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At least 1,531 records · Page 85Linked to original sources
PubMed1991

Presynaptic parkinsonism in olivopontocerebellar atrophy: clinical, pathological, and neurochemical evidence.

The substrate for olivopontocerebellar atrophy parkinsonism is obscure due to the lack of clinical and pathological reports and the absence of studies on dopamine receptors in this entity. We describe a patient with olivopontocerebellar atrophy whose clinical presentation was levodopa-responsive parkinsonism in whom pathological examination disclosed pronounced nigral cell loss with no striatal damage. Autoradiographic labeling with 3H-spiperone showed normal densities of D2 dopamine striatal receptors. These data show that indistinguishable nigral, presynaptic parkinsonism occurs in patients with idiopathic Parkinson's disease and in patients with olivopontocerebellar atrophy, and also how a favorable response to levodopa is neither synonymous with idiopathic Parkinson's disease, nor does it exclude multiple-system, atrophy-related parkinsonism.

Aged↗
PubMedDate not supplied

[Multi-system atrophy or symptomatic orthostatic hypotension].

The authors describe a case of a 74-year-old patient with orthostatic hypotension syndrome. They discuss the possible symptomatic or idiopathic mechanisms of the aetiology of this syndrome, taking into account their possible coincident.

Aged↗
PubMed2003

Movement disorders.

Movement disorders are commonly encountered in clinical practice. The diagnosis of movement disorders relies on a focused history and neurologic examination. Diagnostic steps include (1) identification of the phenomenology of the movements (eg, tremor); (2) characterization of appropriate clinical syndromes; and (3) differential diagnosis of specific disease entities. Accurate diagnosis is essential because symptomatic treatment exists for most movement disorders.

Adult↗
PubMed2007

[Primary central nervous system degeneration in elderly patients. Characteristic imaging features].

Despite further development of new magnetic resonance imaging techniques, e.g., diffusion tensor imaging and 1H magnetic resonance spectroscopy, structural imaging will continue to play a major role in the diagnosis of primary central nervous system degeneration in ageing. Characteristic imaging patterns of multisystem atrophies and primary dementias as well as differential diagnostic features are demonstrated. While such features may have high specificity, their sensitivity is low especially in cross-sectional studies. Longitudinal studies are the optimal method to characterize the dynamic neuroanatomical correlates of the disease. However, according to disease duration and progression, neuroimaging will show increased overlapping and convergence of pathological changes in multisystem atrophy as well as in dementia.

Aged↗
PubMed2003

Pure cerebello-olivary degeneration of Marie, Foix, and Alajouanine presenting with progressive cerebellar ataxia, cognitive decline, and chorea.

Parenchymatous cerebellar cortical atrophy (CCA) usually presents with a "pure" cerebellar ataxia. We describe a patient with a sporadic, late-onset progressive cerebellar ataxia plus cognitive decline and chorea who had CCA at post mortem. We discuss this unique case in the current context of classification of idiopathic cerebellar ataxia.

Brain↗
PubMed1999

An early description of striatonigral degeneration.

Our aim was to revisit the papers published by Scherer 1933 describing four cases of sporadic olivopontocerebellar atrophy (OPCA) thought to represent the earliest description of striatonigral degeneration. One should note that extrapyramidal rigidity associated with OPCA was then considered a type of cerebellar parkinsonism. Two of Scherer's four patients had severe parkinsonism masking cerebellar signs. Pathologically both cases displayed marked degeneration of the striatum and nigra and partially developed pontocerebellar atrophy. Cerebellar ataxia was the outstanding feature in the other two, their pathological study showing severe pontocerebellar lesions and incipient striatonigral atrophy. Scherer stated that the severity of parkinsonism in OPCA is not correlated with the degree of cerebellar degeneration but with that of striatum and nigra. We conclude that Scherer gave the first accurate description of striatonigral degeneration. Moreover, his contribution was essential in ruling out the prevalent notion of cerebellar parkinsonism in OPCA.

