The authors used 1H-MRS to investigate hypothalamic metabolism in 26 patients with cluster headache (CH) and 12 healthy subjects. Hypothalamic N-acetylaspartate/creatine was reduced in patients with CH vs controls (p < 0.01). Dividing the patients into episodic CH outside- and in-cluster periods and chronic CH, the hypothalamic N-acetylaspartate/creatine in all three subgroups of patients was reduced. The reduction of the neuronal marker N-acetylaspartate is consistent with hypothalamic neuronal dysfunction in patients with CH.
Summary:Purpose: Hypohidrosis during topiramate (TPM) treatment was recently reported in children. We describe an adult epilepsy patient who developed inability to sweat as well as heat intolerance while undergoing treatment with TPM.Methods: To detect the site of the sweat block, patient underwent examination of sweat gland function, cardiovascular autonomic test, and body temperature rhythm determination.Results: During TPM treatment, cardiovascular autonomic function and circadian rhythm of body core temperature were normal, whereas thermoregulatory sweat test (TST) showed anhydrosis. This adverse drug effect was quickly resolved after drug discontinuation.Conclusions: Because of normal cardiovascular autonomic function and central and peripheral thermoregulatory mechanisms, we hypothesize that hypohidrosis during TPM treatment could be due to a carbonic anhydrases (CA) block at the level of sweat gland.
We present a questionnaire-based survey on methods adopted in European autonomic nervous system (ANS) laboratories in the year 2002. Seventy-nine laboratories from 16 countries in Western and Eastern Europe provided information on test methods, equipment, staff, educational programme and research activities. The results confirmed the anticipated heterogeneity of techniques used to investigate ANS disorders. However, cardiovascular (84.8%) and sudomotor (43.0%) tests were the most common. There were no standards regarding equipment, but devices used varied widely, in part commercially available, in part self-developed. Some ANS laboratories performed cardiovascular tests using standard EMG devices with implemented routines to measure heart rate variability. Many investigators used published normative data (75.4%). Most of the responders were involved in scientific work, half were active in education of trainees. A wide variation was found in number and profession of staff members. As more than 60% of questionnaires were returned by neurologists our results may be limited to neurological ANS laboratories. Given the wide variation of methods and equipment used in European ANS laboratories, minimal standards for routine ANS testing are desirable. Standards may help to compare results of diagnostic tests, evaluate equipment and test laboratory methods and commercial devices and set-up educational programmes.
The sympathetic skin response (SSR) detects changes in the electrical potential in the skin in response to physiological and electrical stimuli and, therefore, may indicate the integrity of sympathetic cholinergic neural pathways to sweat glands. This has been evaluated in 21 patients with three forms of peripheral autonomic failure. Of these, 15 had pure autonomic failure (PAF) without additional neurological features; investigations indicated both sympathetic and parasympathetic failure. Four patients had pure cholinergic dysautonomia (PCD), with clinical and laboratory features indicating only cholinergic failure. Two siblings had dopamine-beta-hydroxylase (DBH) deficiency with only sympathetic adrenergic failure. None was on drugs affecting cholinergic function. Ten normal individuals were aged-matched with PAF patients and studied as controls. The SSR was recorded from the palmar hand and plantar foot surfaces, using previously described techniques, in response to physiological (auditory, cough and inspiratory gasp) and electrical stimuli. Nerve conduction studies excluded an associated motor or sensory neuropathy. The SSR was present in all normal individuals, and in both patients with DBH deficiency who had preserved cholinergic and sudomotor function, It was absent in all 15 PAF and all four PCD patients with impaired cholinergic function. Therefore, we conclude that the SSR reflected sympathetic cholinergic function in these three different groups with peripheral autonomic failure.
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