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October 1996, Vol 53, No. 10 >
Article | October 1996

Motor Neuron Disease With Parkinsonism

Adnan I. Qureshi, MD; George Wilmot, MD, PhD; Bhupesh Dihenia, MD; Julie A. Schneider, MD; David A. Krendel, MD
Arch Neurol. 1996;53(10):987-991. doi:10.1001/archneur.1996.00550100061015
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Objective:  To report clinical characteristics of patients with combined features of parkinsonism and motor neuron disease (MND).

Design:  Medical chart review.

Setting:  University medical center.

Patients:  Thirteen patients, identified by computer-assisted search, who had diagnoses of both parkinsonism and MND.

Results:  Median age was 68 years. There were 7 men and 6 women. All had clinical and electrodiagnostic evidence of both upper and lower motor neuron degeneration. One or more clinical manifestations of parkinsonism were observed in all patients either before the diagnosis of MND (n=2), at the time of initial evaluadon (n=10), or after the diagnosis of MND (n=1). The median time from symptom onset to presentation was 18 months. Improvement was seen in 5 of the 11 patients treated with levodopa. Dementia and autonomic dysfunction were absent in all patients. Postmortem neuropathological evaluation, available in 1 patient, demonstrated degeneration of the substantia nigra with Lewy bodies, mild pallor of the medullary pyramids, and neurogenic atrophy of the skeletal muscle.

Conclusion:  The onset of MND and parkinsonism within a relatively short period in most of our patients favors a common pathogenic mechanism over coincidental occurrence of 2 unrelated diseases. In patients with MND, it is important to recognize signs of parkinsonism that levodopa might alleviate.

