Objective: This case report aims to describe a rare presentation of Olivopontocerebellar atrophy in HIV related disease.

Background: Olivopontocerebellar atrophy (OPCA) is the term describe the degeneration of neuron lead to demyelination and  fibrillary gliosis of the cerebellar white matter, and formation of olivocerebellar, external arcuate, and pontocerebellar fibers. OPCA may be the pathological hallmark of neurodegenerative diseases, including hereditary and sporadic ataxias, multiple system atrophy ( MSA) , prion disorders, and hereditary metabolic diseases but none of report in HIV related disease.

Method: case Presentation : A 29-year-old vertical transmitted HIV infected female who was in viral suppressed status  was consulted due to behavior change for a year. She developed pathologic crying in every time speaking without feeling of depression , sadness or suicidal idea. Physical examination showed mild symmetrical parkinsonism , scanning speech , mild cerebellar ataxia , hyporeflexia  and non emotional crying.

Results: Brain MRI revealed significant pontine and diffuse bilateral cerebellar atrophy associated with cruciform hyperintensity T2FLAIR in an atrophic area compatible with Olivopontocerebellar atrophy ( OPCA) and hot cross bun (HCB) sign. Blood test for autoimmune and paraneoplastic antibodies all were negative . Cerebrospinal fluid ( CSF)  examination was normal but was  limited for sending CSF HIV viral load and JC virus. Genetic testing was absent of repeated expansion for SCA 1,2,3 and SCA 17. According to history of one year non viral suppressed HIV disease prior to her clinical presentation, the diagnosis was HIV encephalopathy with olivopontocerebellar atrophy.  This brainstem pathology interfere central to peripheral autonomic network of crying and explain of distress vocalization of non emotional pathological crying of her.

Conclusion: From my knowledge, this is the first case of HIV infected disease who presented with non emotional pathologic crying which is atypical presentation of Olivopontocerebellar atrophy. OPCA affect to cerebro-ponto-cerebellar to peripheral pathway of pathological non emotional crying.

Neurobiology of crying

MRI brain of this patient with OPCA and HCB sign

OPCA classification

OPCA and HCB sign differential diagnosis

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MDS Abstracts - https://www.mdsabstracts.org/abstract/olivopontocerebellar-atrophy-and-hot-cross-bun-sign-in-vertical-transmitted-hiv-disease-with-non-emotional-pathological-crying-case-report-and-review-literature/