Cerebral Malakoplakia in a Patient with a Background of Common Variable Immune Deficiency Treated with Intravenous Antibiotics: A Case Report

Abstract

Cerebral Malakoplakia is an extremely uncommon entity within the realm of neuroinflammatory disorders, characterized by the formation of distinctive Michaelis-Gutmann bodies within macrophages. Malakoplakia was first described in the genitourinary tract but has been sporadically reported in various extra genital sites, including the brain. These reports remain sparse due to the rarity of cerebral malakoplakia, making each documented case a valuable contribution to our understanding of this intriguing condition. It originates from the Greek words “malakos” (soft) and “plakos” (plaque) [1]. Important Clinic Findings : We present a 20-year-old patient with a background of Common Variable Immune deficiency, epilepsy and Evans Syndrome complicated with recurrent pancytopenia since early childhood who is on monthly treatment with IVIG and usually receives rituximab if his platelet dropped. The primary hospital presentation was a sudden and severe occipital headache, neck pain and vomiting. His clinical examination was insignificant. Routine blood, including inflammatory markers, ANA, ANCA, immunoglobulins, and C, C4, were all normal. CSF analysis failed to identify any organism. Diagnoses and M anagement: MRI brain revealed enhancing left cerebellar mass with surrounding oedema, mass effect with effacement of the fourth ventricle and acute hydrocephalus. Accordingly, he was referred to neurosurgery centre as a suspected metastatic versus primary cerebellar tumour for biopsy and further management. Craniotomy and biopsy were conducted and the histopathology was reported as leptomeningeal and cerebellar lesion, favour inflammatory, dominated by oncolytic histiocytic with central area of necrosis, suggestive of malakoplakia. Accordingly, he received treatment with different regimens of intravenous antibiotics based on previous case reports with similar diagnoses and his repeated MRI brain showed initial debulking of the brain mass, which later became static in size. Conclusion: Although it is very rare, Cerebral Malakoplakia should be considered in patients who are immunocompromised and presented with symptoms, signs and brain imaging that are suggestive of intracerebral tumours with or without a mass effect. Several case reports, including this one, have demonstrated good response to antibiotics as treatment for this condition.