Acute disseminated encephalomyelitis in children. A descriptive study in Tehran, Iran

Objectives: To determine the frequency, etiology (viral infection or vaccination), presenting signs and symptoms, response to therapy, complication and course of acute disseminated encephalomyelitis (ADEM) in our hospitals. Methods: A 2-year retrospective, descriptive, chart review of children with final diagnosis of ADEM in 2 hospitals (Hazrat Rasool and Mofid in Tehran, Iran during 2000-2002) were carried out. The diagnosis is based upon clinical presentation, physical examination and ruling out of other disease (imaging, laboratories and so forth) of expert pediatric neurologists. Acute disseminated encephalomyelitis was documented in all cases by characteristics MRI changes included inflammation and demyelination in subcortical or periventricular regions. Results: Acute disseminated encephalomyelitis were diagnosed in 15 patients. More than half of patients were between 9-14 years old. It was rare in 1-5 years old children. It had an abrupt onset, preceding infection/vaccination with no gender differences. Approximately 46.4 of cases had a recent upper respiratory tract illness. Varicella zoster virus infection, urinary tract infection, and mycoplasma pneumoniae were observed. Presentation signs included ataxia, decreased consciousness, fever plus nausea/vomiting, cranial nerve involvement, dysarthric speech, convulsion, hemiparesis, paresthesia, meningismus, and headache. We identified inflammation and demyelination in subcortical than periventricular lesions by magnetic resonance imaging. Prognosis was excellent with low mortality rate (6.6). Conclusion: Acute disseminated encephalomyelitis is common in our children, possibly because of the high prevalence of causative infections. Due to advances in control of traditional exanthematous diseases such as measle, rubella and so forth, most cases of ADEM in this study followed non-specific upper respiratory infections. Differentiation of ADEM from a single episode of multiple sclerosis is difficult. Diagnosis of multiple sclerosis should be carried out if new symptoms and signs or imaging abnormalities appear, more than 3 months after the onset of clinical symptoms in ADEM cases

Immunity status in children with Bacille Calmette-Guérin adenitis. A prospective study in Tehran, Iran

Objective: To determine the immunity status of children with Bacille Calmette-Guerin (BCG) lymphadenitis (patient group) and unaffected children (control group) in Iran. Methods: We performed this longitudinal case-control study on 75 children between 2 months to 14 years old in Rasool Akram and Markaz Tebbi Hospital, Tehran, Iran during the period of 2 years (2000-2002). Results: Ninety percent of patients had normal immunoglobulin, 10 had low level, 96.1 had normal nitro blue tetrazolium test and 3.9 had lower activity. There was a significant difference in the total lymphocyte CD3, CD8, CD19, CD16/CD56 and natural killers (NK) cell but no significant difference in the CD4/CD8 ratio and CD4 between case (n=75) and control (n=100) groups. Thirty-eight cases with mild lymphopenia, isolated CD4, CD3, CD19, NK cells (CD16/CD56) deficiency in 3 (22); idiopathic disseminated BCG infection (unknown immunodeficiency type) in 3 (22) patients were observed. Thirty-eight cases were diagnosed as mild immune deficient without any previous recurrent infections (mild lymphopenia; Isolated CD4; CD3 or CD19 deficiency. Natural killers (CD16/CD56) deficiency in 3 (22); idiopathic disseminated BCG infection (unknown immunodeficiency type) in 3 (22) patients. The natural killers (CD16/CD56) deficient cases responded well to 3 antimycobacterial drugs without immunomodulator. Natural killers cell deficiency not yet reported as a risk factor for progression and complication of BCG infection. All cases of idiopathic disseminated BCG infection (unknown immunodeficiency type) with nonlethal and indulgent BCG infections responded well to needlle aspiration and antimycobacterial drugs with immunomodulator (gamma interferon). Conclusion: In cases with multiple and recurrent BCG lymphadenitis without any previous recurrent infection complete immunological studies should be carried out. Most cases with mild immune deficiency usually response well to needle aspiration alone or combine with antimycobacterial drugs. The combination of IFN-gamma and chemotherapy in cases of idiopathic disseminated infections caused by BCG and without previous recurrent other infection except mycobacterium species, can limit the disease

Role of cytomegalovirus in sensorineural hearing loss of children: A case-control study Tehran, Iran

Background: Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss in children. More than 40 of deafness cases with an unknown cause are due to congenital CMV. When CMV infection is diagnosed in the first year of life, treatment with gancyclovir is recommended and is associated with diminished occurrence of SNHL. Previous studies in Tehran showed congenital CMV infection developed in 2.6 of neonates. Objective: To determine the role of cytomegalovirus in sensorineural hearing loss in children by comparing the CMV antibodies quantitatively between SNHL and controls. Methods and materials: This case-control study was done in the ENT Department of Hazrat Rasoul Akram Hospital in Tehran (2002-2003). This study was carried out based on diagnostic parameters (AAO criteria) for SNHL and a healthy control. We compared the specific cytomegalovirus antibodies (IgM and IgG measured by ELISA method) in 95 blood samples of infants with SNHL (mean age = 35 ± 30 months) and 63 healthy matched infants (mean age = 38.7 ± 27.3 months) as controls. 59.6 of cases were male; 40.4 were female. Acute and previous immunity to cytomegalovirus (IgM and IgG) was found in 34.7 and 72.6 of SNHL children, acute infection detected in 3.5 of controls, previous immunity (IgG) detected in 94.7 of them. Acute CMV infection was higher in the cases (P-value = 0.000) but previous immunity (CMV-IgG) was higher in the controls (P-value = 0.001). Mean age of cases with acute infection (CMV-IgM) was 40 months and for previous immunity (CMV-IgG) was 35 months in SNHL cases and 40 months in controls, respectively. Conclusion: Cytomegalovirus is one of the most common infectious agents in SNHL children compared to the healthy children. Probably both congenital and acquired CMV can induce progressive hearing loss in our cases. We prefer at least in our country to consider seropositive (CMV-IgM) SNHL children (less than 1 year old) as congenital form. But we are not able to differentiate the congenital from the acquired infection in seropositive (CMV-IgM) SNHL children after first year of life. It should be subjected to randomized controlled trial for treatment of acquired type of CMV infection in SNHL children with ganciclovir. © 2007 Elsevier Ireland Ltd. All rights reserved

Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection

The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech-language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 +/- 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good