Category: Rare Genetic and Metabolic Diseases
Objective: A 17 years-old male develops rapidly progressive impairment of balance and gait, left sided weakness, falls, headache, vomiting, photophobia and phonophobia. No history of trauma, infections, use of medications or migraine. Family history is unremarkable.
Background: Focal neurological deficits in young patients are often associated to infectious, demyelinating, vascular or neoplastic lesions in the central nervous system (CNS), often requiring extensive evaluation with disease progression. Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare inflammatory disease of unclear pathogenesis involving the brainstem, cerebellum and less commonly supratentorial regions of the brain with a characteristic radiologic pattern. Rapid progression may obscure the diagnosis requiring considering alternate etiologies. We describe a challenging case of an aggressive presentation of CLIPPERS.
Method: Case Report: neurological examination, laboratories, imaging performed
Mental status: awake, oriented, depressed mood
Cranial nerves: left nasolabial flattening
Motor: left spastic hemiparesis progressing to hemiplegia, spasticity left upper and lower limbs
DTR’S: hypereflexia worse in the left, left corticospinal sign
Station/Gait: left hemiplegic gait; loss of balance
Results: Work-up for infection, malignancy, demyelinating disease, autoimmune disease and neurosarcoidosis was unremarkable.
CSF: lymphocytic pleocytosis, increased protein
Brain MRI with gadolinium: enhancing lesion with associated edema and mild mass effect involving the right basal ganglia, thalamus, hypothalamus, cerebral peduncle, midbrain, and pons with areas of nodular and punctate contrast enhancement and early obstructive hydrocephalus consistent with CLIPPERS.
The patient received high dose intravenous corticosteroids with marked clinical and radiological improvement supporting a diagnosis of CLIPPERS. After steroid treatment was completed, stepwise deterioration was observed responding to corticosteroids treatment.
Conclusion: This case of rapidly progressive spastic hemiplegia demonstrates the challenges in the diagnosis of CLIPPERS. Unique to this case is the young age and focal findings. Early recognition of symptoms and the characteristic brain MRI findings are important as the condition can rapidly improve with corticosteroids. Close monitoring after treatment is essential to identify and treat relapses.
To cite this abstract in AMA style:J. Abreu, C. Serrano, D. Reyes, B. Deliz, E. Labat. A young man with rapidly progressive spastic hemiplegia. [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/a-young-man-with-rapidly-progressive-spastic-hemiplegia/. Accessed December 3, 2023.
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