Objective: To describe a case report of a rare cause of Parkinsonism

Background: Vascular malformations such as cerebral cavernomas are known to predispose to haemorrhagic strokes, seizures, and focal neurological deficits. Very few cases in the literature describe Parkinsonism caused by Cavernomas.The present case describes a patient with multiple Cavernomas, the largest in the left midbrain involving substantia nigra and nigrostriatal pathway leading to Parkinsonian symptoms.

A 73-year-old lady presented to the movement disorder clinic for evaluation of unsteady gait. Her other symptoms included micrographia, stiffness and slowness in her movements, constipation, and minor memory problems. Her examination showed bradykinesia with mild to moderate rigidity in the right lower limb and normal facial expression. Her gait showed dragging of the right foot, with mild reduction in right arm swing.

DaT scan was markedly abnormal with nearly abolished uptake throughout left striatum suggestive of a structural cause.

The MRI did not show any striatal abnormality. However, there were multiple Cavernomas, the largest one extending from the left midbrain to the thalamus and involving the left substantia nigra and nigrostriatal pathway. Smaller ones were found in brain stem and cerebellum.

At one year follow-up, she had developed mild resting tremor in her right hand without significant worsening of her preceding symptoms. She was started on 125 mg Levodopa/Carbidopa TDS. However, she did not report subjective improvement of her symptoms.

Method: A search for similar cases was made in PubMed, Google scholar and embase databases

Results: Although there are cases of multiple cavernomas described in unusual locations such as brain stem, basal ganglia and thalamus in the medical literature, presentation with symptoms of Parkinsonism is uncommon. Since the repeat MRI after 2 years showed no change in the lesion, we have decided to adopt a wait and watch approach.

Conclusion: Cavernomas of the midbrain involving substantia nigra and its projecting fibres can cause contralateral parkinsonian symptoms, poorly responsive to Levodopa. Management is still controversial with some centres preferring stereotactic radiosurgery for deep-seated cavernomas. A case-by-case approach is needed.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/case-report-cavernoma-involving-substantia-nigra-and-nigrostriatal-pathway-as-a-rare-cause-of-secondary-parkinsonism-with-a-3-year-follow-up/