Cerebellar Ataxia as a Rare Presenting Manifestation of Juvenile Systemic Lupus Erythematosus: A Case Report
A. Lotfi *
Department of Pediatric Rheumatology, Children’s Hospital, Rabat, Morocco.
K. Tmara
Department of Pediatric Rheumatology, Children’s Hospital, Rabat, Morocco.
K. Mouadin
Department of Pediatric Rheumatology, Children’s Hospital, Rabat, Morocco.
C. Nahi
Department of Pediatric Rheumatology, Children’s Hospital, Rabat, Morocco.
B. Chkirat
Department of Pediatric Rheumatology, Children’s Hospital, Rabat, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Aims: To describe cerebellar ataxia as a rare presenting manifestation of juvenile systemic lupus erythematosus (jSLE) and to emphasise the diagnostic value of associated systemic findings.
Presentation of Case: An 11-year-old previously healthy girl presented with a facial eruption and gait disturbance. Examination revealed a malar rash, frontal cicatricial alopecia, chilblain-like finger lesions, hypomimia, a predominantly left-sided resting tremor, broad-based gait ataxia, motor incoordination, and involuntary blinking. Laboratory investigations demonstrated normocytic normochromic anaemia, severe thrombocytopenia, marked inflammatory activity, hypocomplementaemia, a positive direct Coombs test, and positive ANA, anti-dsDNA, anti-Sm, anti-SSA, anti-ribosomal P, and anti-RNP antibodies. The infectious work-up was negative. Brain computed tomography was normal, while magnetic resonance imaging showed widening of the cerebral cortical and cerebellar sulci, suggestive of cerebral and cerebellar volume loss. The patient received intravenous methylprednisolone pulses followed by oral corticosteroids and antimalarial therapy, with subsequent improvement in cutaneous lesions, resting tremor, gait ataxia, motor coordination, and laboratory abnormalities.
Discussion: Cerebellar ataxia is an exceptionally uncommon manifestation of SLE, particularly as an initial presentation in childhood. Attribution to lupus requires exclusion of alternative causes and integration of neurological, systemic, immunological, and neuroimaging findings.
Conclusion: jSLE should be considered in children with otherwise unexplained cerebellar ataxia or movement abnormalities when accompanying mucocutaneous, haematological, or immunological features are present.
Keywords: Cerebellar ataxia, juvenile systemic lupus erythematosus, neuropsychiatric lupus, resting tremor, hypomimia, movement disorder, cerebellar dysfunction, autoantibodies, child, magnetic resonance imaging