open access

Vol 47, No 4 (2013)
OPIS PRZYPADKU
Submitted: 2012-08-08
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Hemiparkinsonism-hemiatrophy syndrome – report on two cases and review of the literature

Artur Dziadkiewicz, Monika Białecka, Piotr Janik, Jarosław Sławek
DOI: 10.5114/ninp.2013.34557
·
Neurol Neurochir Pol 2013;47(4):387-392.

open access

Vol 47, No 4 (2013)
OPIS PRZYPADKU
Submitted: 2012-08-08

Abstract

Hemiparkinsonism-hemiatrophy (HPHA) is a rare neurological syndrome. The main clinical features of HPHA consist of atrophy of one side of the body (face, trunk, limbs), ipsilateral hemiparkinsonism (bradykinesia, rigidity, tremor) and in many cases dystonia. There are no data on prevalence of HPHA as the condition is rare. The mean age of parkinsonism onset is earlier than in idiopathic Parkinson disease (43.7 years, range: 15–63). Changes in magnetic resonance imaging (MRI) (cortical, basal ganglia atrophy contralaterally to the side of clinical presentation) are described in 30% of patients. The pathogenesis of HPHA is unknown, but in many cases a history of prenatal injuries was reported.

We present two male patients with HPHA – 45 and 55 years old, with left-sided parkinsonism, dystonia and hemiatrophy (to our knowledge, the first Polish cases). Both patients had no atrophic changes in MRI and levodopa treatment was ineffective. In the discussion the authors review current literature on HPHA.

Abstract

Hemiparkinsonism-hemiatrophy (HPHA) is a rare neurological syndrome. The main clinical features of HPHA consist of atrophy of one side of the body (face, trunk, limbs), ipsilateral hemiparkinsonism (bradykinesia, rigidity, tremor) and in many cases dystonia. There are no data on prevalence of HPHA as the condition is rare. The mean age of parkinsonism onset is earlier than in idiopathic Parkinson disease (43.7 years, range: 15–63). Changes in magnetic resonance imaging (MRI) (cortical, basal ganglia atrophy contralaterally to the side of clinical presentation) are described in 30% of patients. The pathogenesis of HPHA is unknown, but in many cases a history of prenatal injuries was reported.

We present two male patients with HPHA – 45 and 55 years old, with left-sided parkinsonism, dystonia and hemiatrophy (to our knowledge, the first Polish cases). Both patients had no atrophic changes in MRI and levodopa treatment was ineffective. In the discussion the authors review current literature on HPHA.

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Keywords

hemiparkinsonism, hemiatrophy, parkinsonism, dystonia, head injury

About this article
Title

Hemiparkinsonism-hemiatrophy syndrome – report on two cases and review of the literature

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 47, No 4 (2013)

Pages

387-392

DOI

10.5114/ninp.2013.34557

Bibliographic record

Neurol Neurochir Pol 2013;47(4):387-392.

Keywords

hemiparkinsonism
hemiatrophy
parkinsonism
dystonia
head injury

Authors

Artur Dziadkiewicz
Monika Białecka
Piotr Janik
Jarosław Sławek

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