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Extra-pyramidal disease

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Diagnostic Tests in Neurology

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Abstract

The clinical triad of bradykinesia, tremor and rigidity is felt to be sufficient to diagnose idiopathic Parkinson’s disease, the assumption being made that the underlying pathology is centred on the zona compacta of the substantia nigra. That assumption is probably correct in the majority of patients with this clinical picture, though it is likely that other neuropathological disorders, generally undiagnosable in life, can present with a similar pattern of disability. Drug-induced Parkinsonism may be indistinguishable from the idiopathic form, though tremor is less conspicuous. The clinical spectrum of post-encephalitic Parkinsonism is broader, whilst extrapyramidal features are only a part of the disorder accruing from multi-infarction. The frequency with which dementia occurs in Parkinson’s disease, and its severity, remains a matter of debate. One reviewer has suggested that one third of Parkinsonian patients become demented in the later stages of the disease [1], based on a study which recorded moderate to marked dementia in 32% of 520 patients [2]. Included among these patients, however, were some with atypical features (wasting, cerebellar tremor, dystop1a or pyramidal signs), though, even in these, the parkinsonian features predominated. Though the authors claimed that dementia was as likely in the typical as in the atypical cases, another paper, including 93 patients, came to the opposite conclusion. There, an organic mental syndrome (principally dementia) was found in 15% of typical but in 68% of atypical cases [3].

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References

  1. Marsden, C.D. (1982) Basal ganglia disease. Lancet, ii, 1141–7.

    Article  Google Scholar 

  2. Lieberman, A, Dziatolowski, M., Kupersmith, M. et al. (1979) Dementia in Parkinson disease. Ann. Neurol., 6, 355–9.

    Article  PubMed  CAS  Google Scholar 

  3. Sroka, H., Elizan, T.S., Yahr, M.D. et al. (1981) Organic mental syndrome and confusional states in Parkinson’s disease. Arch. Neurol., 38, 339–42.

    Article  PubMed  CAS  Google Scholar 

  4. Esteban, A. and Giménez-Roldân, S. (1975) Blink reflex in Hunting-ton’s chorea and Parkinson’s disease. Acta Neurol. Scand., 52, 145–57.

    Article  PubMed  CAS  Google Scholar 

  5. Gawel, M.J., Das, P., Vincent, S. and Rose, F.C. (1981) Visual and auditory evoked responses in patients with Parkinson’s disease. J. Neurol. Neurosurg. Psychiatry, 44, 227–32.

    Article  PubMed  CAS  Google Scholar 

  6. Kupersmith, M.J., Shakin, E., Siegel, I.M. and Lieberman, A. (1982) Visual system abnormalities in patients with Parkinson’s disease. Arch. Neurol., 39, 284–6.

    Article  PubMed  CAS  Google Scholar 

  7. Dinner, D.S., Luders, H., Hanson, M. et al. (1985) Pattern evoked potentials (PEPs) in Parkinson’s disease. Neurology, 35, 610–13.

    Article  PubMed  CAS  Google Scholar 

  8. Williams, A., Houff, S., Lees, A. and Calne, D.B. (1979) Oligoclonal banding in the cerebrospinal fluid of patients with postencephalitic Parkinsonism. J. Neurol. Neurosurg. Psychiatry, 42, 790–2.

    Article  PubMed  CAS  Google Scholar 

  9. Behan, P.O., Thomas, A.M. and Behan, W.M.H. (1981) Cerebrospinal fluid immunoglobulin profiles in Parkinson’s disease. in Research Progress in Parkinson’s Disease (ed. F.C. Rose and R. Capildeo ), Pitman Medical, London, pp. 181–5.

    Google Scholar 

  10. Johansson, B. and Roos, B-E. (1967) 5-Hydroxyindoleacetic and homovanillic acid levels in the cerebrospinal fluid of healthy volunteers and patients with Parkinson’s syndrome. Life Sci., 6, 1449–54.

    Google Scholar 

  11. Abbott, R.J., Pye, I.F. and Nahorski, S.R. (1982) CSF and plasma GABA levels in Parkinson’s disease. J. Neurol. Neurosurg. Psychiatry, 45, 253–6.

    Article  PubMed  CAS  Google Scholar 

  12. Nutt, J.G., Mroz, E.A., Leeman, S.E. et al. (1980) Substance Pin human cerebrospinal fluid: Reductions in peripheral neuropathy and autonomic dysfunction. Neurology, 30, 1280–5.

