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Query: UMLS:C0155339 (Brown)
 12,436 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Motor disorders reported in the present paper do not result from cortical ablations stricto sensu since some white matter was excised in every patient. However they appear to suggest that, as suggested by Walshe (1935), the central region and premotor area are a functional entity, i.e. they work as a whole. The extensive lesions of the premotor area, leaving untouched the motor region, have the same motor and tonic consequences as lesions limited to the central region. This point which appears specific for man does not imply that the premotor region subserves activities similar to those subserved by the central region. Rather it may suggest a deafferentiation of the central region, the consequences of which would be more important than is generally assumed. Extensive central or premotor lesions determine various tonic disorders: a well known spasticity, with exaggeration of the stretch reflex, associated with an increase in passive swinging of segments of limbs and in extensibility of joints. These two latter phenomena are usually defined as hypotonia. With premotor and precentral lesions the hypotonia disappears and a hemiplegic posture is observed. This hemiplegic posture is a dystonia which apparently does not result directly from the exaggeration of the stretch reflex. Anatomically it appears to result from lesions of both central and premotor regions. This is in agreement with Denny-Brown's (1966) contention that an extrapyramidal region lies rostral to the prerolandic sulcus. As suggested by Evarts (1973) motor regions appear to control automatic as well as voluntary movements. They probably play a role in the trophic function of muscle, since, despite rehabilitation, amyotrophy was present in every case reported in the present paper.
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PMID:Motor consequences of motor area ablations in man. 83 9

Four pedigrees of Machado-Joseph disease (MJD) were reported. Main clinical features of 21 patients in these pedigrees were cerebellar ataxia, limb spasticity, gaze nystagmus, facio-lingual twitchings, and external ophthalmoparesis. Amyotrophy, hypokinesia, or dystonia were manifested with advance of the illness. In patients with younger onset age, such extrapyramidal signs were dominated. Neuropathological study of one autopsied case disclosed that there were degeneration of spinocerebellar tract, anterior horn cells, pontine nuclei, dentate nucleus, red nucleus, substantia nigra, internal segment of globus pallidus, subthalamic nucleus, and motor nuclei of brain stem; neurons of cerebellar cortex and inferior olivary nucleus were preserved. From these clinical and pathological features, these 4 pedigrees satisfied the criteria of MJD, and were differentiated from hereditary olivopontocerebellar atrophy. Currently, MJD is accepted as a new entity of hereditary spinocerebellar ataxias. However, there are still controversies as to whether Azores-Portuguese MJD and Japanese MJD are identical disorder. Furthermore, the nosological relationship between MJD and a number of similar cases, as reported historically under the diagnosis of Brown type ataxia or Marie's ataxia, has not been clearly established. From reviewing such cases critically, pathological and clinical features of our cases are so similar to those of the latter, indicating that the probably identical genetic disorder has been classified under the different categories.
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PMID:[Clinicopathological study of Joseph disease: report of 4 pedigrees and its nosological consideration]. 159

Cramp syndromes pose a challenge for neuroscientists. The motor disorders of Isaacs syndrome have been ascribed to peripheral neuropathy, and sometimes there is ample supporting evidence of neuropathy. However, signs of overt neuropathy are found in a minority of cases and the essential findings (carpal and pedal spasm, pseudomyotonia and myokymia) may arise from abnormal excitability of the perikaryon because similar manifestations are seen in tetany and multiple sclerosis. The Moersch-Woltman (stiffman) syndrome differs from Isaacs' syndrome in essential characteristics. Hyperventilation syndromes may mimic either simple cramps, the Isaacs syndrome, the Moersch-Woltman syndrome, or the Foley and Denny-Brown syndrome of benign fasciculation and cramps. New approaches are needed to define the etiology and pathogenesis of these neurogenic disorders because the results of peripheral nerve block and spinal anesthesia have not been consistent in cases of typical Isaacs syndrome. Occupational cramps can be regarded as a form of action dystonia but that statement is a clue, not an "explanation". Myopathic disorders are only rarely a cause of cramp syndromes. In the glycogen storage disorders, the chemical basis of the cramp is still unproven. Whether myoadenylate deaminase is a cause of cramps is debated.
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PMID:Cramps, spasms and muscle stiffness. 299 4

