Cases reported "Gliosis"

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1/16. Bielschowsky bodies (Lafora bodies of Bielschowsky type): report of a case associated with Rosenthal fibers in the brain stem.

    Bielschowsky bodies are an uncommon type of polyglucosan body. Similar to Lafora bodies, they are characteristically identified within neuronal perikarya and neurites. However, they lack the diffuse distribution of Lafora bodies, and instead are typically restricted to the external pallidum, often in association with status marmoratus or atrophy of the putamen. Fewer numbers of Bielschowsky bodies have also been identified in other areas such as the substantia nigra, putamen and inner globus pallidus. We report an additional case with Bielschowsky bodies in an 18-year old female with cerebral palsy. This case demonstrated multifocal Bielschowsky bodies and abundant Rosenthal fibers in the midbrain and pons. To our knowledge the association of Bielschowsky bodies with this peculiar distribution of Rosenthal fibers has not previously been reported.
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ranking = 1
keywords = palsy
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2/16. 4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy.

    We report a 67-year-old man with 4-repeat (4R) tauopathy sharing both features of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Although CBD and PSP have a common pathological feature that 4R tau accumulates in neurons and glia, recent pathological studies have confirmed differences between the two disorders. Clinical features of the present case were asymmetrical apraxia, parkinsonism, memory disturbance, disorientation and left limb myoclonus with a 5-year history. Pathological features were the widespread occurrence of 4R tau-positive structures including pre-tangles, neurofibrillary tangles, astrocytic plaques, tufted astrocytes, coiled bodies and argyrophilic threads. Biochemically, immunoblotting of insoluble tau demonstrated the low molecular fragments of 37 kDa and 33 kDa observed in typical CBD and PSP, respectively, in addition to the presence of 4R tau isoforms. The present case shared tau-related pathological and biochemical features of CBD and PSP. These findings support that CBD and PSP are closely associated disorders having a pathogenesis common to 4R tauopathy.
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ranking = 86092.888456465
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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3/16. dopamine D1 and D2 receptors in progressive supranuclear palsy: an autoradiographic study.

    dopamine D1 and D2 receptors were studied in brain tissue sections from a typical patient with progressive supranuclear palsy and in 7 age-matched brains. The density of D1 receptors in the caudate-putamen and frontal cortex of the patient was within control limits. By contrast, the density of nigral D1 receptors and striatal D2 receptors was dramatically reduced in the patient as compared to the control brains. This work shows again that the loss of striatal D2 receptors is the most plausible explanation for the poor response to dopaminergic drugs in patients with progressive supranuclear palsy. While the loss of nigral D1 receptors can be explained by the loss of nigral neurons, it seems that neurons bearing striatal D1 receptors are spared in progressive supranuclear palsy. The clinical effects of selective D1 agonists are worth testing in this devastating disorder.
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ranking = 120530.04383905
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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4/16. character changes from idiopathic cranial pachymeningoencephalitis.

    A 66-year-old man with idiopathic cranial pachymeningoencephalitis was described. He suffered from left orbital pain, and character changes. He became short tempered, and was very attached to trifles. Two years prior to these symptoms, he had developed transient left abducent nerve palsy. head MRI showed a thickening and enhancement of the dura mater on gadolinium-enhanced T1-weighted images, and high signal intensity lesions at bilateral frontal lobes predominantly in the white matter on T2-weighted images. Biopsies revealed microglial proliferation in the cerebral parenchyma, and mild lymphocytic perivascular infiltration. No evidence of intracranial infection was detected. We therefore treated him with methylprednisolone pulse therapy followed by oral prednisolone. His character became gradually normalized, and bilateral frontal lobe lesions seen on MRI disappeared. This is the first case to describe recurrent pachymeningoencephalitis with character changes, and symptoms were probably due to frontal lobe dysfunction.
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ranking = 1
keywords = palsy
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5/16. Pure akinesia: an atypical manifestation of progressive supranuclear palsy.

