemisphere in the non-musicians.
Monocular circumduction nystagmus is a very rare ocular movement. A type of circumduction movement, it is caused by successive movements of all the ocular muscles within the framework of a pendular nystagmus. As a monocular movement, it raises the question of the physiopathological mechanism of such a movement which is so complex and is also limited to one(More)
Ocular bobbing is classified as an abnormal eye movement, resulting from pathological neuronal activity of the brain stem after bilateral pontine lesions. Clinical oculographic study of 5 patients suffering from ocular bobbing shows that upwards voluntary eye movements are abnormal. We suggest that ocular bobbing should not to be regarded as an abnormal eye(More)