J Clin Neurol.  2007 Sep;3(3):150-153. 10.3988/jcn.2007.3.3.150.

Focal Hand Dystonia Secondary to Basal Ganglia Germinoma

• Kim JS
• Han SJ
• Kim W
• Kim BS
• Kim YI
• Lee KS

• KMID: 2135494
• DOI: http://doi.org/10.3988/jcn.2007.3.3.150

Abstract

Descriptions of symptomatic focal dystonia caused by focal lesions of the central nervous system (CNS) are rare in the literature. We report a 9-year-old child who experienced sudden-onset left-hand dystonia for 6 months. Brain magnetic resonance imaging showed a mass lesion involving the putamen, globus pallidus, head of caudate, and the anterior limb of the internal capsule. Histopathological and immunocytochemical examinations of the mass revealed features characteristic of malignant germinoma. CNS germinoma in the basal ganglia is very rare. Combining previous reports in the literature with the anatomical and clinical presentation of our case suggests that this phenomenon results from disruption of the pathways within and adjacent to the basal ganglia.

Keyword

Basal ganglia; Germinoma; Focal dystonia

MeSH Terms

Basal Ganglia*
Brain
Central Nervous System
Child
Dystonia*
Dystonic Disorders
Extremities
Germinoma*
Globus Pallidus
Hand*
Head
Humans
Internal Capsule
Magnetic Resonance Imaging
Putamen

Figure

• 

  Figure 1 Photograph showing dystonia of the left hand during arm extension (A) and playing the piano (B, C).

• 

  Figure 2 Magnetic resonance imaging showing an ill-defined mass lesion involving the right basal ganglia. The lesion was located in the putamen, globus pallidus, head of caudate, and the anterior limb of the internal capsule. (A) Axial T2- weighted images, and (B) axial T1-weighted contrast enhancement images.

• 

  Figure 3 Positron-emission tomography using 18F-deoxyglucose demonstrated diffuse hypometabolism in the right basal ganglia, right thalamus, and right insular and temporal cortices.

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  Figure 4 Histopathological and immunohistochemical findings revealed (A) large tumorous cells (germ cells) with a clear nucleus and a light-colored and vacuolated cytoplasm (hematoxylin-eosin stain, ×400) and (B) a placental alkaline phosphatase (PLAP) profile with large tumorous cells labeled for PLAP in the cell membrane and cytoplasm (×400).

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