Article
“Pure” striatonigral degeneration and Parkinson's disease: A comparative clinical study
Dr. Neziha Gouider-Khouja,
Marie Vidailhet,
Anne-Marie Bonnet,
Jacques Pichon,
Yves Agid,
Corresponding Author
Dr. Neziha Gouider-Khouja
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Service de Neurologie et Neuropsychologie, Hôpital de la Salpêtrière, 47 Bd. de l'Hôpital, 75013 Paris, FranceSearch for more papers by this authorMarie Vidailhet
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorAnne-Marie Bonnet
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorJacques Pichon
Service de Rééducation Neurologique, Hópital de la Salpétrière, Paris, France
Search for more papers by this authorYves Agid
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorDr. Neziha Gouider-Khouja,
Marie Vidailhet,
Anne-Marie Bonnet,
Jacques Pichon,
Yves Agid,
Corresponding Author
Dr. Neziha Gouider-Khouja
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Service de Neurologie et Neuropsychologie, Hôpital de la Salpêtrière, 47 Bd. de l'Hôpital, 75013 Paris, FranceSearch for more papers by this authorMarie Vidailhet
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorAnne-Marie Bonnet
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorJacques Pichon
Service de Rééducation Neurologique, Hópital de la Salpétrière, Paris, France
Search for more papers by this authorYves Agid
INSERM U 289 and Service de Neurologie et Neuropsychologie, Paris, France
Search for more papers by this authorAbstract
Striatonigral degeneration (SND) is difficult to diagnose in vivo. The purpose of this study was to detect the best indicators for an early and reliable diagnosis of the disease. Eighteen patients clinically diagnosed as having SND were selected with rigorous inclusion criteria and compared to 18 patients with Parkinson's disease (PD) matched for age and disease duration. Apart from dysautonomia, the principal discriminant clinical features that distinguished SND from PD were the early appearance of the following symptoms and signs: (a) severe and atypical progressive parkinsonism characterized by bilateral bradykinesia and rigidity, slowness of gait, postural instability, and falls, and poor or absent response to adequate levodopa treatment; (b) increased tendon reflexes associated or not with frank pyramidal signs, severe dysarthria, and less consistently, dysphagia, stridor, antecollis, and stimulussensitive myoclonus, which, when present, are highly suggestive of the disease.
References
- 1
Quinn N.
Multiple system atrophy.
In: CD Marsden,
S Fahn, eds.
Movement disorders 3.
London:
Butterworth,
1994:
262–281.
- 2
Graham JG,
Oppenheimer DR.
Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy.
J Neurol Neurosurg Psychiatry
1969;
32:
28–34.
- 3
Bannister R,
Oppenheimer DR.
Degenerative diseases of the nervous system associated with autonomic failure.
Brain
1972;
95:
457–474.
- 4
Oppenheimer D.
Neuropathology of progressive autonomic failure.
In: R Bannister, ed.
Autonomic failure. A textbook of clinical disorders of the autonomic nervous system.
1st ed.
New York:
Oxford University Press,
1983:
267–283.
- 5
Gray F,
Vincent D,
Hauw JJ.
Quantitative study of lateral horn cells in 15 cases of multiple system atrophy.
Acta Neuropathol
1988;
75:
513–518.
- 6
Fearnley JM,
Lees AJ.
Striatonigral degeneration: a clinicopathological study.
Brain
1990;
113:
1823–1842.
- 7
Papp MI,
Kahn JE,
Lantos PL.
Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome).
J Neurol Sci
1989;
94:
79–100.
- 8
Nakazato Y,
Yamazaki H,
Hirato J,
Ishida Y,
Yamaguchi H.
Oligodendroglial microtubular tangles in olivopontocerebellar atrophy.
J Neuropathol Exp Neurol
1990;
49:
521–530.
- 9
Costa C,
Duyckaerts C,
Cervera P,
Hauw JJ.
Les inclusions oligodendrogliales, un marqueur des atrophies multisystématisées.
Rev Neurol
1992;
148:
274–280.
- 10
Quinn N.
Multiple system atrophy–the nature of the beast.
J Neurol Neurosurg Psychiatry
1989;
Suppl:
78–89.
- 11
Wenning GK,
Quinn N.
Multiple system atrophy: recent advances in clinical and pathological definition.
Curr Med Lit Neurol Neurosurg
1993;
8:
95–98.
- 12
Aotsuka A,
Paulson GW.
Striatonigral degeneration.
In: MB Stern,
WC Koller, eds.
Parkinsonian syndromes.
New York:
Marcel Dekker,
1993:
33–42.
- 13
McLeod JG,
Tuck RR.
Disorders of the autonomic nervous system: part 2. Investigation and treatment.
Ann Neurol
1987;
21:
519–529.
- 14
Folstein MF,
Folstein SE,
McHugh PR.
Mini-Mental State: a practical method for grading the cognitive state of patients for the clinician.
J Psychiatr Res
1975;
12:
189–198.
- 15
Fahn S,
Elton RL
Members of the UPDRS development committee. Unified Parkinson's Disease Rating Scale.
In: S Fahn,
CD Marsden,
M Goldstein,
DB Calne, eds.
Recent developments in Parkinson's disease.
Vol 2.
New York:
Macmillan,
1987:
153–163.
- 16
Adams RD,
Salam-Adams M.
Striatonigral degeneration.
In: PJ Vinken,
GW Bruyn,
HL Klawans, eds.
Handbook of clinical neurology, vol 5 (49): extrapyramidal disorders.