    Article  PubMed  CAS  Google Scholar 

  13. Gath, I., Jörgensen, A., Sjaastad, O. and Berstad, J. (1975) Pneumoencephalographic findings in Parkinsonism. Arch. Neurol., 32, 769–73.

    Article  PubMed  CAS  Google Scholar 

  14. Mayeux, R., Stern, Y., Rosen, J. and Benson, D.F. (1981) Subcortical dementia: A recognizable clinical entity. Ann. Neurol., 10, 100–1.

    Google Scholar 

  15. Wolfson, L.I., Leenders, K.L., Brown, L.L. and Jones, T. (1985) Alterations of regional cerebral blood flow and oxygen metabolism in Parkinson’s disease. Neurology, 35, 1399–405.

    Article  PubMed  CAS  Google Scholar 

  16. Nahmias, C., Garnett, E.S., Firnau, G. and Lang, A. (1985) Striatal dopamine distribution in Parkinsonian patients during life. J. Neurol. Sci., 69, 223–30.

    Article  PubMed  CAS  Google Scholar 

  17. Starr, A. (1967) A disorder of rapid eye movements in Huntington’s chorea. Brain, 90, 545–64.

    Article  PubMed  CAS  Google Scholar 

  18. Scott, D.F., Heathfield, K.W.G., Toone, B. and Margerison, J.H. (1972) The EEG in Huntington’s chorea: a clinical and neuropathological study. J. Neurol. Neurosurg. Psychiatry, 35, 97–102.

    Article  PubMed  CAS  Google Scholar 

  19. Ellenberger, C. Jr, Petro, D.J. and Ziegler, S.B. (1978) The visually evoked potential in Huntington’s disease. Neurology, 28, 95–7.

    Article  PubMed  Google Scholar 

  20. Wilson, R.S. and Garron, D.C. (1979) Cognitive and affective aspects of Huntington’s disease. In Advances in Neurology, Vol. 23 (ed. T.N. Chase, N.S. Wexler and A. Barbeau ), Raven Press, New York, pp. 193–201.

    Google Scholar 

  21. Bala Manyam, N.V., Hare, T.A. and Katz, L. (1979) Cerebrospinal fluid GABA levels in Huntington’s disease, `at risk’ for Huntington’s disease and normal controls. in Advances in Neurology, Vol. 23 (ed. T.N. Chase, N.S. Wexler and A. Barbeau ), Raven Press, New York, pp. 547–56.

    Google Scholar 

  22. Cramer, H., Kohler, J., Oepen, G. et al. (1981) Huntington’s chorea–measurements of somatostatin, substance P and cyclic nucleotides in the cerebrospinal fluid. J. Neurol., 225, 183–7.

    Article  PubMed  CAS  Google Scholar 

  23. Barr, A.N., Heinze, W.J., Dobben, G.D. et al. (1978) Bicaudate index in computerized tomography of Huntington’s disease and cerebral atrophy. Neurology, 28, 1196–200.

    Google Scholar 

  24. Martin, W.R.W., Hayden, M.R., Suchowersky, O. et al. (1984) Striatal metabolism in Huntington’s disease and in benign hereditary chorea. Ann. Neurol., 16, 126.

    Google Scholar 

  25. Patterson, R.M., Bagchi, B.K. and Test, A. (1948) The prediction of Huntington’s chorea. An electroencephalographic and genetic study. Am. J. Psychiatry, 104, 786–97.

    PubMed  CAS  Google Scholar 

  26. Chandler, J.H. (1966) EEG in prediction of Huntington’s chorea. An eighteen year follow-up. Electroencephalogr. Clin. Neurophysiol., 21, 79–80.

    Article  PubMed  CAS  Google Scholar 

  27. Esteban, A., Mateo, D. and Giménez-Roldân, S. (1981) Early detection of Huntington’s disease. Blink reflex and levodopa load in presymptomatic and incipient subjects. J. Neurol. Neurosurg. Psychiatry, 44, 43–8.

    Article  PubMed  CAS  Google Scholar 

  28. Oepen, G. and Ostertag, Ch. (1981) Diagnostic value of CT in patients with Huntington’s chorea and their offspring. J. Neurol., 225, 189–96.

    Article  PubMed  CAS  Google Scholar 

  29. Klawans, H.L. Jr, Paulson, G.W., Ringel, S.P. and Barbeau, A. (1972) Use of L-dopa in the detection of presymptomatic Huntington’s chorea. N. Engl. J. Med., 286, 1332–4.

