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Multi system atrophy: report of two cases.

CASE REPORTS:

Case 1: 55 yrs. old woman complained of gait and speech alteration. On physical and neurological examination he presented slurred speech, gait ataxia and bilateral appendicular cerebellar ataxia. The deep tendon reflexes were +++/4 and there was bilateral Babinski sign. Arterial pressure (AP) was 130X80mmHg and radial pulse frequency 60bpm with the patient laying down; 100X70 and 70bpm when standing up. Brain MRI showed important pontine atrophy with the "Cross sign" (Fig. 1A) as well as cerebellar atrophy (Fig. 1B). There was no neurological disease in her family.

Case 2: A 64 year-old man presented with rigidity of arms. Levodopa started as the diagnosis of Parkinson's disease was made but there was no improvement. He reported urinary incontinence. There was progressive deterioration of daily activities and he required aid to walk. On general physical and neurological evaluation, she presented extrapyramidal rigidity, bradykinesia and some periods of akinesia. There was no rest or postural tremor, but the speech was slurred, very difficult to be understood. There was hyperreflexia and Babinski sign on both sides. AP laying down was 149X90 and seated was 90X 50mmHg. Brain MRI shows sign alteration on lenticular nucleus (Fig 2A and 2B). There was no neurological disease in her family.

We report two patients presenting two different forms of MSA, although in the first case cerebellar syndrome was the main feature and in the second case, parkinsonian symptoms were predominant. Both of them had orthostatic hypotension and the second case also had urinary incontinence (Autonomic system alterations).

Watanabe et al. (8) evaluating the progression of MSA in 230 Japanese patients concluded that patients presenting MSA-P have a more rapid functional deterioration when compared to those with MSA-C. In the other hand, there was no difference in the survival time.

MRI is a useful diagnostic tool in the early course of MSA-C and MSA-P. Horimoto et al. (7) report that pontine "Cross sign" and lenticular nucleus sign alteration appears early in MSA-C and MSA-P respectively. Both of them appears lately in MSA-A. The characteristic T2 hyper intense sign in pons and middle cerebellar peduncle ("Cross sign") reflects pontocerebellar fibers degeneration and despite very suggestive of MSA it can be found in other forms of parkinsonism. (9) Asato et al. (10) have showed that the anteroposterior diameter of the inferior portion of the pons in MSA-C patients is lower when compared to patients in the control group or with progressive supranuclear palsy.

Putaminal abnormalities may be present in MSA-P patients MRI, other findings include hypo intense sign of the putamen with marginal hyper intense sign in T2. Atrophy or hyper intense sign at the pons, middle cerebellar peduncle and cerebellum may be seen. Putaminal atrophy is the most specific finding in MSA-P. (9) Our case number 2 presented putaminal hypo intensity as well as marginal hyper intensity in T2 images.

In conclusion, our cases are classified as likely MSA according to criteria in consensus, since the diagnosis of MSA is defined just with pathological analysis. (6) in the two cases, we try to contribute to the importance of a good MRI interpretation. We concluded that the brain MRI changes might increase the accuracy diagnosis of MSA.

DOI: 10.14260/jemds/2015/1890

REFERENCES:

(1.) Vanacore N. Epidemiological evidence on multiple system atrophy. J Neural Transm 2005; 112:1605-1612.

(2.) Weimer LH. Neurogenic orthostatic hypotension and autonomic failure. In Rowland LP. Merrit's Neurology. Philadelphia: Lippincott Willians and Wilkins, 2000:799-801.

(3.) Berciano J. Olivopontocerebellar atrophy. In Jankovic J, Tolosa E (Eds). Parkinson's disease and movement disorders. Urban & Schwarzenberg, 1988:131-151.

(4.) Litvan I, Goetz CG, Jankovic J, et al. What is accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study. Arch Neurol 1997; 54:937-944.

(5.) Wenning GK, Ben-Shlomo Y, Magalhaes M, Daniel SE, Quinn NP. Clinicopathological study of 35 cases of multiple system atrophy. J Neurol Neurosurg Psychiatry 1995; 58:160-166.

(6.) Gilman S, Low P, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. Clin Auton Res 1998; 8:359-362.

(7.) Horimoto Y, Aiba I, Yasuda T, et al. Longitudinal MRI study of multiple system atrophy: when do the findings appear, and what is the course? J Neurol 2002; 249:847-854.

(8.) Watanabe H, Saito Y, Terao S, et al. Progression and prognosis in multiple system atrophy an analysis of 230 Japanese patients. Brain 2002; 125:1070-1083.

(9.) Seppi K, Schocke MF, Wenning GK, Poewe W. How to diagnose MSA early: the role of magnetic resonance imaging. J Neural Transm 2005; 112:1625-1634.

(10.) Asato R, Akiguchi I, Masunaga S, Hashimoto N. Magnetic resonance imaging distinguishes progressive supranuclear palsy from multiple system atrophy. J Neural Transm 2000; 107:1427-1436.

Dipu Bhuyan [1], Rohit Kr. Chandak [2], Pankaj Kr. Patel [3], Sushant Agarwal [4], Debjanee Phukan [5]

AUTHORS:

[1.] Dipu Bhuyan

[2.] Rohit Kr. Chandak

[3.] Pankaj Kr. Patel

[4.] Sushant Agarwal

[5.] Debjanee Phukan

PARTICULARS OF CONTRIBUTORS:

[1.] Associate Professor, Department of Radiology, Gauhati Medical College.

[2.] Junior Resident, Department of Radiology, Gauhati Medical College.

[3.] Junior Resident, Department of Radiology, Gauhati Medical College.

[4.] Senior Resident, Department of Radiology, Gauhati Medical College.

[5.] Junior Resident, Department of Radiology, Gauhati Medical College.

FINANCIAL OR OTHER COMPETING INTERESTS: None

NAME ADDRESS EMAIL ID OF THE CORRESPONDING AUTHOR:

Dr. Dipu Bhuyan, Associate Professor, Department of Radiology, Gauhati Medical College and Hospital, Guwahati-781022, Assam.

E-mail: dipubhuyan@gmail.com

Date of Submission: 20/08/2015.

Date of Peer Review: 21/08/2015.

Date of Acceptance: 11/09/2015.

Date of Publishing: 15/09/2015.
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Title Annotation:CASE REPORT
Author:Bhuyan, Dipu; Chandak, Rohit Kr.; Patel, Pankaj Kr.; Agarwal, Sushant; Phukan, Debjanee
Publication:Journal of Evolution of Medical and Dental Sciences
Date:Sep 17, 2015
Words:921
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