Haloperidol induced catalepsy with a continuous dose-response between 0 or 0.03 and 10 mg/kg, IP in Brown Norway (BN), Fischer (FR). Long-Evans (LE), and Sprague-Dawley (SD) rats, with similar ED50 values for males (0.23-0.42 mg/kg), and generally greater but more varied drug sensitivity in females (ED50 = 0.13-0.45 mg/kg). Inconsistent findings regarding a biphasic dose-effect relationship in SD male and LE female rats led to testing both sexes and strains up to 200 mg/kg. Females showed a greater tendency than males of both strains to yield lower catalepsy scores at doses greater than 10 mg/kg, but in association with gross neurological toxicity. This paradigm evidently is not an adequate model for a proposed biphasic relationship between doses of high potency neuroleptic agents and acute dystonia in man.
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PMID:Dose-catalepsy response to haloperidol in rat: effects of strain and sex. 320 87

This study was conducted to investigate the degree of accuracy with which three groups of listeners could use perceptual analysis alone for identification of specific dysarthria types. The dysarthria types to be identified in this study were identical to those described by Darley, Aronson, and Brown (1969a): Flaccid, Spastic, Ataxic, Hypokinetic, Hyperkinetic Chorea, Hyperkinetic Dystonia, and Mixed. Each group demonstrated minimal success in the accurate identification of specific dysarthria types. Factors that possibly contributed to this poor success and potential implications are discussed.
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PMID:Identification of dysarthria types based on perceptual analysis. 365 12

We review Denny-Brown's hypotheses on the mechanisms underlying motor behavior and his views on how dysfunction in these mechanisms can result in dystonia. His formulations were based upon clinical observations of patients with diseases of the central nervous system and on monkeys with surgically-induced lesions of structures affected by diseases in his patients. Denny-Brown viewed dystonia as resulting from an imbalance of reflex responses to natural stimulation, both tonically and phasically. The evidence for this view came from demonstration of alterations of dystonic postures by natural vestibular, cutaneous, proprioceptive and visual stimuli. His formulations remain highly useful to clinicians faced with a bewildering variety of postural and movement disorders.
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PMID:Denny-Brown's views on the pathophysiology of dystonia. 1052 55

The occurrence of cognitive and behavioral symptoms in patients with primary dystonia remains a matter of debate. We compared 45 patients with primary dystonia with 27 control subjects for performance on neuropsychological tasks with a load on executive-Wisconsin Card Sorting Test (WCST) and Stroop test, and visuospatial-Benton's visual retention test (BVRT) and Block assembly test from Wechsler Adult Intelligence Scale BAT-functions, as well as for intensity of obsessive-compulsive symptoms (Yale Brown Obsessive Compulsive Scale, Y-BOCS). Correlation analysis was performed between neuropsychological performance, dystonia characteristics (duration, age of onset) and severity (Unified Dystonia Rating Scale, UDRS), and Y-BOCS. Patients made more perseverative errors on the WCST (P = 0.042) and had a higher mean Y-BOCS (P = 0.003) score than controls. Timed tests (BVRT, BAT, Stroop test) correlated with UDRS. Y-BOCS, WCST, and UDRS scores were not significantly correlated with one another.These results suggest that patients with primary dystonia may have set-shifting deficits and a higher intensity of obsessive compulsive symptoms when compared to healthy subjects. This may reflect a pattern of complex neurophysiological dysfunction involving dorsolateral, orbitofrontal, and motor frontostriatal circuits.
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PMID:Set-shifting and behavioral dysfunction in primary focal dystonia. 1804 8

