Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of transient post-varicella lingual-mandibular dystonia is presented. This case was compared with the eight previously reported instances of involuntary movement disorders which rarely follow varicella infection.
J Neurol Neurosurg Psychiatry 1987 Sep
PMID:Transient dystonia as a complication of varicella. 366 73

The use of baclofen, a structural analog of gamma-aminobutyric acid (GABA), is described in the treatment of a patient with tardive dystonia. The patient, a woman with a clinical diagnosis of Alzheimer's disease, developed tardive dystonia after 8 weeks of haloperidol therapy and experienced complete remission of her dystonia while taking baclofen 60 mg/day. This case suggests that baclofen may facilitate remission of tardive dystonia in some cases and provides a basis for further investigation.
J Clin Psychiatry 1986 Sep
PMID:Baclofen treatment in a patient with tardive dystonia. 374 31

In five patients with parkinsonism, the optimal dosage of a controlled-release levodopa/carbidopa preparation (CR-3) was three times higher than the dosage of Sinemet and produced higher plasma levodopa concentrations, but did not reduce the fluctuations in plasma levodopa or clinical response. Plasma levodopa concentrations were higher and clinical responses better before the first dose of the day with CR-3. CR-3 treatment benefited two patients, reducing the severity of off periods and off dystonia. Two patients were worse on CR-3 despite higher plasma levodopa levels than those adequate for clinical response to Sinemet or levodopa infusions. CR-3 could benefit a few severely affected patients, but it is necessary to understand the factors that affect absorption of levodopa from sustained-release preparations, as well as the consequences of prolonged elevation of plasma levodopa levels.
Neurology 1986 Sep
PMID:Clinical and biochemical studies with controlled-release levodopa/carbidopa. 374 87

In a 5-year-old boy, an early onset psychomotor retardation with non-progressive ataxia and without dysmorphic features, associated with lysosomal storage disease found on ultrastructural examination of the conjunctiva, led to the diagnosis of Salla disease. This was supported by a tenfold excretion of urinary free sialic acid, without abnormal oligosacchariduria or anomaly in lysosomal enzymes. This boy is a native of Southern France. Screening of urinary sialic acid has to be introduced in aetiological investigations of patients with apparently non-progressive psychomotor retardation associated with ataxia or dystonic movements.
Eur J Pediatr 1986 Sep
PMID:Salla disease in one non-Finnish patient. 377 5

Clinical observations show a close relationship between neuralgia of the superior laryngeal nerve and disorders of the larynx. Neuralgia, and more minor symptoms are usually caused by hyper- and hypotonic phonatory disorders. An unphysiological compensation for glottic insufficiency causes irritation of the sensory telodendrons of the superior laryngeal nerve. As incomplete adduction of the vocal cords can often be found in patients with an autonomic laryngeal dystonia, a syndrome related to anxiety, these disturbances are often misinterpreted as "globus hystericus". However, this diagnosis does not take into account the cause and should therefore no longer be used.
HNO 1986 Sep
PMID:[Neuralgia of the superior laryngeal nerve caused by phonatory malfunctions]. 377 Dec 97

Four patients with primary writing tremor had a focal, task-specific tremor that responded to anticholinergic drugs. Physiologic features included EMG activity alternating in antagonist muscles, 5- to 20-microV cerebral potentials evoked by stretch of pronator teres, and no C-reflexes. Another patient had myoclonic jerks of the forearm on attempts to write ("myoclonic writer's cramp") that also responded to anticholinergic drugs; EMG activity appeared synchronously or alternating in antagonist muscles. These disorders have features of dystonia and enlarge the spectrum of writer's cramp.
Neurology 1985 Sep
PMID:Primary writing tremor and myoclonic writer's cramp. 402 90

The pathophysiology of reflexes mediated by the fifth and seventh cranial nerves has been studied in 16 patients with blepharospasm and oromandibular dystonia compared with normal age-matched subjects. The EMG activity of the dystonic spasms in the periocular and jaw muscles was similar to that described in other muscles in patients with generalized torsion dystonia. The latency of the R1 and R2 components of the blink reflex and of the corneal reflex was normal. However, the amplitude and the duration of the R1 and R2 and the duration of the corneal reflex were increased. In some patients the R1 component was also present on the side contralateral to the stimulus, while in normal subjects it was present only on the ipsilateral side. The excitability cycle of recovery of the R2 component of the blink reflex after a prior conditioning shock was enhanced in the patients. There were no EEG potentials preceding blepharospasms in the patients, although a Bereitschaftspotential was seen beginning some 500 ms prior to voluntary blinks in the same individuals. Exteroceptive suppression in the contracting masseter and orbicularis oculi muscles was absent in 40 to 50 per cent of the patients. The jaw jerk was present in all the patients with normal latency. These results indicate that the neuronal arcs of the facial reflexes in blepharospasm and oromandibular dystonia are normal. However, there is an abnormal excitatory drive, perhaps from the basal ganglia, to the facial motoneurons and the interneurons which mediate the facial reflexes in the brainstem.
Brain 1985 Sep
PMID:Pathophysiology of blepharospasm and oromandibular dystonia. 404 76