    Article  PubMed  Google Scholar 

  30. Klawans, H.L., Goetz, C.G., Paulson, G.W. and Barbeau, A. (1980) Levodopa and presymptomatic detection of Huntington’s disease - eight-year follow-up. N. Engl. J. Med., 302, 1090.

    Article  PubMed  CAS  Google Scholar 

  31. Young, A.B., Penney, J.B., Starosta-Rubinstein, S. et al. (1987) Normal caudate glucose metabolism in persons at risk for Huntington’s disease. Arch. Neurol., 44, 254–7.

    Article  PubMed  CAS  Google Scholar 

  32. Gusella, J.F., Wexler, N.S., Conneally, P.M. et al. (1983) A polymorphic DNA marker genetically linked to Huntington’s disease. Nature, 306, 234–8.

    Article  PubMed  CAS  Google Scholar 

  33. Richardson, J.C., Steele, J. and Olszewski, J. (1963) Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. Trans. Am. Neurol. Assoc., 88, 25–7.

    PubMed  CAS  Google Scholar 

  34. Albert, M.L., Feldman, R.G., and Willis, A.L. (1974) The `subcortical dementia’ of progressive supranuclear palsy. J. Neurol. Neurosurg. Psychiatry, 37, 121–30.

    Article  PubMed  CAS  Google Scholar 

  35. Perkin, G.D., Lees, A.J., Stern, G.M. and Kocen, R.S. (1978) Problems in the diagnosis of progressive supranuclear palsy (Steele-RichardsonOlszewski syndrome). Can. J. Neurol. Sci., 5, 167–73.

    Google Scholar 

  36. Pfaffenbach, D.D., Layton, D.D. and Kearns, T.P. (1972) Ocular manifestations in progressive supranuclear palsy. Am. J. Ophthalmol., 74, 1179–84.

    PubMed  CAS  Google Scholar 

  37. Haldeman, S., Goldman, J.W., Hyde, J. and Pribram, H.F.W. (1981) Progressive supranuclear palsy, computed tomography and response to antiparkinsonian drugs. Neurology, 31, 442–5.

    PubMed  CAS  Google Scholar 

  38. Baron, J.C., Maziere, B., Loch, C. et al. (1985) Progressive supranuclear palsy: loss of striatal dopamine receptors demonstrated in vivo by positron tomography. Lancet, 1, 1163–4.

    Article  PubMed  CAS  Google Scholar 

  39. Strickland, G.T. and Leu, M-L. (1975) Wilson’s disease. Clinical and laboratory manifestations in 40 patients. Medicine (Baltimore), 54, 113–37.

    CAS  Google Scholar 

  40. Ross, M.E., Jacobson, I.M., Dienstag, J.L. and Martin, J.B. (1985) Late-onset Wilson’s disease with neurological involvement in the absence of Kayser-Fleischer rings. Ann. Neurol., 17, 411–13.

    Article  PubMed  CAS  Google Scholar 

  41. Roach, E.S., Ford, C.S., Spudis, E.V. et al. (1985) Wilson’s disease: evoked potentials and computed tomography. J. Neurol., 232, 20–3.

    Article  PubMed  CAS  Google Scholar 

  42. Williams, F.J.B. and Walshe, J.M. (1981) Wilson’s disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents. Brain, 104, 735–52.

    Article  PubMed  CAS  Google Scholar 

  43. Lawler, G.A., Pennock, J.M., Steiner, R.E. et al. (1983) Nuclear magnetic resonance (NMR) imaging in Wilson’s disease. J. Comput. Assist. Tomogr., 7, 1–8.

    Article  PubMed  CAS  Google Scholar 

  44. Marsden, C.D. (1976) Dystonia: the spectrum of the disease. in The Basal Ganglia (ed. M.D. Yahr ), Raven Press, New York, pp. 351–67.

    Google Scholar 

  45. Marsden, C.D., Obeso, J.A., Zarranz, J.J. and Lang, A.E. (1985) The anatomical basis of symptomatic hemidystonia. Brain, 108, 463–83.

    Article  PubMed  Google Scholar 

  46. Bodensteiner, J.B., Goldblum, R.M. and Goldman, A.S. (1980) Progressive dystonia masking ataxia in ataxia-telangiectasia. Arch. Neurol., 37, 464–5.

    Article  PubMed  CAS  Google Scholar 

  47. Neville, B.G.R., Lake, B.D., Stephens, R. and Sanders, M.D. (1973) A neurovisceral storage disease with vertical supranuclear ophthalmoplegia, and its relationship to Niemann—Pick disease. A report of nine patients. Brain, 96, 97–120.

