فهرست مطالب mohammadmahdi nasehi

Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE’s safety and feasibility data in these conditions.

The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE’s efficacy was studied in these patients.

The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients.

Patients with PNID tolerated therapeutic plasma exchange, which was a safe process.

According to previous reports, between 2%-5% of multiple sclerosis (MS) cases occur before the age of 16 years. 

This study aims to investigate the clinical and epidemiological features of MS in Iranian children to provide diagnostic criteria for policymakers and physicians.

This study objectives are achieved in two phases. The first phase was conducted and the second phase is underway. The first phase was performed in one year to design and finalize a pediatric MS registry software. For this reason, all variables and registration methods were determined. The second phase which has been started since 2016 consists of a routine data registry in the national phase. The project information, including all cases, epidemiological, clinical, and diagnostic factors is being reported based on different geographical areas of Iran.

We registered 932 cases with MS since 2017, of whom 74.4% were girls. There was no significant difference in mean age between girls and boys with MS (mean difference=0.73 years, P=0.133). Most of the children who were diagnosed were between the ages of 15 to 18 years (52.4%).

The registry for MS in children in Iran can improve the knowledge of health policymakers on the incidence of MS in children throughout the country, the first and most common symptoms of MS in children, raising physicians’ awareness about early diagnosis and treatment that consequently reduce the economic, psychological, and psychological burden of the disease.

Neuroimmunological diseases in children encompass a range of disorders that lead to neurological complications in patients due to immune responses and systemic circulating antibodies. Limited research has been conducted on therapeutic plasma exchange’s efficacy and potential side effects in children with neuroimmunological diseases.

This study aimed to investigate this procedure’s effectiveness and potential side effects in children afflicted by these diseases.

This cross-sectional study examined a cohort of 18 children with neuroimmunological diseases who were admitted to the neurology department of Mofid Hospital over one year from March 2021 and underwent therapeutic plasma exchange.

The study included 18 patients, with an equal distribution of 9 females and 9 males. A total of 121 procedures were performed across 6 different disease groups: Multiple Sclerosis (22%, n = 4), Autoimmune Encephalitis (22%, n = 4), Neuromyelitis Optica Spectrum Disorder (22%, n = 4), Guillain-Barré syndrome (22%, n = 4), Acute Disseminated Encephalomyelitis (6%, n = 1), and Optic Neuritis (6%, n = 1). Following the plasma exchange, 17 patients (95%) showed immediate clinical improvement, while one patient diagnosed with optic neuritis did not respond to the treatment. During the follow-up period, 14 patients (78%) demonstrated significant improvement, one patient (6%) showed moderate improvement, and two patients (11%) exhibited mild improvement compared to their pre-plasmapheresis condition. Laboratory examinations revealed that only one patient experienced thrombocytopenia, which resolved without requiring treatment. No complications were observed during the follow-up visits for any of the patients.

Plasma exchange is a safe procedure for children with neuroimmunological diseases and yields favorable clinical responses.

 Guillain-Barre syndrome (GBS) is a post-infectious immune-mediated peripheral neuropathy, progressing bilaterally and often symmetrically and affecting sensory and motor function. Most cases completely recover, but around 20% of cases may lead to complications, incomplete recovery, or even death.

 This study aims to assess the prognosis of GBS in pediatric patients and possible associated conditions regarding recovery or prognosis.

 We investigated 71 cases of GBS admitted to Mofid Pediatric Hospital from March 2014 to March 2017. Demographic, clinical, and laboratory data were retrospectively recorded and analyzed. Two follow-up visits were performed after 1 to 3 and 5 to 8 years from onset, according to the GBS Disability Scale, and recovery of motor function was assessed during patients’ visits to the clinic.

 We found 35 male and 36 female subjects with an average age of 6.17 ± 3.82 (range 0.9 up to 15 years old); cases were mostly presented with myalgia and weakness (78.9%) followed by headache, found in 5 patients (7%). Around 84.5% of patients had an upper respiratory infection as their antecedent infection. Fifteen cases of autonomic dysfunction were observed, and 15 patients had cranial nerve involvement. Most cases had the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) form of GBS on electrophysiologic tests. Analysis showed only axonal involvement was significantly correlated with poor prognosis (P-value<0.05), and other variables were not significantly correlated.

 Compared to the current literature, we found fewer autonomic dysfunctions, cranial neuropathies, and a smaller percentage of AIDPs in our data. Altogether, the axonal form of GBS is reported as a predictor of an unfavorable prognosis in GBS patients.

Neuromuscular diseases (NMDs) affect muscle function directly or indirectly by affecting nerves or neuromuscular junctions. One of the leading causes of death in patients with NMD is respiratory muscle weakness (RMW). Respiratory involvement in patients with NMD can manifest widely, from mild failure that may initially affect only sleep to severe failure that can be life-threatening. Care approaches include arranged and precise clinical follow-ups of signs of sleep-disordered breathing, daytime hypoventilation, coughing, and swallowing disturbances. This manuscript will review the mechanisms and abnormalities of respiratory function in patients with NMD and help optimize NMD management.

