1/11. Bilateral substantia nigra changes on MRI in a patient with encephalitis lethargica.A 33-year-old woman admitted for meningoencephalitis had features of encephalitis lethargica develop on her third day of illness. She had ophthalmoplegia, akinetic mutism, and prominent extrapyramidal signs consisting of lip and hand tremors, cogwheel rigidity, and facial bradykinesia.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
2/11. Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration.A 58-year-old Chamorro female patient, who died in 1993, was examined clinicopathologically. At the age of 51, she suffered from hemiparkinsonism, then bradykinesia, rigidity without tremor, and dementia. Extrapyramidal symptoms developed, and at the age of 57, vertical gaze palsy was noted. The clinical diagnosis was parkinsonism-dementia complex (PDC) with vertical gaze palsy. The brain showed atrophy in the frontal and temporal lobes, and the atrophy was accentuated in the dentate gyrus, Ammon's horn and parahippocampal gyrus. The basal ganglia, thalamus and midbrain were moderately atrophic. The substantia nigra and locus ceruleus were completely depigmented. Numerous neurofibrillary tangles (NFTs) were seen in the subiculum and amygdaloid nucleus. Many NFTs were evident in the parahippocampal gyrus, lateral occipitotemporal gyrus, insula, Sommer sector, basal nucleus of meynert, lateral nucleus of the thalamus, subthalamic nucleus and brain stem, and several were observed in the globus pallidus and hypothalamus. The Sommer sector, substantia nigra, locus ceruleus and basal nucleus of meynert showed severe loss of neurons, and a moderate loss of neurons was exhibited by the globus pallidus. These findings were apparently consistent with those associated with PDC. However, in this patient, severe neuronal loss was seen in the subthalamic nucleus and lateral nucleus of the thalamus, and grumose degeneration, which has not previously been reported in PDC, was seen in the dentate nucleus. In addition, many tufted astrocytes, which have been reported to occur in progressive supranuclear palsy (PSP) and postencephalitic parkinsonism, but scarcely observed in PDC, were present. Furthermore, astrocytic plaques, which have been considered as a specific finding of corticobasal degeneration (CBD), were observed in the cerebral cortex. On the other hand, granular hazy astrocytic inclusions, previously reported to occur in PDC, were not seen. Chromatolytic neurons were not observed. The question thus arises as to whether it is appropriate to consider this patient as having suffered from a combination of PDC, PSP and CBD. From the view points of absence of granular hazy astrocytic inclusions and chromatolytic neurons, and of tufted astrocytes in the neostriatum, it is conceivable that this patient is a case of a new disease entity.- - - - - - - - - - ranking = 0.5keywords = nigra (Clic here for more details about this article) |
3/11. amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators.We describe two patients with sporadic amyotrophic lateral sclerosis (ALS), who had developed progressive external ophthalmoplegia of a predominantly supranuclear type while they survived on respirators, and displayed histopathological abnormalities both typical and atypical of ALS. Patient 1 was a 43-year-old man with ALS of 5-year duration, who had initially exhibited fulminant ALS, and remained on a respirator for 4 years. Patient 2 was a 51-year-old man with ALS of 13-year duration, who remained on a respirator for 8 years. Both patients died in a "totally locked-in state". autopsy of both patients revealed not only histopathological abnormalities consistent with ALS, but also multisystem degeneration which involved the pontine tegmentum, substantia nigra, Clarke's dorsal nuclei and spinocerebellar tracts. In addition, Patient 2 displayed intracytoplasmic neuronal basophilic inclusion bodies which exhibited marked immunoreactivity to anti-ubiquitin antibodies. Our case reports indicate that the longer survival which is possible through the use of respirators may make one subgroup of ALS patients prone to develop atypical clinical and neuropathological features which are not observed during the natural course of ALS.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
4/11. motor neuron disease with slow eye movements and vertical gaze palsy.Two middle-aged patients with motor neuron disease showed common eye movement disorders and intellectual impairment in the later stage of the illness. Eye movement disorders were characterized by slow saccades and vertical glaze palsy, which seemed to be supranuclear ophthalmoplegia. Neuropathological examination of one patient revealed degeneration of the substantia nigra without pathological changes in the ocular motor nuclei, in addition to findings compatible with motor neuron disease. These cases appear to raise a possibility of a distinct group of multiple system degenerations.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
5/11. Immunohistochemical study of a case with progressive supranuclear palsy without ophthalmoplegia.A case of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome; PSP) with parkinsonism and absence of gaze palsy or mental changes is reported. Neuropathological examination, apart from typical changes, showed, lack of midbrain tegmentum demyelination, marked loss of purkinje cells and presence of hyalin-like bodies in individual neurons of the substantia nigra. Immunostaining against tau-1 protein revealed the prevalence of a diffuse reaction in locus coeruleus neurons; reflecting either different ability of these cells to accumulate straight filaments, or a various time sequence of neurofibrillary tangles formation. Ferritin immunohistochemistry demonstrated widespread microglial cell proliferation, confirming further the generalized character of CNS pathology in PSP.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
