Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026837 (muscle rigidity)
 1,077 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 4.9-year-old boy with congenital hydrocephalus and obstruction of his shunt who just after the induction of anaesthesia suddenly developed generalized muscle rigidity that made intubation impossible. Because of temporary mydriasis the situation could hardly be differentiated from acute cerebral herniation. The lack of any decrease in muscle tone after emergency trephination and drainage of the right lateral ventricle and the immediate improvement following intravenous dantrolene left no doubt about the diagnosis of malignant hyperthermia. The diagnosis was confirmed by the increase in serum creatine phosphokinase and the documentation of massive myoglobinuria. Reconstruction of the course of the first anaesthetic necessary for shunt implantation at the age of 62 days revealed that the same symptoms already had occurred. However, they then were not attributed to malignant hyperthermia but interpreted as symptoms of acute herniation. A detailed description of this first anaesthetic is given which again elucidates the problems associated with the abrupt onset of muscular hypertonus in a patient with neurologic disorder; moreover this may well be the first published case report of malignant hyperthermia at the age of just 2 months.
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PMID:[Symptoms of acute cerebral hernia following induction of anesthesia in hydrocephalus--malignant hyperthermia in reality]. 378 92

GAIT ARRESTS: They affect the evolution of the disease. This freezing phenomenon which induces falls sometimes constitutes an initial sign. Like the gait initiation failure, freezing can be controlled by sensory stimulation, notably visual inputs, but also by more sustained attention. FALLS ARE MAINLY CONNECTED WITH BOTH POSTURAL INSTABILITY AND RIGIDITY: They are poorly influenced by dopaminergic therapies. The progressive decrease of step width represents a main factor in their occurrence. PRECOCITY OF GAIT DISORDERS IS UNUSUAL IN PARKINSON'S DISEASE: Other parkinsonian syndromes such as progressive supranuclear palsy, multiple system atrophy and vascular parkinsonian syndrome must then be evoked. Their association with a cognitive impairment and abnormal sphincter behaviour infers a diagnosis of normal pressure hydrocephalus. GAIT IMPROVES WITH L-DOPA THERAPY: Speed, step length and duration of the swing phase are increased without change of cadence. Progressive loss of L-dopa efficiency on gait and postural stability contrasts with the persistent effect on tremor, rigidity and bradykinesia; a functional abnormality of nondopaminergic systems can explain these symptoms. In the following stages, gait troubles increased by motor fluctuations and abnormal involuntary movements are less controlled by L-dopa therapy. PHYSICAL THERAPY PLAYS A MAJOR ROLE IN THERAPEUTIC MANAGEMENT: An individual or collective rehabilitation project must be established according to the stage of evolution; the exercises aim to protect postural control and coordination. Visual or sound rhythmic inputs can be employed in the case of gait initiation failure. THE EFFECTS OF FUNCTIONAL NEUROSURGERY ARE IN THE COURSE OF EVALUATION: Thermolesion and chronic electrical stimulation of deep brain structures have opposite effects on gait troubles. Bilateral thalamotomy or pallidotomy are sometimes a source of disequilibrium. Chronic thalamic stimulation does not induce either benefits or adverse effects. On the other hand, stimulation of the internal pallidum improves gait kinematic parameters; improved postural adjustments have also been reported. The effect of subthalamic nucleus stimulation is comparable to that of L-dopa, however the long-term effect remains to be evaluated.
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PMID:[Gait disorders in Parkinson disease. Gait freezing and falls: therapeutic management]. 1128 86