Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Reports of 62 cases with a movement disorder associated with a focal lesion in the thalamus and/or subthalamic region were analyzed. Thirty-three cases had a lesion confined to the thalamus. Sixteen cases had a thalamic lesion extending into the subthalamic region and/or midbrain. Thirteen cases had a lesion in the subthalamic region or a subthalamic lesion extending into the midbrain. Nineteen cases with dystonia, 18 with asterixis, 17 with ballism-chorea, three with paroxysmal dystonia, and five with clonic or myorhythmic movements have been described. No case with isolated tremor has been described. In 53 cases with unilateral thalamic or subthalamic lesions, all but one with bilateral blepharospasm (associated with right posterior thalamic, pontomesencephalic, and bilateral cerebellar lesions) had dyskinesias in the limbs contralateral to the lesion. The other nine cases had bilateral paramedian thalamic lesions; seven developed bilateral dyskinesias, and the remaining two had unilateral dyskinesias. Regarding the 19 patients with dystonia, the two with bilateral blepharospasm had thalamic and upper brainstem lesions, and one with hemidystonia and torticollis had a subthalamic lesion. The other 16 patients all had a unilateral thalamic lesion with contralateral dystonia (10 hemidystonia, five focal dystonia affecting a hand and/or and one segmental dystonia involving face, arm, and hand). The exact location of the thalamic lesion was mentioned in 10 cases; the posterior or posterolateral thalamus was involved in six and the paramedian thalamus in four. These areas are more posterior or medial to the ventrolateral and ventroanterior thalamic nuclei, which receive pallido-thalamic and nigro-thalamic afferents. Two cases developed dystonia immediately after thalamotomy, and one case developed it 4 days after head trauma. The others initially had a hemiplegia and developed dystonia 1-9 months after the acute insult. Fifteen of the 17 patients with chorea had a unilateral lesion in the subthalamic nucleus or subthalamic region (eight due to infarcts, one to hemorrhage, five to mass lesions, and one to multiple sclerosis). All had contralateral hemichorea or hemiballism. One other case had bilateral chorea of the hands and tongue due to paramedian thalamic infarction. Another case with generalized chorea and thalamic atrophy was complicated by stereotaxic surgery. Thirteen of the 18 cases with asterixis had lesions confined to the thalamus. Eight were associated with thalamotomy, and five others had a stroke (four infarction and one hemorrhage) affecting the contralateral thalamus.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Movement disorders following lesions of the thalamus or subthalamic region. 799 Aug 45

Dyskinetic syndromes are conditions with involuntary movements. They can have different causes, but are often due to dysfunction of the basal ganglias. The clinical picture varies but all show spontaneous alterations in intensity as well as deterioration with stress. This often leads to misjudgment of cases of dyskinesia. It is however important to be aware of these syndromes as medical treatment is effective in many cases. The treatment of tremor, tics, chorea, myoclonus, dystonia and medically induced dyskinesia is reviewed and the clinical pictures are briefly described.
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PMID:[Treatment of dyskinetic syndromes]. 799 12

Dyskinetic syndromes are conditions with involuntary movements. They can have different causes, but are often due to dysfunction of the basal ganglias. The clinical picture varies, but all show spontaneous alterations in intensity as well as deterioration with stress. This often leads to misjudgement of cases of dyskinesia. It is however important to be aware of these syndromes as medical treatment is effective in many cases. The treatment of tremor, tics, chorea, myoclonus, dystonia and medically induced dyskinesia is reviewed and the clinical pictures are briefly described.
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PMID:[Treatment of dyskinetic syndromes]. 799 72