Diagnosis, Differential↗
PubMed1998

Increased cortical inhibition induced by apomorphine in patients with Parkinson's disease.

The electromyographic silent period following the motor potential evoked by cortical magnetic stimulation is decreased in parkinsonian patients. In this study we investigated whether the decrease in the silent period is connected only with parkinsonian symptoms. We evaluated the effect of apomorphine (a potent and rapid dopamine-agonist) on the changes in the peripheral and central silent period in 29 patients with Parkinson's disease and in two patients affected by multisystem atrophy (MSA). Apomorphine injection was found to induce a significant improvement in the central silent period in parkinsonian patients but not in the MSA patients, suggesting a relation between the clinical parkinsonian symptoms (akinesia and rigidity) and the silent period duration. The central silent period changes after apomorphine injection could be used as an adjunctive, safe and effective diagnostic tool to assess dopamine responsiveness of parkinsonian syndromes.

Aged↗
PubMed2003

Severe forward flexion of the trunk in Parkinson's disease: focal myopathy of the paraspinal muscles mimicking camptocormia.

Pronounced forward flexion of the trunk, often termed camptocormia, is a typical symptom of patients with Parkinson's disease. In 4 parkinsonian patients with camptocormia, paraspinal muscles were studied by electromyography (EMG) and axial computerized tomography (CT) or magnetic resonance imaging (MRI) scans and muscle biopsy. EMG of the lumbar and thoracic paravertebral muscles showed abundant fibrillations, positive sharp waves, and bizarre high-frequency discharges. Spinal CT and MRI scans revealed variable degrees of atrophy and fatty replacement of the thoracolumbar paraspinal muscles on both sides. No other signs of neuromuscular disease were found. Biopsy of the paraspinal muscles revealed end-stage myopathy with autophagic vacuoles, chronic inflammatory myopathy, unspecific myopathic changes, or mitochondrial myopathy. In parkinsonian patients with pronounced forward flexion of the trunk, myopathy confined to the erector spinae muscles must be considered.

Aged↗
PubMedDate not supplied

Animal models of shaken baby syndrome: revisiting the pathophysiology of this devastating injury.

To better understand outcomes after early brain injuries, studies must address multiple variables including age at injury, the mechanisms and severity of injury, environmental factors (before and after injury) and developmental factors. Animal models are helpful for elucidating these different aspects. First, this paper describes a new model of shaken baby syndrome (SBS) in mice, without impact or hypoxia. Mortality was 27%; 75% of survivors had focal brain lesions consisting of haemorrhagic or cystic lesions of the white matter, corpus callosum and cerebellum. All shaken animals, with and without focal lesions, showed delayed white matter atrophy. White matter damage and atrophy were reduced by pre-treatment with an NMDA receptor antagonist, indicating that excess glutamate release contributed to the pathophysiology of the lesions. Secondly, it discusses data on neuroprotection after early brain injuries; drugs targeting the NMDA receptors cannot be used in clinical practice but indirect neuroprotection strategies including anti-NO, anti-free radicals and trophic factors hold promise for limiting the excitotoxic white matter damage induced by early injury, in particular caused by shaking, during brain development. Thirdly, it describes two experimental models in which SBS outcomes are determined when the trauma is combined with environmental influences, namely medications during the acute phase, most notably anti-epileptic drugs and rearing conditions.

Animals↗
PubMed1999

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

Dentatorubral and pallidoluysian atrophy (DRPLA) is a member of a family of progressive neurodegenerative diseases caused by polyglutamine repeat expansion. Transgenic mice expressing full-length human atrophin-1 with 65 consecutive glutamines exhibit ataxia, tremors, abnormal movements, seizures, and premature death. These mice accumulate atrophin-1 immunoreactivity and inclusion bodies in the nuclei of multiple populations of neurons. Subcellular fractionation revealed 120 kDa nuclear fragments of mutant atrophin-1, whose abundance increased with age and phenotypic severity. Brains of DRPLA patients contained apparently identical 120 kDa nuclear fragments. By contrast, mice overexpressing atrophin-1 with 26 glutamines were phenotypically normal and did not accumulate the 120 kDa fragments. We conclude that the evolution of neuropathology in DRPLA involves proteolytic processing of mutant atrophin-1 and nuclear accumulation of truncated fragments.