    Two patients with "pure akinesia" who showed the characteristic changes of progressive supranuclear palsy (PSP) at necropsy are described. They had akinesia but no rigidity or tremor, and ophthalmoplegia was not observed during the course of illness. The symptoms of "pure akinesia" was not improved by levodopa therapy but was considerably improved by L-threo-3,4-dihydroxy-phenylserine. At necropsy, pathological findings were not different from those reported for PSP. It is suggested that "pure akinesia" is an atypical manifestation of PSP, and that norepinephrinergic neurons may be involved in some types of PSP.
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ranking = 86116.401575567
keywords = progressive supranuclear, supranuclear, supranuclear palsy, ophthalmoplegia, palsy
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6/16. Parkinsonism and extraocular motor abnormalities with unusual neuropathological findings.

    Parkinsonian patients with ocular motility abnormalities are usually considered to have progressive supranuclear palsy. However, a number of other conditions have been noted to have the combination of parkinsonism and ocular problems. We report a case of rigid akinetic parkinsonism, oculomotor palsy, and eyelid apraxia with postmortem examination. Our findings are unusual in that there was marked gliosis of the substantia nigra with a large amount of free extracellular neuromelanin despite a 3-year clinical course. Only rare hyaline inclusion bodies and no neurofibrillary tangles were seen in the brainstem. Excessive calcification of the vessels of the globus pallidus were also noted. This case represents another example of the diversity of conditions producing parkinsonism with extraocular motor abnormalities.
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ranking = 17219.577691293
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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7/16. Brain iron in progressive supranuclear palsy: clinical, magnetic resonance imaging, and neuropathological findings.

    A patient with progressive supranuclear palsy demonstrated a region-specific decrease in T2 signal during high-field-strength brain magnetic resonance imaging. At autopsy the T2-signal hypointensity was found to correspond topographically to increased deposition of ferric iron. The potential clinical, radiologic, and pathophysiologic implications of these findings are discussed.
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ranking = 86092.888456465
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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8/16. Progressive supranuclear palsy with lewy bodies.

    An autopsy case is reported which revealed not only clinical and neuropathological features of progressive supranuclear palsy, but also the presence of large numbers of lewy bodies in the brain stem nuclei and cerebral cortex. This case seems to be progressive supranuclear palsy with lewy bodies distributed as in Parkinson's disease. Such case has not been previously reported.
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ranking = 58891.206404146
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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9/16. A case of progressive supranuclear palsy with fibrillary gliosis of the midbrain and pontine reticular formation.

    An autopsy case of progressive supranuclear palsy with onset at age 53 and a clinical course of 7 years is reported. The clinico-pathological characteristics of the case were as follows: Fibrillary gliosis of the midbrain and pontine reticular formation corresponded clinically to personality changes: The patient had formerly been irascible and became extremely mild-mannered. He showed lack of spontaneity and initiative and slowing of thought processes, taking him longer than normal to find words and ideas. gliosis of the nucleus ventralis lateralis of the thalamus was secondary to the degeneration of the cerebellar dentate nucleus and the globus pallidus from which they receive fibers. neurofibrillary tangles were found in the cerebral cortex.
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ranking = 86092.888456465
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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10/16. amyotrophic lateral sclerosis with ophthalmoplegia. A clinicopathologic study.

    ophthalmoplegia is rarely observed in patients with amyotrophic lateral sclerosis (ALS). We describe a patient with ALS in whom ophthalmoplegia, initially appearing midway in her course, progressed to total paralysis of extraocular movements by the time of death. In addition to the usual postmortem findings of ALS in the brain stem and spinal cord, there was extensive neuronal loss and gliosis involving the caudal portions of the dorsal and intermediate components of the oculomotor nuclei, the caudal part of the trochlear nuclei, and the abducens nuclei. This represents the first detailed report of the findings in the nuclei of cranial nerves III, IV, and VI in a patient with ALS and ophthalmoplegia.
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ranking = 141.07871461201
keywords = ophthalmoplegia
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