Re: RESEARCH: Progression and prognosis in multiple system atrophy:

[Guest guest]

Pam, this is an interesting study. I read somewhere the Japanese have high

rate of PD. Cindy

RESEARCH: Progression and prognosis in multiple system

atrophy:

> 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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