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 forty year old previously healthy Sri Lankan male presented with acute onset bilateral leg weakness which was progressive over two days following a brief febrile illness of three days. He did not give any history of trauma nor there was any history of tuberculosis. Initially he did not have urinary retention nor there was any sensory symptoms. He did not complain of shortness of breathing.\nOn examination he was found to have asymmetric flaccid paralysis of bilateral lower limbs with power grade one to two. Reflexes were diminished bilaterally and Babinski sign was equivocal. Examination of optic fundi and cranial nerves were normal. There was no demonstrable sensory level and joint position sense was initially preserved. Upper limbs were neurologically normal. Respiratory movements and sphincter function were preserved. Depending on initial findings a clinical diagnosis of Guillain-Barré syndrome was suspected and he was closely observed for the development of respiratory paralysis with monitoring of forced vital capacity and oxygen saturation.\nOn the fifth day of onset of symptoms his weakness worsened with additional flaccid paralysis of upper limbs and neck muscles. He was catheterized for acute retention of urine. Patient was urgently transferred to a high dependency unit but he did not develop respiratory paralysis necessitating ventilator support. Later he developed bilateral spastic paraparesis and positive Babinski sign with mild paraesthesia of lower limbs. But he did not develop a definite sensory level at anytime during the course of his illness.\nA nerve conduction study failed to show demyelination or axonal loss. Magnetic resonance imaging study of the spine showed hyperintensity of central portion of mid cervical cord from C 2 to C 5 level in T2 weighted and FLAIR images (Figure \n) There was mild swelling of cervical cord without significant change of signal intensity on T1 weighted image (Figure \n).\nBasic investigations were as follows; Na+ 139 mmol/l, K+-4.2 mmol/l, Serum creatinine-62 μmol/l, blood urea 8.7 mmol/l, White cell count 5000/mm3(N-76,L-18), platelet count 17500/mm3. Haemoglobin 9.3 g/dl, AST-51u/l, ALT-74u/l, Bilirubin 12 μmol/l, ESR-12 mm/hr, CRP-0.4 mg/dl, VDRL-negative, Fasting blood sugar 5.4 mmol/l.\nCerebrospinal fluid analysis done on the tenth day after onset of weakness was suggestive of a viral CNS infection with a protein content of 300 mg/l, glcose 4.3 mmol/l(RBS 10.9 mmol/l) and a leukocyte count of 350/mm3(92% lymphocytes). CSF infectious screen was positive for cytomegalovirus PCR(sensitivity more than 100copies/ml). This test was repeated and result was confirmed. Cytomegalovirus IgG and IgM were not detected in CSF. Polymerase chain reaction of CSF for herpes simplex virus(HSV) and Japanese encephalitis virus(JEV) were negative. Oligoclonal bands were not detected in CSF.\nConsidering the overall clinical picture, a working diagnosis of CMV associated transverse myelitis was made. Immunoglobulin levels and lymphocyte subset analysis failed to identify any immunodeficiency while HIV serology was negative.\nPatient received treatment with intravenous ganciclovir for a duration of 21 days starting on the 10th day of illness. He was also given maximal supportive care with physiotherapy and psychological support. A second CSF analysis done 22 days after the onset of symptoms revealed no cells and protein content of 390 mg/l. (CSF glucose 3.6 mmol/l RBS-6.5 mmol/l) Repeated CMV serology in blood, CSF and CSF PCR for CMV were negative on the 22nd day of illness.\nOne month after the onset of symptoms the upper limb power was improved (grade 5) but the lower limb power remained grade 3. He was transferred to a long term rehabilitation facility.