RESEARCH: Progression and prognosis in multiple system atrophy:

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Brain 2002 May;125(Pt 5):1070-1083

Progression and prognosis in multiple system atrophy: An analysis of 230

Japanese patients.

Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y,

Tamakoshi A, Doyu M, Hirayama M, Sobue G.

Department of Neurology and Department of Preventive Medicine/Biostatistics

and Medical Decision Making, Nagoya University Graduate School of Medicine,

Department of Neurology, Nagoya National Hospital, Department of Neurology,

Higashi Nagoya National Hospital, Department of Neurology, Nagoya Daini Red

Cross Hospital, Nagoya, Division of Neurology, Department of General

Medicine, Aichi Medical University and. Department of Neurology, Chubu

National Hospital, Japan.

We investigated the disease progression and survival in 230 Japanese

patients with multiple system atrophy (MSA; 131 men, 99 women; 208 probable

MSA, 22 definite; mean age at onset, 55.4 years). Cerebellar dysfunction

(multiple system atrophy-cerebellar; MSA-C) predominated in 155 patients,

and parkinsonism (multiple system atrophy-parkinsonian; MSA-P) in 75. The

median time from initial symptom to combined motor and autonomic dysfunction

was 2 years (range 1-10). Median intervals from onset to aid-requiring

walking, confinement to a wheelchair, a bedridden state and death were 3, 5,

8 and 9 years, respectively. Patients manifesting combined motor and

autonomic involvement within 3 years of onset had a significantly increased

risk of not only developing advanced disease stage but also shorter survival

(P < 0.01). MSA-P patients had more rapid functional deterioration than

MSA-C patients (aid-requiring walking, P = 0.03; confinement to a

wheelchair, P < 0.01; bedridden state, P < 0.01), but showed similar

survival. Onset in older individuals showed increased risk of confinement to

a wheelchair (P < 0.05), bedridden state (P = 0.03) and death (P < 0.01).

Patients initially complaining of motor symptoms had accelerated risk of

aid-requiring walking (P < 0.01) and confinement to a wheelchair (P < 0.01)

compared with those initially complaining of autonomic symptoms, while the

time until confinement to a bedridden state and survival were no worse.

Gender was not associated with differences in worsening of function or

survival. On MRI, a hyperintense rim at the lateral edge of the dorsolateral

putamen was seen in 34.5% of cases, and a 'hot cross bun' sign in the

pontine basis (PB) in 63.3%. These putaminal and pontine abnormalities

became more prominent as MSA-P and MSA-C features advanced. The atrophy of

the cerebellar vermis and PB showed a significant correlation particularly

with the interval following the appearance of cerebellar symptoms in MSA-C

(r = 0.71, P < 0.01, r = 0.76 and P < 0.01, respectively), but the

relationship between atrophy and functional status was highly variable among

the individuals, suggesting that other factors influenced the functional

deterioration. Atrophy of the corpus callosum was seen in a subpopulation of

MSA, suggesting hemispheric involvement in a subgroup of MSA patients. The

present study suggested that many factors are involved in the progression of

MSA but, most importantly, the interval from initial symptom to combined

motor and autonomic dysfunction can predict functional deterioration and

survival in MSA.

PMID: 11960896 [PubMed - as supplied by publisher]

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