Involuntary movements caused by disorders in the central nervous system are peculiar, purposeless movements whose characteristics have been elaborately described in the literature of clinical neurology, although their neural mechanism has not yet been fully clarified. As involuntary movement is the result of abnormal contraction of the skeletal muscles, simultaneous recording from many muscles or muscle groups with different functions furnishes information on characteristic patterns of involuntary movements based on physiological features and provides us with a tool for differential diagnosis and for evaluation of the degree of disorder. For these purposes concurrent examination of muscle tone and of voluntary contraction is desirable. In this article, quantitation of movement and strength by EMG recorded with surface electrodes, characteristic patterns of EMG in various involuntary movements including tremor, chorea, ballism and dystonia were described.
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PMID:[EMG for study of involuntary movements]. 827 64

A variety of inheritable metabolic disorders produce movement disorders. A lists of conditions associated with tremor, athetosis, chorea, dystonia and myoclonus are presented as a guide for the differential diagnosis of such abnormal involuntary movements. The list includes aminoacidopathies, lipidoses, mucopolysaccharidoses, mucolipidoses, organic acidemias, mitochondrial cytopathies and disorders of carbohydrate, purine, and metal metabolism. Clinical, pathological and biochemical features of movement disorders of three typical examples, Wilson's disease, Lesch-Nyhan syndrome and glutaric acidemia type 1, are described.
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PMID:[Movement disorders in miscellaneous disorders--inherited metabolic diseases]. 827 72

Leigh syndrome (LS) is the clinical prototype of a genetically-determined mitochondrial encephalopathy. Twenty-two of 34 patients with LS had evidence of a movement disorder (MD). Dystonia, the most common MD, was present in 19 cases, rigidity in 4, tremor in 2, chorea in 2, hypokinesia in 2, myoclonus in 1, and tics in 1. Dystonia was most commonly multifocal at onset and showed progression in six patients. In half of the cases an enzymatic defect was detected, most commonly cytochrome C oxidase. The neuroradiologic findings showed prominent basal ganglia lesions in 20/21 patients. Putamen, caudate, substantia nigra and globus pallidus were involved in this order of frequency. This experience was reflected in a literature review encompassing 284 cases of LS. However, only 26.4% had MD. Eleven patients, including one of our cases, presented as the primary torsion dystonia phenotype. There are clinical and pathological similarities between LS and other metabolic diseases affecting the central nervous system. The enhanced vulnerability of the nervous system to metabolic stress and the resemblance in the distribution of the pathology of these diverse conditions suggests a common pathogenetic mechanism. An excitotoxin-mediated mechanism is favored, one which might account for the frequent involvement of the basal ganglia in LS.
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PMID:Disorders of movement in Leigh syndrome. 839 42

We reviewed the medical records and videotapes of 100 patients with tardive dyskinesia (TD) referred to our movement disorders clinic to characterize the spectrum of hyperkinetic movement disorders caused by dopamine receptor blocking drugs (DRBD). Tardive stereotypy, present in 78 patients, was the most common type of TD, followed by tardive dystonia, akathisia, tremor, chorea, and myoclonus. Sixty-four had a combination of these hyperkinesias. In a second study, a "blind" review of videotapes of patients with a variety of movement disorders found that DRBD were the cause of stereotypic movements in 89.3% of patients, and 96.1% of patients with TD had stereotypy. We conclude that stereotypy can be readily differentiated from other hyperkinetic movement disorders and that its presence in an adult is highly suggestive of prior exposure to DRBD.
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PMID:Tardive stereotypy and other movement disorders in tardive dyskinesias. 849 49

