Clonidine, a centrally active alpha 2-adrenoreceptor agonist used to lower blood pressure, has been proposed to differentiate central from peripheral autonomic deficits and multiple system atrophy (MSA) from untreated idiopathic Parkinson's disease (IPD). A lack of growth hormone (GH) increase after clonidine infusion is found in patients with MSA, but not in those with IPD or with pure autonomic failure. We studied 19 IPD and 7 MSA patients to assess whether this test could be used in clinical practice to distinguish MSA from IPD, whatever the stage of the disease. Serum GH levels were measured 15, 30, 45 and 60 min after a 10-min infusion of 2 micrograms/kg clonidine. GH levels remained stable after clonidine infusion in all 7 MSA patients but increased in only 12 of the 19 IPD patients, while remaining stable in the other 7. No correlation was found with the presence of orthostatic hypotension. We conclude that the GH response to clonidine infusion has a very high sensitivity (100% in our series and in previous studies) for the diagnosis of MSA. However, this response cannot be used as a diagnostic test because of its poor specificity.
Expression of a mutant superoxide dismutase 1 (SOD1) gene in transgenic mice induces a gradual degeneration of cholinergic motor neurons in the spinal cord, causing progressive muscle weakness and hindlimb paralysis. Transgenic mice over-expressing the human SOD1 gene containing a Gly-->Ala substitution at position 93 (G93A) were employed to explore the effects of the SOD1 mutation on choline acetyltransferase (ChAT) expression in the striatum, and in the lumbar and cervical spinal cord. These mice showed a progressive loss of their spinal cord motor neurons, and at 130 days of age showed an up-regulation of ChAT mRNA expression in the striatum. On the other hand, ChAT mRNA decreased in cervical and lumbar motor neurons. These findings suggest that cholinergic interneurons in striatum in SOD1 transgenic mice are over-activated in an attempt to compensate for the death of spinal motor neurons.
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