Vol 47, No 4 (2013)

open access

Page views 318
Article views/downloads 801
Get Citation

Connect on Social Media

Connect on Social Media

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

Artur Dziadkiewicz12, Monika Białecka3, Piotr Janik4, Jarosław Sławek12
DOI: 10.5114/ninp.2013.34557
Neurol Neurochir Pol 2013;47(4):387-392.

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.

Article available in PDF format

View PDF Download PDF file



Neurologia i Neurochirurgia Polska