6/11. Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators.patients with amyotrophic lateral sclerosis (ALS), who survive longer on a life-support system, exceeding the natural course of this disease, show new features of ALS. We report here a clinico-pathologic study of a 51-year-old patient with sporadic ALS who developed progressive external ophthalmoplegia 3 years after he remained on a respirator and died 5 years later, 13 years after the onset of his illness. The external ophthalmoplegia was initially accompanied by preserved doll's eye phenomenon, which later became absent. autopsy revealed not only degeneration of the upper and lower motor neuron systems typical of ALS, but also degeneration of the Clarke's dorsal nuclei, spinocerebellar tracts, substantia nigra and inferior olives in addition to intracytoplasmic neuronal inclusion bodies in various areas. The oculomotor and abducens nuclei were variably involved, accompanied by neurogenic atrophy of the extraocular muscles. Our case report is consistent with the idea that ALS comprises a heterogeneous group of disorders, and also indicates that long-term use of respirators may make some patients with this illness prone to developing atypical clinical and neuropathologic features which are not observed during the natural course of ALS.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
7/11. Sporadic case resembling autosomal-dominant motor system degeneration (Azorean disease complex).We describe a case of an adult-onset progressive dystonia with external ophthalmoplegia, occurring in a black man without a family history of neurologic disorders. Neuropathologic examination demonstrated neuronal loss and gliosis in the anterior horn and Clarke's column in the spinal cord, nuclei of cranial nerves III, VI, X, and XII, vestibular complex, lateral cuneate nucleus, lower pontine tegmentum, red nucleus, substantia nigra, and dentate nucleus. The cerebral cortex, corpus striatum, basis pontis, inferior olives, and cerebellum were spared. The clinical and pathologic findings closely resemble autosomal-dominant motor system degeneration or "Azorean disease," without, however, demonstrable familial transmission. In addition to the absence of a family history, unique features of the case include the presence of Alzheimer type II glial cells in the red nucleus and an unexplained persistent elevated concentration of serum amylase.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
8/11. Oculomotor characteristics of parkinsonism in comparison with those of cerebellar ataxia.Oculomotor characteristics of patients with Parkinsonism have been analyzed. 1. The Parkinsonian patients showed relatively high frequency, but mild degree of abnormalities in various oculomotor functions such as pursuit eye movements, saccade and optokinetic nystagmus and frequently had positional and positioning nystagmus, although all of them might not relate to the pathology of parkinsonism. 2. When a visual target was presented laterally to a subject and head was free to move, the Parkinsonian patients showed relatively good eye-head coordination for gaze shift, resulting in accurate and stable gaze. However, the reaction times of head and eyes were prolonged and overt head movement was observed. 3. Ocular dyskinesias, characterized by smooth, slow, to-and-fro deviations with large amplitude and prominent in darkness were observed in 8 patients out of 10 with bodily dyskinesia while taking levodopa. 4. Oculogyric crises, paralysis of downward gaze, prolonged reaction time of saccade and poor optokinetic nystagmus were seen in a patient with juvenile Parkinsonism, whose neuropathological examination revealed the most striking changes in the substantia nigra, central grey and midline portion, and ventral and caudal to the central grey.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
9/11. The pathology of machado-joseph disease. Report of a possible homozygous case.The clinical and pathological findings in a boy suffering from machado-joseph disease are described. The patient was the son of two affected parents and signs first appeared at the age of 8 years. A younger brother also became affected at the age of 7. The patient presented all the characteristic features of the disease which consist of progressive cerebellar ataxia, pyramidal signs, progressive external ophthalmoplegia with variable degrees of extrapyramidal and peripheral signs. He died at the age of 15, after an unusually short duration of the disease. Pathological examination showed degeneration and mild gliosis of the substantia nigra, dentate, pontine and cranial nerve nuclei, anterior horns and Clarke's columns. Additional findings, not previously described were the involvement of sensory ganglia, intermediolateral columns and gracile and cuneate nuclei. It is suggested that the present case is homozygous for the gene of machado-joseph disease. The pattern of distribution of the pathological lesions and the sparing of some anatomical structures confirm our view that machado-joseph disease should be considered a distinct clinico-pathological entity within the group of the autosomal dominant ataxias.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
10/11. motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction.We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian systems; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.- - - - - - - - - - ranking = 0.25keywords = nigra (Clic here for more details about this article) |
| | Next -> |