Levodopa-Dependent Movement Disorder and Oligoastrocytoma Double Trouble or Dependent from Each Other?

Background: Inborn errors of neurotransmission and decreased function of the dopaminergic and serotoninergic systems could often lead to movement disturbances and impaired development.

The diagnosis is made by examination of the cerebrospinal fluid (CSF) and in many cases by genetic analysis. Besides genetical causes also parainflammational causes are known. A relationship between brain tumors like an oligoastrocytoma and neurotransmitter, respectively, Dopa-responsive disturbances is not known so far.

Case Report: A now 18-year-old young man was first presented at the age of 2 years due to developmental delay. At the age of 4 years, an ataxia was remarkable. In the further course, a left-sided accentuated spastic-dystonic movement abnormality, an inner ear hearing loss, and a restrictive ventilation problem were found and in spite of normal magnetic resonance imaging (MRI) a cerebral palsy was diagnosed. At the age of 8 years, a second MRI showed a left-sided subcortical gray matter change and a focal cortical dysplasia (FCD) was suspected. This finding was stable over 10 years. In the meantime, the boy was nearly unable to walk independently and became wheelchair dependent. CSF examination showed a decreased homovanillic acid- and 5-hydroxyindoleacetic acid-level. After substitution with Levodopa (5 mg/kg/d), the patient improved suddenly and was able to walk again independently. A phenylalanine challenge was without pathological results and could not confirm a Segawa disease. Meanwhile, focal epilepsy marked by aggression and disturbance of the short lasting memory was remarkable, it was successfully treated with levetiracetam. In the course of reevaluation, several MRIs were performed and the FCD has been stable until the age of 15 years. At the age of 18 years, the MRI showed a remarkable progress in size and dimension, histology showed an oligoastrocytoma WHO grade II, which was removed subtotally. Four months after the resection, controls of MRI have shown no growth of the tumor’s rest.

Conclusion: Disturbances of neurotransmission with a broad spectrum of symptoms are known, their causes can be variable. Because of the remarkable effects of Levodopa in our patient, but a normal phenylalanine challenge, no further genetic analysis was performed. No relation between this brain tumor and a movement disorder could be made. Our patient relies on Levodopa leading to dramatical improvement of independent walking. In contrast, a dystonic component and the residual findings caused by oligoastrocytoma and its subtotal resection remain.