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 29 years-old man was admitted to our neurological department with five months of progressive disability in walking, paresthesia in the arms when bending the neck, numbness in the hands, and back pain. He had been treated for many years with aerosol salbutamol during exacerbations of asthma. Apart from periodic jaw spasms during the last five years, there were no apparent previous illnesses and no suspicion of infection within the last six months. During the last two years, he had been successfully treated for his injectable drug abuse with daily oral methadone. The patient had had negative hepatitis C virus RNA (HCV) serology two years before presentation. The neurological examination revealed positive Lhermitte`s sign, hypoesthesia in the hands, normal muscle strength in the upper limbs, reduced muscle strength in the legs (4 on a MRC scale), and reduced dorsiflexion of the feet (4 on a MRC scale). In addition, the patient had severe increased spasticity in the extensor muscles of the lower limbs, bilateral-positive Babinski, and a broad-based gait requiring support.\nSpinal cord MRI demonstrated extensive longitudinal spinal cord lesions from the cervical vertebral segment C1 to C5 and thoracic vertebral segment T2 to T11 (Figure ). The cervical lesion was located in the dorsal part and over a short distance in the lateral part on the right side on T2-weighted images. Discrete enhancement was found in the posterior part. Changes in the thoracic part were seen almost over the cord's entire cross section on the T2-weighted images, but enhancement was only demonstrated in the lateral part on both sides. The brain MRI was normal.\nA lumbar puncture was performed with cerebrospinal fluid showing slight pleocytosis with 11 mononuclear cells, a slightly increased protein to 0.64 g/l, normal IgG index and no oligoclonal bands, no antibodies against the varicella-zoster virus, herpes virus, entero-virus, or borrelia. The blood tests showed normal B12 vitamin, rheumatoid factor IgM and ANA, negative HIV serology, negative syphilis RPR, negative TB gamma interferon release assay, negative MOG- and AQP-4 antibodies, and increased ALAT to 200 U/l. Tests for HCV serology and HCV RNA were both positive.\nTreatment of HCV infection with Zepatier (elbasvir 50 mg and grazoprevir 100 mg) one tablet daily was initiated and continued for three months. Follow-up blood tests showed normalization of ALAT, negative HCV serology, and normalization of the spinal cord changes on MRI. The neurological findings were unchanged. There was no clinical suspicion of rheumatic, vascular or skin diseases and normal thyroid, white blood cells and renal function tests. With that condition, we found no indication for steroid treatment also taking into account previous episodes of psychosis in relation to his drug abuse.\nDespite a four-week course of inpatient rehabilitation, the physical condition was only slightly improved. Currently (two years later), walking distance is reduced, a stick is required when walking outside, and medication is still necessary for spasticity and neuropathic pain in the legs.