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.
A 17-year-old female presented with a history of flu-like symptoms (low-grade fever and body aches). One week after the initial presentation, she experienced a rapidly progressive and symmetric weakness in all four extremities. There was no visual or sensory impairment. No abdominal, respiratory, or cardiovascular symptoms were reported. The past medical history was negative, and the patient had no previous surgery. There was no current medication or recent vaccination history. She was not sexually active, and there was no environmental or sickly contact exposure. Subsequently, the patient became lethargic and progressed to a coma. She did not have seizures. On admission, the neurologic examination showed no response to verbal and pain stimuli. There was no neck rigidity. Kernig's sign was negative. A Babinski's sign was noticed in both extremities, along with clonus. Hyperreflexia was elicited in all four limbs. There was a maculopapular rash all over the trunk and extremities.\nA list of differentials were considered, which included infective meningitis, subarachnoid hemorrhage, encephalitis, multiple sclerosis, neuromyelitis optica (NMO), central nervous system (CNS) vasculitis, brain tumor, acute intoxication, and metabolic/endocrinologic conditions. A complete blood count and basic metabolic panels were normal. Urine toxicologic screening was negative. Head computed tomography (CT) showed no abnormality, and cerebrospinal fluid (CSF) analysis was unremarkable. Herpes virus, human immunodeficiency virus (HIV), CMV, rubeola, measles, and hepatitis serology panels were pending. NMO-immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG) antibodies were not done due to technical conditions.\nShe was put on acyclovir, ceftriaxone, and vancomycin to cover both bacterial meningitis and herpetic encephalitis. Subsequently, HIV and serologies for hepatitis, rubella, and measles were negative. Polymerase chain reaction (PCR) for herpes virus in the CSF was negative as well. However, serum IgG and immunoglobulin M (IgM) for CMV demonstrated high titles, confirming recent systemic infection. A lumbar puncture was repeated, and at this time, the CSF showed a severe hypercellularity of 426 white cells/μL (normal range: 0 - 5 cells/μL) with 65% lymphocytes and 62 mg/dL of protein (normal range: 15 - 60 mg/dL). A glucose level was 95 mg/dL (normal range: 50 - 80 mg/dL). CSF oligoclonal bands (OCB) were not detected. MRI of the brain demonstrated multifocal, poorly marginated, and extensive areas of hyperintense signal on fluid-attenuated inversion recovery (FLAIR) sequences in the white matter, asymmetrically distributed in both hemispheres, as well as in the brainstem and cerebellar peduncles, compatible with acute demyelination lesions (Figure ). Cervical and thoracic spine MRI scans did not demonstrate an altered signal.\nAt this point, acute disseminated encephalomyelitis (ADEM) was diagnosed, and pulse therapy with methylprednisolone (15 mg/kg/day) was initiated for five days. On the third day of treatment, the patient showed neurologic improvement with the recovery of consciousness. She was able to move all four limbs and muscle power became normal. She had progressive neurologic recovery and was discharged after seven days with no motor deficits and mild cognitive decline.