Adolescent↗
PubMed2001

Functional decline in Parkinson disease.

OBJECTIVES: To determine the overall rate of functional decline and to assess the progression of different signs of Parkinson disease (PD). PATIENTS AND METHODS: Patients with clinically diagnosed PD followed up for at least 3 years were included in this study. Demographic and clinical data (including the Unified Parkinson's Disease Rating Scale [UPDRS]) were analyzed by the multivariate unbalanced repeated-measurements design using the mixed-effects model to study the association between different symptoms and various demographic variables. Regression models helped estimate the rates of progression of the disease in relationship to the various components of the UPDRS. Patients were categorized as having tremor-dominant or postural instability-gait difficulty-dominant PD and the 2 categories were compared for progression of their total UPDRS scores. DESIGN: A multivariate mixed-effects model was used to study the relationship between the different symptoms and various demographic variables. Nonparametric statistical tests were used to compare the progression of symptoms in the "on" (good function) state and the "off" (poor function) state groups for 2 age-at-onset categories (< or =57 and >57 years). RESULTS: Data from 1731 visits on 297 patients (181 men) followed up for an average of 6.36 years (range, 3-17 years) were analyzed. The annual rate of decline in the total UPDRS scores was 1.34 when assessed during the on state and 1.58 when assessed during the off state. Patients with an older age at onset had more rapid progression of PD than those with a younger age at onset. Furthermore, the older-onset group had statistically significantly more progression in mentation, freezing, and parts I (mentation) and II (activities of daily living) UPDRS subscores. Handwriting was the only component of the UPDRS score that did not notably deteriorate during the observation period. Regression analysis of 108 patients whose symptoms were rated during their off state showed a faster rate of cognitive decline as age at onset increased. The slopes (ie, the annual rates of decline) of progression in the UPDRS scores, when adjusted for age at the initial visit, were steeper for the postural instability--gait difficulty--dominant group compared with the tremor-dominant group. CONCLUSION: Based on longitudinal follow-up data, our findings provide evidence for a variable course of progression of the different PD symptoms, thus implying different biochemical or degenerative mechanisms for the various clinical features associated with PD.

Adolescent↗
PubMed2001

Synucleinopathies: clinical and pathological implications.

The synucleinopathies are a diverse group of neurodegenerative disorders that share a common pathologic lesion composed of aggregates of insoluble alpha-synuclein protein in selectively vulnerable populations of neurons and glia. Growing evidence links the formation of abnormal filamentous aggregates to the onset and progression of clinical symptoms and the degeneration of affected brain regions in neurodegenerative disorders. These disorders may share an enigmatic symmetry, i.e., missense mutations in the gene encoding for the disease protein (alpha-synuclein) cause familial variants of Parkinson disease as well as its hallmark brain lesions, but the same brain lesions also form from the corresponding wild-type brain protein in the more common sporadic varieties of Parkinson disease. It is likely that clarification of this enigmatic symmetry in 1 form of synucleinopathy will have a profound impact on understanding the mechanisms underlying all these disorders. Furthermore, these efforts will likely lead to novel diagnostic and therapeutic strategies in regard to the synucleinopathies.

Animals↗
PubMed2000

Serum type IV collagen increases with duration of amyotrophic lateral sclerosis.

We analyzed the serum levels of type IV collagen, a major component of the basement membrane, during various stages of sporadic amyotrophic lateral sclerosis (ALS). Serum concentrations of type IV collagen increased significantly with the duration of illness. Findings indicate that the metabolism of type IV collagen and the basement membrane may be affected in the disease process of ALS.

Adult↗