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1/12. White matter hyperintensities on MRI in a patient with corticobasal degeneration.

    We describe a patient who presented with the clinicopathological features of corticobasal degeneration (CBD). Over the course of 8 years, the patient developed myoclonus, dystonia, and supranuclear gaze palsy associated with an akinetic-rigid syndrome. To our knowledge, no previous report of a patient with CBD has described clear-cut regional white matter changes as revealed by magnetic resonance imaging (MRI) scans. In our patient, a T2-weighted MR image of the brain showed focal atrophy of the bilateral frontal cortex and asymmetric regional hyperintensities of the subjacent white matter. These signal changes seemed to primarily reflect the progression of neuronal degeneration, especially the demyelination secondary to axonal loss or change.
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ranking = 1
keywords = supranuclear, palsy
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2/12. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease.

    We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. magnetic resonance imaging showed evidence of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the literature. We have also reviewed the literature of PT secondary to neurodegenerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknown etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progressive cerebellar ataxia and approximately two thirds of them have rhythmic tremors elsewhere. ear clicks are observed in 13% and evidence of hypertrophy of the inferior olivary nucleus in 25% of the patients. The common neurodegenerative causes of PT are OPCA/multiple system atrophy, Alexander's disease, and, in most of them, the result of an unknown cause.
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ranking = 4.2744767667135
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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3/12. A case of clinically and neuropathologically atypical corticobasal degeneration with widespread iron deposition.

    A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy, the unfixed brain weighed 820 g. atrophy was circumscribed in the frontal lobe on both sides. The globus pallidus and the caudate nucleus were markedly atrophic and gold yellow in color, and the substantia nigra was strikingly pale. The cortical area showed neuronal loss and status spongiosus of the second and third cortical layers with ballooned neurons. Marked neuronal loss was observed in the dorsomedial nucleus of the thalamus, Meynert basal nucleus and substantia nigra. With Holzer stain, fibrillary gliosis was found to be severe in the frontal lobe, globus pallidus, subthalamic nucleus, hippocampus, dorsomedial nucleus of thalamus, substantia nigra, pontine tegmentum and inferior olivary nucleus. With Bielschowsky-Hirano stain, neurofibrillary tangles were observed in the cortex, hippocampus, substantia nigra, dentate nucleus, subthalamic nucleus, pontine nucleus, the inferior olivary nucleus, dorsomedial nucleus of the thalamus and, to a lesser extent, the neostriatum. Strikingly numerous argyrophilic and tau-positive threads were present in the cerebral white matter. These neuropathological findings corresponded to corticobasal degeneration, but lesions characteristic of progressive supranuclear palsy were also found. Moreover, widespread iron deposition throughout the central nervous system was the most striking finding of the present case. To our knowledge, such a case has not been reported in the literature to date.
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ranking = 4.2744767667135
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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4/12. 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 = 21.372383833567
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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5/12. Discrepancy between clinical and pathological diagnoses of CBD and PSP.

    Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are often clinically confused with each other. Moreover, the discrepancy between clinical and pathological diagnoses of CBD and PSP are still controversial. We report here two atypical cases of PSP and CBD. A 73-year old woman was admitted with right hand rigidity, limb kinetic apraxia and cortical sensory loss. Brain atrophy, hypoperfusion and hypometabolism predominantly in the left frontoparietal lobes indicated CBD clinically. Pathological studies revealed neuronal loss and spongy change without ballooned neurons (BN) in the cerebral cortex. Modified Gallyas-Braak (G-B) staining revealed neurofibrillary tangles (NFTs) and tufted astrocytes, indicating pathological diagnosis of PSP. A 75-year-old man admitted with vertical gaze palsy, neck dystonia, parkinsonism and dementia. atrophy of the frontal lobes and tegmentum of the midbrain and symmetrical frontal hypoperfusion in SPECT indicated PSP. However, neuronal loss and BN in the frontal lobes and clusters of astrocytic plaques indicated CBD pathologically. The G-B staining was useful for differentiating between CBD and PSP, but our atypical cases bring up a new issue about differential diagnosis of CBD and PSP.
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ranking = 1.3958577009934
keywords = supranuclear, supranuclear palsy, palsy
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6/12. Visual facial grasp.