    Article  PubMed  CAS  Google Scholar 

  48. Longstreth, W.T. Jr, Farrell, D.F., Bolen, J.W. et al. (1982) Adult dystonic lipidosis: clinical, histologic, and biochemical findings of a neurovisceral storage disease. Neurology, 32 (2), A141.

    Google Scholar 

  49. Dooling, E.C., Schoene, W.C. and Richardson, E.P. Jr (1974) Hallervorden—Spatz syndrome. Arch. Neurol., 30, 70–83.

    Article  PubMed  CAS  Google Scholar 

  50. Hall, K. and Gardner-Medwin, D. (1978) CT scan appearances in Leigh’s disease (subacute necrotizing encephalomyelopathy). Neuro-radiology, 16, 48–50.

    CAS  Google Scholar 

  51. Marsden, C.D., Lang, A.E., Quinn, N.P. et al. (1986) Familial dystonia and visual failure with striatal CT lucencies. J. Neurol. Neurosurg. Psychiatry, 49, 500–9.

    Article  PubMed  CAS  Google Scholar 

  52. Aminoff, M.J. (1972) Acanthocytosis and neurological disease. Brain, 95, 749–60.

    Article  PubMed  CAS  Google Scholar 

  53. Larsen, T.A., Dunn, H.G., Jan, J.E. and Calne, D.B. (1985) Dystonia and calcification of the basal ganglia. Neurology, 35, 533–7.

    Article  PubMed  CAS  Google Scholar 

  54. Fahn, S., Marsden, C.D. and Calne, D.B. (1987) Classification and investigation of dystonia. in Movement Disorders, 2 (ed. C.D. Marsden and S. Fahn ), Butterworths, London, pp. 332–58.

    Google Scholar 

  55. Lang, A.E., Clarke, J.T.R., Resch, L. et al. (1985) Progressive longstanding `pure’ dystonia: a new phenotype of juvenile metachromatic leukodystrophy (MLD). Neurology, 35, (Suppl. 1), 194.

    Article  Google Scholar 

  56. Nakano, T., Ikeda, S.-I., Kondo, K. et al. (1985) Adult GM, -gangliosidosis: clinical patterns and rectal biopsy. Neurology, 35, 875–80.

    Article  PubMed  CAS  Google Scholar 

  57. Tahmoush, A.J., Alpers, D.H., Feigin, R.D. et al. (1976) Hartnup disease. Clinical, pathological and biochemical observations. Arch. Neurol., 33, 797–807.

    Article  PubMed  CAS  Google Scholar 

  58. Schwartz, J.F., Rowland, L.P., Eder, H. et al. (1963) Bassen—Kornzweig syndrome: deficiency of serum 13-lipoprotein. Arch. Neurol., 8, 438–54.

    Article  Google Scholar 

  59. Gross, K.B., Skrivanek, J.A., Carlson, K.C. and Kaufman, D.M. (1985) Familial amyotrophic chorea with acanthocytosis. New clinical and laboratory investigations. Arch. Neurol., 42, 753–6.

    Article  PubMed  CAS  Google Scholar 

  60. Greenhouse, A.H. (1966) On chorea, lupus erythematosus, and cerebral arteritis. Arch. Intern. Med., 117, 389–93.

    Article  Google Scholar 

  61. Krumholz, A., Singer, H.S., Niedermeyer, E. et al. (1983) Electrophysiological studies in Tourette’s syndrome. Ann. Neurol., 14, 638–41.

    Article  PubMed  CAS  Google Scholar 

  62. Lees, A.J., Robertson, M., Trimble, M.R. and Murray, N.M.F. (1984) A clinical study of Gilles de la Tourette syndrome in the United Kingdom. J. Neurol. Neurosurg. Psychiatry, 47, 1–8.

    Article  PubMed  CAS  Google Scholar 

  63. Brainin, M., Reisner, Th. and Zeitlhofer, J. (1983) Tardive dyskinesia: clinical correlation with computed tomography in patients aged less than 60 years. J. Neurol. Neurosurg. Psychiatry, 46, 1037–40.

    Article  PubMed  CAS  Google Scholar 

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© 1988 G.D. Perkin

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Perkin, G.D. (1988). Extra-pyramidal disease. In: Diagnostic Tests in Neurology. Diagnostic Tests Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-3320-1_5

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  • DOI: https://doi.org/10.1007/978-1-4899-3320-1_5

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