REFERENCES

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Rowland LP.  Diverse forms of motor neuron diseases . In: Rowland LP, ed. Human Motor Neuron Diseases . New York, NY: Raven Press; 1982;:1-11.
2 +
Gardner-Medwin D, Walton JN.  Classification of neuromuscular diseases . In: Walton JN, ed. Disorders of Voluntary Muscle . 3rd ed. New York, NY: Churchill Livingstone Inc; 1974;:546-548.
3 +
Cornblath DR, Kuncl RW, Mellits D, et al.  Nerve conduction studies in amyotrophic lateral sclerosis . Muscle Nerve . 1992;;15:1111-1115.
4 +
Lambert EH.  Electromyography in amyotrophic lateral sclerosis . In: Norris FH, Kurkland LT, eds. Motor Neuron Diseases: Research on Amyotrophic Lateral Sclerosis and Related Disorders . New York, NY: Grune & Stratton; 1967;:135-153.
5 +
Yealland L.  Progressive muscular atrophy associated with paralysis agitans . Proc R Soc Med . 1937;;31:211-212.
6 +
Patrikios MJ.  Sclérose latérale amyotrophique avec mouvement involontaire des doigts et du poignet gauches de caractère extrapyramidal . Rev Neurol (Paris) . 1951;;85:60-62.
7 +
Cordier J.  Syndrome parkinsonien avec des amyotrophies rappelant la sclérose latérale amyotrophique et d'origine posttraumatique . Acta Neurol Belg . 1951;;51:194-205.
8 +
LeGrand R, Linquette M, Delahousse J, et al.  A propos d'un nouveau cas d'association d'une maladie de Parkinson et une sclérose latéral amyotrophique . Acta Neurol Belg . 1959;;101:191-193.
9 +
Adams RD, Victor M.  Degenerative diseases of the nervous system . In: Adams RD, Victor M, eds. Principles of Neurology . 4th ed. New York, NY: McGraw-Hill International Book Co; 1989;:921-967.
10 +
Brait K, Fahn S, Schwarz GA.  Sporadic and familial parkinsonism and motor neuron disease . Neurology . 1973;;23:990-1002.
11 +
Rosati G, Pinna L, Granieri E, et al.  Studies on epidemiological, clinical and etiological aspects of ALS disease in Sardinia, Southern Italy . Acta Neurol Scand . 1977;;55:231-244.
12 +
Eisen A, Calne D.  Amyotrophic lateral sclerosis, Parkinson's disease and Alzheimer's disease: phylogenetic disorders of the human neocortex sharing many characteristics . Can J Neurol Sci . 1992;;19:117-123.
13 +
Hudson AJ.  Amyotrophic lateral sclerosis and its association with dementia, parkinsonism, and other neurological disorders: a review . Brain . 1981;;104:217-247.
14 +
Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP.  Clinical features and natural history of multiple system atrophy: an analysis of 100 cases . Brain . 1994;;117:835-845.
15 +
Wechsler IS, Davison C.  Amyotrophic lateral sclerosis with mental symptoms: a clinico-pathologic study . Arch Neurol Psych . 1932;;27:857-880.
16 +
Caidus M, Marcutu V, Vuia O.  Sclérose latérale amyotrophique associée à la démence et au parkinsonisme . Acta Neurol Psych Belg . 1966;;66:719-731.
17 +
Boudouresques J, Toga M, Roger J, et al.  Etat dementiel, sclérose latérale amyotrophique, syndrome extrapyramidal: étude anatomique . Rev Neurol . 1967;;116:693-704.
18 +