Beside the importance of its prehensile function the human hand plays an essential role in the conveyance of expression. Manual gestures support the mimic and confirm the spoken word. Based on the psychofunctional connection between mimic and gesture the hand becomes an outlet for psychogenic disorders. The resultant projection of emotional processes into motor disorders has been a matter of conjecture etiologically. Psychogenic manifestation in isolated motor disorders of the hand is considered to be either a functional nervous disorder or a focal dystonia. In comparison to the conversion phenomena reported up until now, in which flexor muscle units of the hand are involved with increased muscular activity (spasm), the cases presented here are characterized by a functional motor disorder manifested as an isolated paralysis of a selective extensor muscle unit (M. extensor pollicis longus) of the hand. After a multidisciplinary approach including clinical morphology, neurology, EMG and psychiatry the final diagnosis of a psychogenic paralysis of the hand is made using direct electro-stimulation of the neuromuscular unit with a needle electrode under simultaneous elimination of the psychic influence (general anesthesia without muscular relaxation). Through such confirmed motor inactivity the psychic genesis of the clinically existing paralysis is proven. Through immediate introduction of a multimodal therapy including physical and psychiatric treatment psychogenic paralyses of the hand can be restored totally.
Handchir Mikrochir Plast Chir 1985 Sep
PMID:[Diagnosis and therapy of psychogenic paralyses in the hand region]. 406 16

The spontaneous occurrnce of blepharospasm and dystonic movements in face muscles, particularly those of the perioral and mandibular regions, has been named Meige's disease. Other dystonic features as spasmodic torticollis, dysphagia, spasmodic dysphonia and segmental dystonia of the limbs may, eventually, be present in the same patient. There is very little knowledge about the pathology of this disease. Many hypotheses concerning the pathophysiology of this entity have been put forward, most of them correlating the clinical response to several drugs with known action on the neurotransmitter system of the brain. There are some evidences that it may exist a dopaminergic preponderance in the disease. In the nigro-striatal pathway, one of the retrograde loops in the feed-back control of dopamine synthesis by nigral neurons is dependent on GABA. Increasing GABA activity through GABA agonists that cross the blood-brain barrier could result in a decreased dopaminergic action in the nigro-striatal pathway and, thus, ameliorate the dystonic symptoms which might have been produced by its increased function. We have used baclofen, a GABA-agonist drug, to treat five patients with Meige's disease, in a single-blinded trial. These were four females and one male, with age ranging from 50 to 63 years. The drug was started at 20mg/day, being increased by 10mg each three days reaching a maximum dose of 70mg/day. One of the patients showed marked improvement of blepharospasm and orofacial dystonia and a second patient had a moderate improvement in the same symptoms. Another patient showed moderate improvement of limb dystonia, but had no benefit in the facial movements.(ABSTRACT TRUNCATED AT 250 WORDS)
Arq Neuropsiquiatr 1985 Sep
PMID:[Treatment of Meige disease with a GABA receptor agonist]. 409 37

The neurological mouse mutant dystonia musculorum exhibits bizarre appendicular and truncal dystonia without known cerebellar histopathology. We evaluated striatal dopamine and cerebellar norepinephrine metabolism in this mutant and compared the results with those obtained in wild-type BALB/c and B6C3 controls. Tyrosine hydroxylase activity and dopamine metabolite levels (homovanillic acid and 3,4-dihydroxyphenylacetic acid) in the striatum of the mutant were similar to controls. Tyrosine hydroxylase activity and the steady-state level of 3-methoxy-4-hydroxyphenethyleneglycol, a metabolite of norepinephrine, in the cerebellum were 38% and 42-66%, respectively, greater in the mutant. However, the level of norepinephrine was similar (approximately 350 ng/g). Further, a Purkinje cell-specific marker, cGMP-dependent protein kinase, was unchanged in the mutant and no Purkinje cell pathology was observed with light microscopy. The lack of Purkinje cell derangement and similar levels of cerebellar norepinephrine and cGMP-dependent protein kinase activity suggest that increased norepinephrine metabolism in the cerebellum of this mutant is not a morphological response to gross target cell loss during morphogenesis. The observed changes may be a reaction to abnormal impulse traffic or altered input/output pathways to the mutant cerebellum during its development.
J Neurochem 1981 Sep
PMID:Increased noradrenergic metabolism in the cerebellum of the mouse mutant dystonia musculorum. 611 89


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>