ABSTRACT
Background and aims: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a defined inflammatory central nervous system disorder characterized by established lesions, predominantly on the cerebellum and pons, with punctate gadolinium enhancement on the MRI and responsiveness to glucocorticosteroid-based immunosuppression. Its pathogenesis is unknown. Methods: A 54-year-old man, with history of obesity and alcoholism, was admitted to our emergency ward due to fever, disorientation, and behavior disorder for 3 days. The neurological examination manifested left eye ptosis and unceasing left gaze torsional nystagmus. He tested positive for SARS-CoV2. A lumbar puncture revealed 41 leucocytes/ mm3, with negative antibody determinations. MRI depicted hyperintense lesions on cerebellum, brain stem, left temporal lobe and right parieto-occipital cortex, with punctate gadolinium enhancement. There was a radiological and clinical response to glucocorticosteroid treatment. He was diagnosed of possible CLIPPERS. Results: Several months following the discharge, after the patient voluntarily abandoned the treatment, the symptoms reappeared. A new MRI showed extensive lesions suitable with primary lymphoma. A biopsy confirmed the diagnosis. In situ hybridization detected Epstein-Barr virus (EBV). Conclusion: Reports describing the development of lymphoma on patients previously diagnosed with CLIPPERS, and the fact that both conditions share anatomopathological findings such as infiltrating CD4+ lymphocytes, support the theory of a syndrome with various prestages encompassing both entities. Furthermore, a nexus between B-cell lymphoma and EBV has already been established, but recent studies dwell on the possibility of this same infection being one of the main triggers of CLIPPERS and its progression into lymphoma.
ABSTRACT
Background and aims: SARS-Cov-2 disease could cause endothelial dysfunction, damage to the blood brain barrier and microvascular alteration, thus having the ability to cause different pathologies. Because these alterations can be subtle, they can be unnoticed except in susceptible individuals. Methods: We present the case of a 25-year-old male with history of Chiari type II malformation, severe triventricular hydrocephalus, ventriculoperitoneal bypass carrier with programmable valve and chronic kidney disease because of which, he has been on dialysis for several years. In April 2020, he developed severe pneumonia from SARS-CoV-2 infection, receiving treatment with Tocilizumab. After that, it presented several episodes during the initial phase of dialysis of decreased level of consciousness, arreactive pupils an minute-long decerebrate posture, without other associated clinic. Results: The EEG performed during the episodes showed no epileptic discharges and neuroimaging performed after the dialysis, showed no change from previous ones. Transcranial doppler was performed during the episodes, observing a high arterial pulsatility index which may reflect an increase in peripheral resistances, likely related to intracranial hypertension during the initial phase of dialysis that would cause the clinic. The patient was referred to neurosurgery for decrease of valvular pressure, with clinical resolution. Conclusion: The increase in intracranial pressure that occurs during the initial stages of dialysis, along with the baseline characteristics of the patient may be related to the clinic presented by this patient. This could lead to an alteration of secondary cerebral vasoregulation to possible endothelial dysfunction caused by the new coronavirus, which in other patients could go unnoticed.