Amsterdam:
Elsevier Science Publishers BV,
1986:
205–212.
- 17
Takei Y,
Mirra SS.
Striatonigral degeneration: a form of multiple system atrophy with clinical parkinsonism.
In: HM Zimmerman, ed.
Progress in neuropathology.
Vol 2.
New York:
Grune & Stratton,
1973:
217–251.
- 18
Hoehn MM,
Yahr MD.
Parkinsonism: onset, progression and mortality.
Neurology
1967;
17:
427–442.
- 19
Sharpe JA,
Rewcastle NB,
Lloyd KG,
Hornykiewicz O,
Hill M,
Tasker RR.
Striatonigral degeneration. Response to levodopa therapy with pathological and neurochemical correlation.
J Neurol Sci
1973:
19:
275hyphen;286.
- 20
Parati EA,
Fetoni V,
Geminiani GC et al.
Response to l-dopa in multiple system atrophy.
Clin Neuropharmacol
1993;
16
(2):
139–144.
- 21
Quinn NP,
Koller WC,
Lang AE,
Marsden CD.
Painful Parkinson's disease.
Lancet
1986;
1:
1366–1369.
- 22
Cohen J,
Low P,
Fealey R,
Sheps S,
Jiang NS.
Somatic and autonomic function in progressive autonomic failure and multiple system atrophy.
Ann Neurol
1987;
22:
692–699.
- 23
Munschauer FE,
Loh L,
Bannister R,
Newsom-Davis J.
Abnormal respiration and sudden death during sleep in multiple system atrophy with autonomic failure.
Neurology
1990;
40:
677–679.
- 24
Bannister R,
Gibson W,
Michaels L,
Oppenheimer DR.
Laryngeal abductor paralysis in multiple system atrophy: a report on three necropsied cases, with observations on the laryngeal muscles and the nuclei ambigui.
Brain
1981;
104:
351–368.
- 25
Kirby R,
Fowler C,
Gosling J,
Bannister R.
Urethro-vesical dysfunction in progressive autonomic failure with multiple system atrophy.
J Neurol Neurosurg Psychiatry
1986;
49:
554–562.
- 26
Agid Y.
Parkinson's disease and cell death.
Lancet
1991;
337:
1321–1324.
- 27
Quinn N.
Disproportionate antecollis in multiple system atrophy.
Lancet
1989;
i:
844.
- 28
Rivest J,
Quinn N,
Marsden CD.
Dystonia in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy.
Neurology
1990;
40:
1571–1578.
- 29
Chen R,
Ashby P,
Lang AE.
Stimulus-sensitive myoclonus in akinetic-rigid syndromes.
Brain
1992;
115:
1875–1888.
- 30
Obeso JA,
Rodriguez ME,
Artieda J et al.
Focal reflex myoclonus: a useful sign in the differential diagnosis of parkinsonism.
Ann Neurol
1989;
26:
164–165.
- 31
Duvoisin RC.
Clinical diagnosis.
In: I Litvan,
Y Agid, eds.
Progressive supranuclear palsy: clinical and research approaches.
New York:
Oxford University Press,
1992:
15–33.
- 32
Robbins TW,
James M,
Lange KW,
Owen AM,
Quinn NP,
Marsden CD.
Cognitive performance in multiple system atrophy.
Brain
1992;
115:
271–291.
- 33
Pillon B,
Gouider-Khouja N,
Deweer B et al.
The neuropsychological pattern of striatonigral degeneration.
J Neurol Neurosurg Psychiatry
(in press).
- 34
Rascol O,
Sabatini U,
Simonetta-Moreau M,
Montastruc JL,
Clanet M.
Square wave jerks in parkinsonian syndromes. Abnormal ocular movements in Parkinson's disease.
J Neurol Neurosurg Psychiatry
1991;
54:
599–602.
- 35
Vidailhet M,
Rivaud S,
Gouider-Khouja N et al.
Eye movements in parkinsonian syndromes.
Ann Neurol
1994;
35:
420–426.
- 36
Ludin SM,
Steiger UH,
Ludin HP.
Autonomic disturbances and cardiovascular reflexes in idiopathic Parkinson's disease.
J Neurol
1987;
235:
10–15.
- 37
Prasher D,
Bannister R.
Brainstem auditory evoked potentials in patients with multiple system atrophy with progressive autonomic failure (Shy-Drager syndrome).
J Neurol Neurosurg Psychiatry
1986;
49:
278–289.
- 38
Uematsu D,
Hamada J,
Gotoh F.
Brainstem auditory evoked responses and CT findings in multiple system atrophy.
J Neurol Sci
1987;
77:
161–171.
- 39
Stern MB,
Braffman BH,
Skolnick BE,
Hurtig HI,
Grossman RI.
Magnetic resonance imaging in Parkinson's disease and parkinsonian syndromes.
Neurology
1989;
39:
1524–1526.
- 40
Stall A,
Meerwaldt JD,
Van Dongen KK,
Muller PGH,
Busch HFM.
Non-familial degenerative disease and atrophy of brainstem and cerebellum. Clinical and CT scan data in 47 patients.
J Neurol Sci
1990;
95:
259–269.
- 41
Drayer BP,
Olanow W,
Burger P,
Johnson GA,
Herfkens R,
Riederer S.
Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron.
Radiology
1986;
159:
493–498.
- 42
O'Brien C,
Sung JH,
McGeachie RE,
Lee MC.
Striatonigral degeneration: clinical, MRI and pathological correlation.
Neurology
1990;
40:
710–711.
