A 57-year old immunocompromised Caucasian woman with a diagnosis of SLE in stable remission for the last nine years and treated with low dose prednisone was admitted to the Internal Medicine Ward because of a poor general condition lasting for seven days and weakness in the right leg without sensory deficit lasting for two days. During that time, she became disorientated and confused, while meningeal signs were negative. Laboratory tests showed a sedimentation rate of 26 mm/h, leucocytes 3.9 × 109/L with neutrophilia, erythrocytes 4.38 × 1012/L with thrombocytopenia of 125 × 109/L, mild liver lesion with aspartate aminotransferase (AST) 43 U/L, and alanine aminotransferase (ALT) 71 U/L. C3 and C4 complement components were normal, as well as immunoglobulins level (IgA 0.91 g/L, IgG 10.1 g/L, and IgM 1.67 g/L). Antiphospholipid antibodies were positive (aCL-IgM 69.1 MPL/mL, aCL-IgG 88.8 GPL/mL), ANA titar (IIF) was 1:1280, and dsDNA (ELISA) 52 IU/mL (normal < 30 IU/mL). An urgent brain MSCT was performed and showed discrete hypodense lesions of the left perinsular and hippocampal region. Due to the previous medical history, clinical presentation and initial imaging findings, central nervous system (CNS) lupus, and subacute ischemic cerebrovascular disease were suspected. Follow-up CT after 5 days showed a new lacunar hypodense lesion in the left cerebellar peduncle. Next, MR angiography was performed excluding signs of vascular anomalies, ischemia, and vasculitis. In the second week, the patient showed further deterioration of clinical symptoms with a decrease in the leucocyte count (L 2.7 × 109/L), normocytic anaemia (hemoglobin 109 g/L), and thrombocytopenia (platelets 92 × 109/L). C3 was 0.83 g/L and C4 was normal. Brain MRI in the second week of hospitalization revealed the full extent of brain impairment: multiple parenchymal supratentorial and brainstem demyelinating lesions on T2 and FLAIR sequences without postcontrast enhancement on T1WI ().\nSince we had an immunocompromised patient, CMV serology (ELISA) was also carried out with both IgM and IgG resulting in positive as well as blood CMV polymerase chain reaction (PCR) that revealed 4340 CMV copies per milliliter. A lumbar puncture was performed. Cerebrospinal fluid (CSF) analysis showed elevated protein at 1567 mg/L with normal glucose and lactate, 6 leucocytes/mm3, 645/mm3 erythrocytes, and negative bacterial culture. PCR detected CMV DNA in the CSF (<1000 copies/mL), while PCR for HSV and VZV were negative. There was no intrathecal oligoclonal bands synthesis. Clinical findings, course of the disease, laboratory tests (lower leucocytes, thrombocytopenia, hepatal lesion), neuroradiological features along with serology, and PCR tests suggested an ADEM associated with CMV reactivation. Antiviral therapy with gancyclovir and subsequently with peroral valganciclovir was administered for 29 days in total (until CMV PCR came negative in CSF and blood). High doses of intravenous methylprednisolone (125 mg intravenously for five days with consequent gradual dose reduction) and intravenous immunoglobulin (IVIG) 0.4 g/kg daily for three days were started simultaneously.\nThe patient‘s neurological status gradually improved. At the discharge, almost a month after admission, the patient was completely conscious with improved right leg paresis. Follow-up brain MRI, three weeks after the initial one, revealed partial regression of previously described demyelinating lesions. Further, a follow-up clinical examination 6 months after hospitalization showed mild right leg paresis and complete MRI regression of demyelinating lesions () Our patient had no similar recurrent attacks since.
We present a clinical case of a 31-year-old man diagnosed with HIV-1 infection, with CD4 T cell count of 35 cells/mm3 (4%) and HIV RNA 305349 copies/mL (log10 5.48) having initiated ART with abacavir/lamivudine and nevirapine. Around two weeks after starting ART, the patient is admitted due to a sudden cognitive impairment (anhedonia and memory loss) with progression to gait change and imbalance. The cranial computerized tomography (CT) scan showed no lesions but the cranial MRI revealed ventriculoencephalitis ().\nThe cerebrospinal fluid (CSF) had 38 nucleated cells/mm3, 175 mg/dL proteins and 37 mg/dL glucose (glycaemia 82 mg/dL). The CSF CMV and EBV viral load were 189000 (log10 5.28) and 799 (log10 2.90) copies/mL with negative CSF neurotropic microorganism serologies and molecular identification (HSV 1/2, VZV, Cryptococcus, Brucella, Treponema pallidum, Borrelia burgdorferi, JC virus, Mycobacterium tuberculosis, and Toxoplasma gondii). The final considered diagnostic was mainly CMV-related ventriculoencephalitis and ganciclovir was started.\nNevertheless, the patient started left conjugate horizontal gaze palsy with abducting horizontal saccadic (or jerk-type) nystagmus of the right eye as well as a slight anisocoria with left eye miosis. These changes were enclosed in the one-and-a-half syndrome and left-sided Horner's syndrome. The patient also presented a grade II-III paresis of the right lower limb. The cranial CT scan (performed fifteen days later) revealed a dubious right linear protuberancial hypodensity without signs of intracranial hypertension.\nCranial MRI was repeated one month later revealing improvement of the ventriculitis signs but a larger hippocampus and left mesial temporal region involvement with a discrete increase of the lateral ventricles dimensions.\nDue to these clinical and imagiological changes and because we could not exclude tuberculosis infection, classic first-line tuberculostatic therapy was empirically started (stopped after excluding this infection) and foscarnet was added to ganciclovir (until a negative CMV viral load was achieved). At this point, the hypotheses of limbic encephalitis, epileptic activity or paraneoplastic encephalitis could not be excluded. The lumbar puncture was repeated and CSF antineuronal antibodies, HHV-8, and other neurotropic microorganisms were negative.\nThe electroencephalogram (EEG) showed frontal and frontotemporal bilateral activity with occasional periodic discharges, independent of nonabrupt three-phase bifrontal lesions. Anticonvulsant therapy with levetiracetam and topiramate was started. One month later, the cranial MRI was repeated and a new frontal lesion was detected. The body CT scan was normal. The imagiological reevaluation showed a slight improvement of the frontal lesion with new cerebellum lesions. The suspicion of lymphoproliferative disease was meaningful. Cranial MRI with diffusion and perfusion study with spectroscopy was performed and revealed very suggestive images of CNS lymphoma (). For definitive diagnosis, a stereotactic brain biopsy was done. The histological study revealed a polymorphic cellular infiltrate with histiocytes, CD3+ T-lymphocytes, and CD 20+ B-lymphocytes (mixture of numerous small cells and large activated cells with positive hybridization results for EBV-encoded RNA (EBER)). The histology was suggestive of polymorphic lymphoproliferative EBV-positive disease. The molecular studies were not done because the sample was insufficient to perform the clonality analysis (CSF EBV viral load was 12405 copies/mL; CSF HIV and CMV viral load were both undetectable).\nAt this point, chemotherapy with rituximab was started and radiotherapy was scheduled. Nevertheless, the patient developed a sudden neurological worsening with a generalized tonic-clonic seizure, refractory to the instituted measures, culminating in death.