Category: Parkinsonism, Others
Objective: To describe a unique case of a healthy 21-year-old female patient diagnosed with idiopathic rhombencephalitis with predominant parkinsonian clinical signs who later developed severe motor fluctuations under dopaminergic treatment and now is a candidate for subthalamic nucleus (STN) deep brain stimulation (DBS).
Background: Encephalitis involving the substantia nigra (SN) causing parkinsonism has been previously described. Numerous neurotropic viruses have been shown to cause transient or permanent parkinsonism such as Influenza, Coxsacki, Japanese encephalitis B, St. Louis, West Nile, HIV and Epstein Barr.
Method: Electronic medical records (EMR) were used to retrieve relevant clinical, laboratory and imaging data.
Results: Our patient presented with a 3-day history of headache and systemic fever. Neurological examination upon arrival was positive for opsoclonus-myoclonus syndrome, pyramidal, parkinsonian and cerebellar signs. Lumbar puncture showed pleocytosis of 800 cells/mm3 and elevated protein. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) panels serology and cultures were negative for common viral and other infectious etiology, as well as autoimmune and paraneoplastic panels. Brain magnetic resonance imaging (MRI) showed high T2-Fluid-attenuated inversion recovery (FLAIR) signal at the brainstem, and in particular the midbrain SN, as well as cerebellum. Patient was treated with prednisone, plasmapheresis and symptomatic dopaminergic therapy with gradual improvement over one month. In the following months, under dopaminergic therapy including L-dopa she developed motor fluctuations with severe OFF-dystonia and hypokinesia and on the other hand severe On-dyskinesias. A year after the encephalitic presentation the CSF was normal, brain MRI showed chronic cystic hyperintense signals of the SN, positron emission tomography (PET) imaging with 6-[18F]fluoro-L-dopa (FDOPA) showed severe bilateral striatal tracer reduction, in accordance with the clinical picture. She is currently a candidate for STN-DBS electrode implantation.
Conclusion: Acute parkinsonism may progress rapidly to a state of advanced fluctuating parkinsonian syndrome as a sequel of rhombencephalitis.
To cite this abstract in AMA style:
S. Anis, H. Abu-Amer, T. Fay-Karmon, S. Hassin-Baer. Idiopathic rhombencephalitis involving the substantia nigra followed by rapidly progressive parkinsonian syndrome in a young patient [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/idiopathic-rhombencephalitis-involving-the-substantia-nigra-followed-by-rapidly-progressive-parkinsonian-syndrome-in-a-young-patient/. Accessed June 15, 2025.« Back to MDS Virtual Congress 2021
MDS Abstracts - https://www.mdsabstracts.org/abstract/idiopathic-rhombencephalitis-involving-the-substantia-nigra-followed-by-rapidly-progressive-parkinsonian-syndrome-in-a-young-patient/