1/4. Chronic progressive spinobulbar spasticity with disturbance of voluntary eyelid closure. Report of a case with special reference to MRI and electrophysiological findings.We describe a 56-year-old man who had a progressive pseudobulbar palsy, spastic tetraparesis, forced laughing and disturbance of voluntary eyelid closure, and was clinically compatible with chronic progressive spinobulbar spasticity. Magnetic resonance images (MRI) revealed atrophy of the bilateral motor cortices and single photon emission tomography after intravenous injection of N-isopropyl-p-iodoamphetamine iodine-123 (IMP-SPECT) showed hyporadioactivity in the same regions. Electrophysiological studies on supranuclear paralysis of eyelid closure demonstrated that so-called apraxia and motor impersistence coexisted and that in attempts to keep the eyelid closed the inhibition of basal activity of the levator palpebrae superioris muscle and activation of the orbicularis oculi muscle were insufficient, indicating the impaired reciprocity of these ocular muscles. The corresponding lesion of these eyelid symptoms was considered to be the bilateral motor cortices.- - - - - - - - - - ranking = 1keywords = chronic progressive (Clic here for more details about this article) |
2/4. Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis.Although it was first described over a century ago (by Charcot in 1865; by Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not universally accepted. Despite this skepticism, several well-documented cases of isolated degeneration with varying degrees of involvement of corticospinal pyramidal pathways have been reported in the literature. The clinical manifestations in these cases can take one of two forms, ie, isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic tetraplegia associated with a pseudobulbar syndrome featuring severe spastic dysarthria (chronic progressive bilateral spinobulbar spasticity) on the other hand. Obviously, without firm pathologic data, PLS is a hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for bilateral spinobulbar spasticity, it would appear to be the only diagnosis possible once investigate findings have eliminated the other possibilities, such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form of multiple sclerosis. To underscore this point, in this report, five cases of chronic progressive bilateral spinobulbar spasticity developed over 5, 10, 12, 10, and 28 years, respectively, for which the only possible diagnosis was PLS. It was concluded that there are three forms of degenerative diseases of the principal motor pathways: one involving both central and peripheral neurons, ie, amyotrophic lateral sclerosis; one involving only peripheral neurons, ie, spinal amyotrophy; and one involving only central motor neurons, ie, PLS.- - - - - - - - - - ranking = 2keywords = chronic progressive (Clic here for more details about this article) |
3/4. Eales' disease with progressive spastic paraparesis.Eales disease is a disease of unknown aetiology characterised by ophthalmic changes and often neurological involvement as in multiple sclerosis. We are reporting a case of Eales' disease in a 27 yr old male with chronic progressive non compressive motor myelopathy.- - - - - - - - - - ranking = 1keywords = chronic progressive (Clic here for more details about this article) |
4/4. An unusual form of measles meningoencephalitis. A report of two cases.Two patients are reported with a chronic progressive illness characterized by dementia, ataxia and spasticity. There were no myoclonic jerks and both had normal electroencephalograms (EEG). Pathological findings in three brain biopsies were those of viral meningoencephalitis with perivenous demyelination. Serological data in both patients indicated the presence of measles virus infection. Intracytoplasmic structures resembling measles virus nucleocapsids were found in the brain biopsy of one patient. Immunofluorescent staining showed antibody in the temporal lobe biopsy of both patients. It is suggested that these patients are examples of a chronic form of measles meningoencephalitis hitherto undescribed.- - - - - - - - - - ranking = 1keywords = chronic progressive (Clic here for more details about this article) |