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Hemiparkinsonism-hemiatrophy syndrome – report on two cases and review of the literature
Affiliations- Zakład Pielęgniarstwa Neurologiczno-Psychiatrycznego, Gdański Uniwersytet Medyczny
- Kliniczny Oddział Neurologii, Samodzielny Publiczny Szpital Kliniczny nr 1, Śląski Uniwersytet Medyczny w Katowicach
- Katedra Farmakologii, Pomorski Uniwersytet Medyczny w Szczecinie
- Katedra i Klinika Neurologii Collegium Medicum, Uniwersytet Jagielloński w Krakowie
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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.
Keywords
hemiparkinsonism, hemiatrophy, parkinsonism, dystonia, head injury
About this articleTitle
Hemiparkinsonism-hemiatrophy syndrome – report on two cases and review of the literature
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
387-392
Page views
308
Article views/downloads
765
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
