A 73-year-old woman with confusion, rigidity, and fever.
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A total of 150 patients, predominantly children, with torsion dystonia++ (TD) were investigated. Their clinical syndromes, pathogenesis and results of treatment were analyzed. Prominent features were interfamilial (true heterogeneity) and intrafamilial clinical polymorphism of the disease. Autosome-dominant++ type of inheritance was found in 15 families and the autosome-recessive in 10. With respect to the clinical syndrome, 2 forms of TD were singled out: dystonic-hyperkinetic++ and rigid. These were different in the neurotransmitter metabolism. Long-term follow-up of the patients (2 to 20 years) proved a high efficiency of treatment with low doses of DOPA-containing drugs in rigid forms of TD.
A 70 year old man recovered from a left putamenal haemorrhage in 1984 with a residual mild right hemiparesis. In 1985 he presented following the sudden onset of inability to stand. The clinical findings of supranuclear ophthalmoplegia, bradykinesia and rigidity resembled those of progressive supranuclear palsy. CT scan revealed a recent haemorrhage deep in the right hemisphere white matter in addition to a low density change in the left basal ganglia reflecting the site of previous haemorrhage. The patient's course was uncomplicated and the ophthalmoplegia resolved. Bradykinesia and rigidity persist. This case illustrates an unusual clinical presentation of bilateral intracerebral haemorrhage with supranuclear ophthalmoplegia, bradykinesia and rigidity.
Rigidity, tremor, akinesia and disorder of postural reflex are the main clinical features of Parkinson's disease. We presented the mechanism underlying rigidity and assessed central motor conduction time (CMCT) using magnetic, with or without vibratory, stimulations. Basal ganglia, especially, the internal pallidum, and the thalamus play major roles in the mechanism of rigidity in Parkinson's disease. Hyperexcitability of the spinal motor nucleus due to low threshold has been recognized. Magnetic stimulation is painless and is simpler than electric stimulation. Therefore, this method is used clinically for evaluating conduction disturbance of the upper motor neurons in multiple sclerosis, cerebrovascular disease and so on. CMCT measured by magnetic and/or electric stimulation may be abbreviated or normal in Parkinson's disease, according to the literature, though controversy persists in this regard. In our study, CMCT was normal in Parkinson patients. However, CMCT was reduced in patients with rigidity and tremor. Furthermore, in a portion of the patients, CMCT was further abbreviated by also applying vibratory stimulation. These observations support the hypothesis that cells in the thalamus, cortex and spinal cord and/or pathways in these portions of the central nervous system are excitable or activated in Parkinson patients with rigidity and tremor. However, elucidation of the mechanisms underlying rigidity and tremor awaits further investigation.
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