Takahashi K, Nakamura H, Okada E.  Hereditary amyotrophic lateral sclerosis: histochemical and electron microscopic study of hyaline inclusions in motor neurons . Arch Neurol . 1972;;27:292-299.
19 +
Bonduelle M, Bougygues P, Escourolle R, Lormeau G.  Evolution simultanée d'une latérale amyotrophique, d'un syndrome parkinsonnien et d'un syndrome parkinsonnien et d'une démence progressive: à propos de deux observations anatomo-cliniques essai d'interprétation . J Neurol Sci . 1968;;6:315-332.
20 +
Kaiya H, Mehraein P.  Zur Klinik und pathologischen Anatomie des Muskelatrophie-Parkinsonismus-Demez-Syndroms . Arch Psychr Nervenkr . 1974;;219:13-27.
21 +
Gilbert JJ, Kish SJ, Chang LJ, Morito C, Shannak K, Hornykiewicz O.  Dementia, parkinsonism, and motorneuron disease: neurochemical and neuropathological correlates . Ann Neurol . 1988;;24:688-691.
22 +
Appel SH.  A unifying hypothesis for the cause of amyotrophic lateral sclerosis, parkinsonism and Alzheimer disease . Ann Neurol . 1981;;10:499-505.
23 +
Rosen DR, Siddique T, Patterson D, et al.  Mutations in Cu/Zn superoxide dismutase gene are associated with familial amytrophic lateral sclerosis . Nature . 1993;;362:59-62.
24 +
Pramatarova A, Figlewicz D, Krizus A, et al.  Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis . Am J Hum Genet . 1995;;56:592-596.
25 +
Olanow CW.  An introduction to the free radical hypothesis in Parkinson's disease . Ann Neurol . 1992;;32( (suppl) ):S2-S9.
26 +
Saggu H, Cooksey J, Dexter D, et al.  A selective increase in particulate superoxide dismutase activity in parkinsonian substantia nigra . J Neurochem . 1989;; 53:692-697.
27 +
Marttila RJ, Lorenz H, Rinne UK.  Oxygen toxicity protecting enzymes in Parkinson's disease: increase of superoxide dismutase-like activity in the subtantia nigra and basal nucleus . J Neurol Sci . 1988;;86:321-331.
28 +
Parboosingh JS, Rousseau M, Rogan F, et al.  Absence of mutations in superoxide dismutase and catalase genes in patients with Parkinson's disease . Arch Neurol . 1995;;52:1160-1163.
29 +
Ambani LM, Van Woert MH, Murphy S.  Brain peroxidase and catalase in Parkinson disease . Arch Neurol . 1975;;32:114-118.
30 +
Jellinger K.  Cytoskeletal pathology of parkinsonism and aging brain . In: Calne DB, Comi DB, Grippa D, Horowski R, Trabucchi M, eds. Parkinsonism and Aging . New York, NY: Raven Press; 1989;:35-56.
31 +
Leigh PN, Anderton BH, Dodson A, Gallo JM, Swash M, Power DM.  Ubiquitin deposits in anterior horn cells in motor neuron disease . Neurosci Lett . 1988;; 93:197-203.
32 +
Burrow JN, Blumbergs PC.  Substantia nigra degeneration in motor neuron disease: a quantitative study . Aust N Z J Med . 1992;;22:469-472.
33 +
Takahashi H, Snow BJ, Bhatt MH, Peppard R, Eisen A, Calne DB.  Evidence for a dopaminergic deficit in sporadic amyotrophic lateral sclerosis on positron emission scanning . Lancet . 1993;;342:1016-1018.
34 +
Deapen DM, Henderson BE.  A case-control study of amyotrophic lateral sclerosis . Am J Epidemiol . 1986;;123:790-799.
35 +
Mulder DW, Kurkland LT, Offord KP, Beard CM.  Familial adult motor neuron disease: amyotrophic lateral sclerosis . Neurology . 1986;;36:511-517.

