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Hemiparkinsonism-Somatic Hemiatrophy Syndrome

Published online by Cambridge University Press:  14 September 2018

Mary Jenkins ,
Dan Mendonca ,
Andrew Parrent  and
Mandar S. Jog
Mary Jenkins
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Centre, London, ON Canada
Dan Mendonca
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Centre, London, ON Canada
Andrew Parrent
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Centre, London, ON Canada
Mandar S. Jog*
Affiliation:
Department of Clinical Neurological Sciences, London Health Sciences Centre, London, ON Canada
*
Mandar S. Jog, Movement Disorders Program, London Health Sciences Centre, 339 Windermere Road, London, ON Canada N6A5A5
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Abstract:

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Purpose:

This paper highlights the hemiparkinsonism-hemiatrophy (HPHA) syndrome as a unique presentation of the parkinsonian state. Clinically relevant diagnostic and treatment aspects are reviewed.

Method:

We report a case of HPHA, in a 21-year-old, otherwise healthy, woman. Clinical and radiographic features of our case are presented. We also review the current literature on the clinical, radiological and pathophysiological mechanisms of HPHA.

Results:

In our case, despite the lack of benefit from anticholinergics and dopamine agonists (the patient declined treatment with levodopa), the patient showed a dramatic improvement with subthalamic nucleus stimulator (STN) implantation. There are no reported cases of use of STN stimulator in HPHA.

Conclusion:

Hemiparkinsonism-hemiatrophy is a distinct entity that may be clinically and pathogenetically different from idiopathic Parkinson’s disease; hence, HPHA needs to be considered as a possible syndrome in patients that have persistent unilateral parkinsonism. As medications are reported to be unhelpful in HPHA cases, early surgical intervention may be an option, such as in our case.

Résumé:

Résumé:Objectif:

Cet article met en lumière le syndrome hémiparkinsonisme-hémiatrophie (HPHA) comme présentation particulière de l’état parkinsonien. Les aspects diagnostiques et thérapeutiques pertinents à la clinique sont revus.

Méthode:

Nous rapportons un cas de HPHA chez une femme âgée de 21 ans, en bonne santé par ailleurs, et présentons les observations cliniques et radiologiques pertinentes. Nous avons revu la littérature sur les mécanismes cliniques, radiologiques et physiopathologiques de l’HPHA.

Résultats:

Chez notre patiente, malgré l’absence de réponse aux anticholinergiques et aux agonistes dopaminergiques (la patiente a refusé de prendre de la lévodopa), une amélioration marquée a été observée suite à l’implantation d’un stimulateur dans le noyau sous-thalamique (NST). Il n’existe pas de cas rapporté de l’utilisation d’un stimulateur du NST dans l’HPHA.

Conclusion:

L’hémiparkinsonismehémiatrophie est une entité distincte qui est peut-être différente de la maladie de Parkinson idiopathique au point de vue clinique et anatomopathologique. L’HPHAdoit donc être considéré dans le diagnostic différentiel des patients qui présentent un parkinsonisme unilatéral persistant. Comme, selon la littérature, la médication n’est pas efficace chez les cas de HPHA, une chirurgie précoce peut être une option valable comme ce fut le cas chez notre patiente.

Type
Case Reports
Information
Canadian Journal of Neurological Sciences , Volume 29 , Issue 2 , May 2002 , pp. 184 - 187
Copyright
Copyright © Canadian Neurological Sciences Federation 2002

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