We studied 53 patients (64% females) with static brain lesions who developed progressive movement disorders. Of these, 50 (94%) had dystonia, 17 (32%) tremor, eight (15%) parkinsonism, seven (13%) myoclonus, and three (6%) chorea. The precipitating insults included perinatal hypoxia/ischemia in 22 (42%), stroke in 12 (23%), head injury in eight (15%), encephalitis in eight (15%), and carbon monoxide poisoning, kernicterus, and radiation necrosis in one patient (2%) each. Among the 30 patients with initial insult occurring at age 2 years or younger (Infant group), distribution of dystonia at follow-up was focal in three (10%), segmental in eight (27%), unilateral in 10 (33%), and generalized in nine (30%). The mean latency between the original injury and onset of movement disorder was 25.5 +/- 16.7 years. Among the nine patients who developed dystonia after an insult occurring between ages 6 and 17 (Childhood group), the distribution of dystonia at follow-up was segmental in two (33%) and unilateral in seven (78%); the mean latency of dystonia onset was 4.9 +/- 7.8 years. Of the 14 patients in the Adult group (injury at age 25 or older), 11 developed dystonia, two developed parkinsonism, and one had carbon monoxide encephalopathy and parkinsonism. The distribution of dystonia in the 11 patients at follow-up was segmental in three (27%) and unilateral in eight (73%). The mean latency of movement disorder onset in the 14 patients of the Adult group was 2.5 +/- 4.9 years. No individuals in the Childhood or Adult groups became left-hand dominant; by comparison, nine of the 30 individuals in the Infant group became left-handed. In conclusion, brain injury at a young age is associated with a longer latency to onset of subsequent movement disorder, a greater tendency to development of generalized dystonia, and a greater probability of altered handedness. These tendencies may result from differences in age-related neuroplasticity.
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PMID:Delayed-onset progressive movement disorders after static brain lesions. 890 76

Regional cerebral glucose metabolism (rCMRGlu-18FDG) was measured in 6 cases with rigid type Parkinson's disease (PD) (2 cases with dopa-induced dyskinesia = DID), 6 cases with chorea (Ch), 5 cases with essential tremor (EssT) and 2 cases with normal subjects (N). The effects of L-Dopa on rCMRGlu was studied in 3 cases with PD. With the aid of depth microrecording study, stereotactic pallidotomy was performed in all cases with PD. Thalamotomy was performed in 3 cases with Ch. In the EssT and N group, the metabolic pattern was high in the frontal cortex (FCx) but low in the lenticular nucleus (LN). In contrast, all cases with a rigid type PD showed lower rCMRGlu in FCx (premotor, prefrontal area). However, 4 out of 6 cases were higher in LN than the control group. Administration of L-Dopa shifted rCMRGlu toward the normal pattern in this group. Five out of 6 cases with Ch represented higher rCMRGlu in FCx (3 focal, 2 diffuse) but lower in LN. Moreover, when DID occurred, it showed almost the same pattern as in Ch. Electrophysiological studies showed high background neuronal activity (BNA) in the medial segment of the globus pallidus (GP) but low BNA in the lateral segment of the GP in the rigid type of PD. In cases with Ch, irregular burst discharges were often encountered in ventro-oral thalamus. From these results, the on-going changes of basal ganglia-thalamocortical motor circuit in cases with a rigid type PD, DID and Ch are discussed. The underlying mechanisms of Parkinsonian rigidity was considered to contrast with those of DID and Ch within the same motor circuit.
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PMID:Parkinsonian rigidity, dopa-induced dyskinesia and chorea--dynamic studies on the basal ganglia-thalamocortical motor circuit using PET scan and depth microrecording. 874 74

Chronic acquired hepatocerebral degeneration (CAHD) is a heterogeneous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Little has been added to the understanding of this disorder since the detailed, early clinical and pathological descriptions. The spectrum of clinical presentations can be neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, parkinsonism, myoclonus, dystonia), or both. Cortical laminar necrosis and polymicrocavitation in the cortex and basal ganglia are combined with cerebral and cerebellar atrophy. Microscopically, Alzheimer type II astrocytes and cytoplasmic glycogen granules are characteristic. Recent neuroradiological observations in patients with liver failure have shown a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the pallidum, putamen, and, rarely, mesencephalon. Using clues from a similar MR appearance in patients receiving total parenteral nutrition as well as animals given parenteral manganese, and the knowledge that manganese is cleared by the hepatobiliary system, deposition of manganese in the brain is postulated in patients with CAHD. In this review we describe three cases of CAHD with detailed clinical and radiological documentation and discuss the aforementioned pathogenetic mechanisms.
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PMID:Chronic acquired hepatocerebral degeneration: case reports and new insights. 886 9


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