RESEARCH: Progression and prognosis in multiple system atrophy

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Here ya go .

The Striatonigral Degeneration (SND) form is called MSA-P in this study.

The Olivopontocerebellar Atrophy (OPCA) form is called MSA-C in this study.

Hugs,

Pam

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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]