In a single-center, case-control study, we investigated the frequency and types of psychiatric disturbances in 89 consecutive patients with various primary focal dystonias (34 had cervical dystonia (CD), 28 blepharospasm (BPS), 16 laryngeal dystonia (LD), and 11 arm dystonia), 62 healthy control subjects and as controls for BPS, 26 patients with hemifacial spasm (HFS). Patients and controls underwent a full psychiatric evaluation. Diagnosis was based on the structured clinical interview for DSM-IV, obsessive-compulsive disorder (OCD) was assessed with the Yale-Brown Obsessive-Compulsive scale, anxiety with the Hamilton Rating Scale for Anxiety, the severity of depression with the Beck Depression Inventory. Of the 89 patients with focal dystonias studied, 51 patients (57.3%) had a diagnosis of psychiatric disorders compared with only 15 of 62 healthy subjects (24.1%) and 9 of the patients with HFS (34.6%). Depressive disorders were more frequent in the CD and BPS groups than in healthy controls, whereas the frequency of anxiety disorders, OCDs or adjustment disorders approached that of healthy subjects. No difference was found in the frequency of any specific psychiatric disorder in patients with LD and arm dystonia and healthy controls. In 35 of 51 patients who had psychiatric disorders, these started before and in 16 patients after the onset of dystonia. No differences were found in age, dystonia severity, and duration of botulinum toxin treatment between patients with and without psychiatric disturbances. The most common psychiatric features in patients with CD and BPS are depressive disorders.
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PMID:Psychiatric disorders in adult-onset focal dystonia: a case-control study. 2153 12

Primary focal dystonia is an idiopathic neurological disorder causing involuntary muscle contraction. Its pathophysiology probably involves the basal ganglia and cortical-basal pathways. Primary dystonia appears to be associated with significant obsessive-compulsive symptoms, but evidence remains scarce and contradictory. We addressed the following research questions: (1) Do primary dystonia patients have high obsessive-compulsive symptom scores? (2) Are these symptoms more severe in dystonia than in controls with equivalent peripheral neurological disorders? and (3) Is psychopathology different in botulinum toxin-treated and -untreated dystonia patients? This work was a cross-sectional, descriptive, controlled study comprising 45 consecutive patients with primary focal dystonia (i.e., blepharospasm, spasmodic torticollis, or writer's cramp) 46 consecutive patients with hemifacial spasm, cervical spondylarthropathy, or carpal tunnel syndrome, and 30 healthy volunteers. Assessment included the DSM-IV based psychiatric interview, Symptom Checklist 90R, Yale-Brown Obsessive-Compulsive Scale and Checklist, and the Unified Dystonia Rating Scale. Dystonia patients had higher Yale-Brown Obsessive-Compulsive Symptom scores than both control groups. Dystonia patients with obsessive-compulsive symptom scores above cut-off for clinical significance predominantly developed hygiene-related symptoms. Major depression and generalized anxiety disorder were the most frequent psychiatric diagnoses in primary focal dystonia. Obsessive-compulsive disorder frequency was 6.7%. Primary focal dystonia patients have higher obsessive-compulsive symptom scores than individuals with similar functional disabilities resulting from other neurological disorders, suggesting that obsessive-compulsive symptoms in dystonia are not reactive to chronic disability. Dystonic muscle contractions and obsessive-compulsive symptoms may share a common neurobiological basis related to cortical-basal dysfunction. Psychopathology, especially obsessive-compulsive symptoms, should be actively explored and treated in primary focal dystonia.
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PMID:Obsessive-compulsive symptoms in primary focal dystonia: a controlled study. 2183 Feb 32

This study is aimed at investigating obsessive-compulsive disorder (OCD) in three groups of patients matched for age and gender; namely, focal dystonia (FD), hemifacial spasm (HFS), and healthy-control subjects (HC). All subjects were investigated with the Structured Clinical Interview for DSM-I, the Hospital Anxiety and Depression Scale, the Symptom Checklist-90, the Yale-Brown Obsessive-Compulsive Scale, and the Structured Clinical Interview for Obsessive-Compulsive Spectrum Self-Report, Lifetime Version (SCI-OBS-SR-LT). The prevalence of OCD was significantly higher in both FD and HFS than in HC participants. On the SCI-OBS, HFS patients showed higher scores than FD or HC for "contamination" and "aggressiveness." Despite the different pathophysiology, OCD is highly represented in both FD and HFS, with different thematic content characterizing the two conditions.
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PMID:Obsessive-compulsive-spectrum symptoms in patients with focal dystonia, hemifacial spasm, and healthy subjects. 2245 Jun 17


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