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Tremor-Dominant Pantothenate Kinase-associated Neurodegeneration

Authors :
Alfonso Fasano
Anthony E. Lang
Mohammad Rohani
Afagh Alavi
Said Razme
Gholam Ali Shahidi
Niloufar Yousefi
Source :
Movement Disorders Clinical Practice. 4:772-774
Publication Year :
2017
Publisher :
Wiley, 2017.

Abstract

Background Neurodegeneration with brain iron accumulation (NBIA) includes rare and heterogeneous group of disorders characterized by iron deposition in the basal ganglia. Pantothenate kinase-associated neurodegeneration (PKAN), is the most common NBIA and has two main presentations: typical and atypical, the latter rarely presenting with tremor. Method Reported patients underwent full neurologic examination, a standard brain MRI and genetic testing for PKAN. Results Three cases of ‘tremor-dominant’ PKAN with a relatively benign course were reported: dystonic tremor was seen in one patient and Parkinsonian tremor in remaining ones. All of them had homozygous mutations in PANK2 gene and typical eye of the tiger sign on brain MRI. Conclusions PKAN (and NBIA in general) might be a potential cause of tremor, thus emphasizing the need to consider this diagnosis even in patients with a clinical diagnosis of essential, dystonic or Parkinsonian tremor. This article is protected by copyright. All rights reserved.

Details

ISSN :
23301619
Volume :
4
Database :
OpenAIRE
Journal :
Movement Disorders Clinical Practice
Accession number :
edsair.doi.dedup.....3458bce1a89294b572bb9277d8e77b63