    Some patients with degenerative neurological diseases have a release of the vestibular-ocular reflex (VOR), as detected by passive head movement during visual fixation on a moving target ("doll's eyes"maneuver). However, a positive doll's eyes sign might be induced by other defects and the purpose of this article is to describe a new ocular sign of cortical dysfunction, the visual facial grasp. We observed three patients, one with progressive supranuclear palsy (PSP), another with probable Alzheimer's disease (AD) and a third with cortico-basal degeneration (CBD) all of whom appeared to demonstrate a release of the vestibulo-ocular reflex (VOR) with passive head movements. Whereas the patient with PSP, who was unable to inhibit the VOR regardless of the visual target used probably had a true release of the VOR, the patients with AD and CBD were able to inhibit this reflex when the visual target was the examiner's moving face. These two patients also exhibited spontaneous preference for visual fixation on the examiner's face and improvement in smooth pursuit when the examiner's face was the visual target. This clinical observation suggests that the deficits in these two patients with AD and CBD were related to the emergence of a primitive stimulus-bound behavior, the visual facial grasp.
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ranking = 4.2744767667135
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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7/12. Neurodegenerative disorders mimicking progressive supranuclear palsy: a report of three cases.

    Progressive supranuclear palsy (PSP) is rarely confused with other parkinsonian disorders once the vertical gaze palsy appears. Corticobasal degeneration is the most common differential diagnostic entity. We describe three cases diagnosed during life as PSP but found to have another neurologic disorder at autopsy. No explanation for the gaze palsies was found in any case.
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ranking = 18.493764767847
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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8/12. Primary progressive freezing gait: a syndrome with many causes.

    Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian degeneration (PNLD) and diffuse lewy body disease. Four others evolved clinically into progressive supranuclear palsy and corticobasal degeneration. PPFG is not a distinct disorder but a syndrome with diverse causes. Long-term follow-up (> or =10 years) and postmortem are required for accurate diagnosis. PNLD may be the primary form of disease.
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ranking = 4.2744767667135
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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9/12. Argininemia: a treatable genetic cause of progressive spastic diplegia simulating cerebral palsy: case reports and literature review.

    Argininemia, a rare autosomal recessive urea cycle disorder, is caused by a deficiency of arginase, with resulting elevated plasma arginine and ammonia levels. Reports to date have focused little on the neurology of this disorder or the efficacy of treatments. A medline search revealed 25 previously reported cases, to which we have added two brothers who presented with late onset progressive spastic diplegia. Though their degree of enzyme deficiency was comparable, the severity of their phenotypic abnormalities differed substantially. With dietary therapy, both showed improved cognitive and motor function. Late metabolic crises occurred in both, resulting in death of the less severely affected brother. Based on analysis of our clinical database, we report on the full spectrum of neurologic abnormalities seen in argininemia with particular focus on the accompanying progressive spastic diplegia and its response to treatment; progressive decline in head growth; distinctive neuroradiologic findings; and life-threatening later complications. Current and potential future therapies and long-term outcome are summarized.
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ranking = 0.00086606472681705
keywords = palsy
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10/12. Distribution of cerebral cortical lesions in corticobasal degeneration: a clinicopathological study of five autopsy cases in japan.

    We investigated five Japanese patients with autopsy-proven corticobasal degeneration (CBD) both clinically and pathologically, and examined the distribution of their cerebral cortical lesions in hemisphere specimens. The lesions were classified into three categories (slight, moderate and severe). Only two of our patients had clinical features considered to be typical of CBD. Severe lesions were present in the posterior portions of the frontal lobe, anterior to the precentral gyrus in two patients with the clinical diagnosis of CBD. By comparison, in two patients with clinically diagnosed frontal Pick's disease, and one with the clinical diagnosis of progressive supranuclear palsy (PSP), severe lesions were seen in the anterior portions of the frontal lobe. The primary motor area of all five had mostly slight to moderate lesions. We postulate that the clinical features of CBD have a much wider spectrum than previously believed. Our data also indicate that the lesion responsible for limb-kinetic apraxia in CBD is in the premotor cortex. We suggest that when the anterior portions of the frontal lobe are damaged, the clinical picture mimics those of Pick's disease and PSP. In addition, we consider that focal cerebral atrophy of CBD is multicentric.
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ranking = 4.2744767667135
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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