Summary
Somatostatin, substance P, cyclic AMP and cyclic GMP were determined in the cerebrospinal fluid of patients with Huntington's disease, in first generation relatives of choreic patients and in neurological control patients. Substance P levels were not significantly altered, but somatostatin levels were markedly decreased both in affected patients and symptom-free offspring. Cyclic AMP was decreased only in patients with advanced stages of the disease while cyclic GMP was normal. Evidence is discussed which may support a role of somatostatin deficiency in the pathophysiology of chorea.
Zusammenfassung
Bei 12 Patienten mit Chorea Huntington und 7 Nachkommen aus Chorea-Familien wurden im lumbalen Liquor die Konzentrationen von Somatostatin, Substanz P, zyklischem Adenosinmonophosphat und zyklischem Guanosinmonophosphat radioimmunologisch bestimmt und mit Werten neurologischer Kontrollpatienten verglichen. Während die Konzentration von Substanz P sich in allen Gruppen nicht signifikant voneinander unterschieden, waren die Konzentrationen von Somatostatin bei Patienten mit manifester Chorea und bei den Nachkommen hochsignifikant vermindert. Zyklisches Adenosinmonophosphat war nur bei weit fortgeschrittener Chorea signifikant herabgesetzt. Mögliche Beziehungen der Somatostatin-Verminderung zur Pathophysiologie der Chorea und der Verminderung des zyklischen Nukleotids zur Hirnatrophie werden diskutiert.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Arimura A, Sato H, Coy DH, Schally AV (1975) Radioimmunoassay for GH-release inhibiting hormone. Proc Soc Exper Biol 148:784–789
Berelowitz M, Hudson A, Pimstone BL, Kronheim S, Bennett GW (1978) Subcellular localization of growth hormone release inhibiting hormone in rat hypothalamus, cerebral cortex, striatum and thalamus. J Neurochem 31:751–753
Biggio G, Guidotti A (1977) Regulation of cyclic GMP in cerebellum by a striatal dopaminergic mechanism. Nature (Lond) 265:240–242
Bird ED, Iversen LL (1974) Huntington's chorea—post mortem measurement of glutamic acid decarboxylase, choline acetyltransferase and dopamine in basal ganglia. Brain 97:457–472
Cailla HL, Vannier CJ, Delaage MA (1976) Guanosine 3′,5′-cyclic monophosphate assay at the 10−15 mole level. Anal Biochem 70:195–202
Cramer H, Ng LKY, Chase TN (1973) Adenosine 3′,5′-monophosphate in cerebrospinal fluid. Effect of drugs and neurologic disease. Arch Neurol (Chic) 29:197–199
Cramer H, Renaud B, Ortega-Suhrkamp E (1975) Konzentration von cyclo-AMP im lumbalen, zisternalen und ventrikulären Liquor bei neurologischen Patienten. Z klin Chem klin Biochem 13:245
Hökfelt T, Efendic S, Hellerström C, Johansson O, Luft R, Arimura A (1975) Cellular localization of somatostatin in endocrinelike cells and neurons of the rat with special references to the A1-cells of the pancreatic islets and to the hypothalamus. Acta Endocrinol Suppl (200) 80:5–41
Kanazawa L, Bird E, O'Connell R, Powell D (1977) Evidence for a decrease in substance P content of substantia nigra in Huntington's chorea. Brain Res 120:387–392
Kebabian J, Petzold GL, Greengard P (1972) Dopamine-sensitive adenylate cyclase activity in caudate nucleus of rat brain and its similarity to the “dopamine receptor”. Proc natn Acad Sci (Wash) 69:2145–2149
McCaughey WTE (1961) The pathological spectrum of Huntington's chorea. J nerv ment Dis 133:91–103
Myllylä VV, Heikkinen ER, Vapaatolo H, Hokkanen E (1975) Cyclic AMP concentration and enzyme activities of cerebrospinal fluid in patients with epilepsy or central nervous system damage. Eur Neurol 13:123–130
Oepen G, Clarenbach C, Thoden U (1981a) Disturbance of eye movements in chorea Huntington. Arch Psychiatr Nervenkr 229:205–213
Oepen G, Doerr M, Thoden U (1981b) Huntington's disease: Alterations of visual and somatosensory cortical evoked potentials in patients and offsprings. In: The clinical applications of evoked potentials in neurology. Raven Press, New York (in press)
Patel YC, Rao K, Reichlin S (1977) Somatostatin in human cerebrospinal fluid. N Engl J Med 296:524–533
Perry PT, Hansen S, Kloster M (1973) Huntington's chorea—deficiency of γ-aminobutyric acid in brain. New Engl J Med 288:337–342
Pimstone BL, Sheppard M, Shapiro B, Kronheim S, Hudson A, Hendricks S, Waligora K (1979) Localization in and release of somatostatin from brain and gut. Fed Proc 38:2330–2332
Podolsky S, Leopold NA (1974) Growth hormone abnormalities in Huntington's chorea: Effect of l-dopa administration. J clin Endocrinol Metab 39:36–39
Schröter E, Cramer H (1980) Wirkung der Neuromodulatoren Adenosin, Substanz P und Somatostatin auf die dopamin-sensitive Adenylatzyklase im Corpus striatum der Ratte. In: Mertens HG, Prunzek H (Hrsg) Pathologische Erregbarkeit des Nervensystems und ihre Behandlung. Springer, Berlin, pp 502–505
Sheppard M, Kronheim S, Adams C, Pimstone BL (1979a) Immunoreactive somatostatin release from rat spinal cord in vitro. Neurosci Letters 15:65–70
Sheppard MC, Kronheim S, Pimstone BL (1979b) Effect of substance P, neurotensin and the enkephalins on somatostatin release from the rat hypothalamus in vitro. J Neurochem 32:647–650
Sorensen KV, Christensen SE, Dupont E, Hansen AP, Pedersen E, Orskov H (1980) Low somatostatin content in cerebrospinal fluid in multiple sclerosis. Acta Neurol Scand 61:186–191
Spokes EGS (1980) Neurochemical alterations in Huntington's chorea: A study of post-mortem brain tissue. Brain 103:179–210
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Cramer, H., Kohler, J., Oepen, G. et al. Huntington's chorea—Measurements of somatostatin, substance P and Cyclic nucleotides in the cerebrospinal fluid. J Neurol 225, 183–187 (1981). https://doi.org/10.1007/BF00313747
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00313747