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1 +
Rowland LP.  Diverse forms of motor neuron diseases . In: Rowland LP, ed. Human Motor Neuron Diseases . New York, NY: Raven Press; 1982;:1-11.
2 +
Gardner-Medwin D, Walton JN.  Classification of neuromuscular diseases . In: Walton JN, ed. Disorders of Voluntary Muscle . 3rd ed. New York, NY: Churchill Livingstone Inc; 1974;:546-548.
3 +
Cornblath DR, Kuncl RW, Mellits D, et al.  Nerve conduction studies in amyotrophic lateral sclerosis . Muscle Nerve . 1992;;15:1111-1115.
4 +
Lambert EH.  Electromyography in amyotrophic lateral sclerosis . In: Norris FH, Kurkland LT, eds. Motor Neuron Diseases: Research on Amyotrophic Lateral Sclerosis and Related Disorders . New York, NY: Grune & Stratton; 1967;:135-153.
5 +
Yealland L.  Progressive muscular atrophy associated with paralysis agitans . Proc R Soc Med . 1937;;31:211-212.
6 +
Patrikios MJ.  Sclérose latérale amyotrophique avec mouvement involontaire des doigts et du poignet gauches de caractère extrapyramidal . Rev Neurol (Paris) . 1951;;85:60-62.
7 +
Cordier J.  Syndrome parkinsonien avec des amyotrophies rappelant la sclérose latérale amyotrophique et d'origine posttraumatique . Acta Neurol Belg . 1951;;51:194-205.
8 +
LeGrand R, Linquette M, Delahousse J, et al.  A propos d'un nouveau cas d'association d'une maladie de Parkinson et une sclérose latéral amyotrophique . Acta Neurol Belg . 1959;;101:191-193.
9 +
Adams RD, Victor M.  Degenerative diseases of the nervous system . In: Adams RD, Victor M, eds. Principles of Neurology . 4th ed. New York, NY: McGraw-Hill International Book Co; 1989;:921-967.
10 +
Brait K, Fahn S, Schwarz GA.  Sporadic and familial parkinsonism and motor neuron disease . Neurology . 1973;;23:990-1002.
11 +
Rosati G, Pinna L, Granieri E, et al.  Studies on epidemiological, clinical and etiological aspects of ALS disease in Sardinia, Southern Italy . Acta Neurol Scand . 1977;;55:231-244.
12 +
Eisen A, Calne D.  Amyotrophic lateral sclerosis, Parkinson's disease and Alzheimer's disease: phylogenetic disorders of the human neocortex sharing many characteristics . Can J Neurol Sci . 1992;;19:117-123.
13 +
Hudson AJ.  Amyotrophic lateral sclerosis and its association with dementia, parkinsonism, and other neurological disorders: a review . Brain . 1981;;104:217-247.
14 +
Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP.  Clinical features and natural history of multiple system atrophy: an analysis of 100 cases . Brain . 1994;;117:835-845.
15 +
Wechsler IS, Davison C.  Amyotrophic lateral sclerosis with mental symptoms: a clinico-pathologic study . Arch Neurol Psych . 1932;;27:857-880.
16 +
Caidus M, Marcutu V, Vuia O.  Sclérose latérale amyotrophique associée à la démence et au parkinsonisme . Acta Neurol Psych Belg . 1966;;66:719-731.
17 +
Boudouresques J, Toga M, Roger J, et al.  Etat dementiel, sclérose latérale amyotrophique, syndrome extrapyramidal: étude anatomique . Rev Neurol . 1967;;116:693-704.
18 +
Takahashi K, Nakamura H, Okada E.  Hereditary amyotrophic lateral sclerosis: histochemical and electron microscopic study of hyaline inclusions in motor neurons . Arch Neurol . 1972;;27:292-299.
19 +
Bonduelle M, Bougygues P, Escourolle R, Lormeau G.  Evolution simultanée d'une latérale amyotrophique, d'un syndrome parkinsonnien et d'un syndrome parkinsonnien et d'une démence progressive: à propos de deux observations anatomo-cliniques essai d'interprétation . J Neurol Sci . 1968;;6:315-332.
20 +
Kaiya H, Mehraein P.  Zur Klinik und pathologischen Anatomie des Muskelatrophie-Parkinsonismus-Demez-Syndroms . Arch Psychr Nervenkr . 1974;;219:13-27.
21 +
Gilbert JJ, Kish SJ, Chang LJ, Morito C, Shannak K, Hornykiewicz O.  Dementia, parkinsonism, and motorneuron disease: neurochemical and neuropathological correlates . Ann Neurol . 1988;;24:688-691.
22 +
Appel SH.  A unifying hypothesis for the cause of amyotrophic lateral sclerosis, parkinsonism and Alzheimer disease . Ann Neurol . 1981;;10:499-505.
23 +
Rosen DR, Siddique T, Patterson D, et al.  Mutations in Cu/Zn superoxide dismutase gene are associated with familial amytrophic lateral sclerosis . Nature . 1993;;362:59-62.
24 +
Pramatarova A, Figlewicz D, Krizus A, et al.  Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis . Am J Hum Genet . 1995;;56:592-596.
25 +
Olanow CW.  An introduction to the free radical hypothesis in Parkinson's disease . Ann Neurol . 1992;;32( (suppl) ):S2-S9.
26 +
Saggu H, Cooksey J, Dexter D, et al.  A selective increase in particulate superoxide dismutase activity in parkinsonian substantia nigra . J Neurochem . 1989;; 53:692-697.
27 +
Marttila RJ, Lorenz H, Rinne UK.  Oxygen toxicity protecting enzymes in Parkinson's disease: increase of superoxide dismutase-like activity in the subtantia nigra and basal nucleus . J Neurol Sci . 1988;;86:321-331.
28 +
Parboosingh JS, Rousseau M, Rogan F, et al.  Absence of mutations in superoxide dismutase and catalase genes in patients with Parkinson's disease . Arch Neurol . 1995;;52:1160-1163.
29 +
Ambani LM, Van Woert MH, Murphy S.  Brain peroxidase and catalase in Parkinson disease . Arch Neurol . 1975;;32:114-118.
30 +
Jellinger K.  Cytoskeletal pathology of parkinsonism and aging brain . In: Calne DB, Comi DB, Grippa D, Horowski R, Trabucchi M, eds. Parkinsonism and Aging . New York, NY: Raven Press; 1989;:35-56.
31 +
Leigh PN, Anderton BH, Dodson A, Gallo JM, Swash M, Power DM.  Ubiquitin deposits in anterior horn cells in motor neuron disease . Neurosci Lett . 1988;; 93:197-203.
32 +
Burrow JN, Blumbergs PC.  Substantia nigra degeneration in motor neuron disease: a quantitative study . Aust N Z J Med . 1992;;22:469-472.
33 +
Takahashi H, Snow BJ, Bhatt MH, Peppard R, Eisen A, Calne DB.  Evidence for a dopaminergic deficit in sporadic amyotrophic lateral sclerosis on positron emission scanning . Lancet . 1993;;342:1016-1018.
34 +
Deapen DM, Henderson BE.  A case-control study of amyotrophic lateral sclerosis . Am J Epidemiol . 1986;;123:790-799.
35 +
Mulder DW, Kurkland LT, Offord KP, Beard CM.  Familial adult motor neuron disease: amyotrophic lateral sclerosis . Neurology . 1986;;36:511-517.

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