To determine the clinical and MRI characteristics of multiple sclerosis (MS) in the children and adolescents.

In this cross-sectional study, information of 95 MS patients was obtained from the Iranian MS registry. Disease characteristics and imaging data were collected using medical records.

 Ninety-five patients including 64 female and 31 male subjects with mean age of 13.97±2.4 (range, 8-18) years were enrolled. The most frequent signs and symptoms were ophthalmic symptoms (n=61, 64.2%), brainstem signs (n=44, 46.3%), cerebellar signs (n=32, 33.6%) and pyramidal signs (n=26, 27.3%). Blurred vision (n=21, 34.4%) was the most common ophthalmic symptom and ataxia (n=24, 75%) the most prevalent cerebellar sign. The most common brainstem signs/symptoms were motor symptoms and vertigo (each n=14, 31.8%) and the most common pyramidal sign/symptom was right upper monoparesis (n=14, 23.3%). Active demyelinating lesions were reported in brain MRI of all patients, mostly appeared as periventricular (n=91, 95.8%) and pericallosal (n=55, 57.9%) lesions. Acute demyelinating spinal lesions were presented in 38 patients (51.3%) with a prominent involvement of the cervical spine (n=33, 86.8%).

In our study, the most frequent signs and symptoms were eye symptoms, brainstem signs, cerebellar signs and pyramidal signs, respectively. Moreover, our results showed that MRI plays a critical role in the diagnostic evaluation of MS in children with presence of brain lesions in all patients and spinal lesion in a considerable portion of patients.

Biotinidase (BTD) deficiency may lead to variable neurologic manifestations. Spinal cord involvement can be an unusual presentation of the late-onset disorder.

We describe a six-year-and-ten-month-old girl with a previous history of recurrent upper respiratory tract infections, frequent falling four years before admission, gradual vision loss, and subacute progressive limb weakness followed by flaccid quadriplegia during admission. The measurements of cerebrospinal fluid (CSF) lactate showed significant elevation. And spinal MRI revealed longitudinally extensive cord involvement, mimicking acquired demyelinating syndrome; however, there was no response to plasma exchange. Profound biotinidase deficiency was confirmed by enzyme assay; the patient revealed dramatic recovery after the biotin prescription. The genetic analysis showed a homozygous missense variant BTD (NM_001370658.1): c.838A>C (p.Asn280His) in exon 4, thereby predicting pathogenic based on the ACMG guidelines. To the best of our knowledge, this is the first Iranian report on BTD deficiency.

In summary, in each patient with longitudinally extensive cord involvement not well responding to the conventional treatment, BTD deficiency should be considered on differential.

To determine the effectiveness of Rituximab (RTX) therapy as the first therapeutic choice for the long-term prevention of secondary relapse in children with Autoimmue Neurological Disease (AIND) had relapse after primary treatment with immunosuppressive agents other than RTX.

We conducted a single-center retrospective study of 9 consecutive pediatric patients (≤ 18 years old) registered on Autoimmune and Demyelinating Disorders Database (ADDD) of Mofid Children Hospital, from 2012 to 2016 and experienced relapse following therapeutic interventions with immunosuppressive agents other than RTX.

A remarkable reduction of 94.13% (p=0.015) occurred in annualized relapse rate (ARR) as a clinical indicator of therapeutic efficacy comparing before and after initiating RTX therapy.

Rituximab is an effective drug in relapse prevention of AIND whenadministrated to patients for whom initial treatment with other immunosuppressive agents fail. POWER OF EVIDENCE: This study represents Class IV evidence that RTX therapy significantly reduces ARR in pediatric AIND including DDCNS.

Imbalance in serum electrolytes seems to play an important role in the development of febrile seizures. This study aimed to evaluate the serum levels of glucose, sodium, calcium, and magnesium in children with febrile seizures admitted to hospitals affiliated to Shahid Beheshti University of Medical Sciences from March 2008 to March 2019.

In this retrospective study, the medical records of all patients who met the inclusion criteria during the study period were reviewed. Demographic and clinical data of patients, as well as serum levels of sodium, potassium, calcium, magnesium, and glucose were extracted and evaluated.

A total of 300 children were enrolled according to the research criteria. 264 patients (88%) had simple seizures and 36 patients (12%) had complex seizures. The mean serum levels of sodium, calcium, potassium, glucose, and magnesium in the patients were in the normal range. On the other hand, there was no statistically significant difference between serum levels in the two groups of simple and complex seizures. Based on the regression analysis, the history of febrile seizures could predict the occurrence of complex seizures (OR=4.5, P=0.00, 95%CI: 2.06-10.11)

Serum levels of sodium, calcium, potassium, glucose, and magnesium did not affect the incidence of seizures in the patients. On the other hand, due to the high risk of complex seizures in patients with a history of febrile seizures, the necessary measures can be taken to manage the disease.

 Due to a lack of data on pediatric multiple sclerosis (MS) epidemiology in Iran, this study aimed to determine the incidence rate of pediatric MS in Iran.

 All the data of the patients with MS registered in the Ministry of Health and Medical Education of Iran for 20 years were collected in this study; therefor; those born in 1982 and diagnosed with the disease and treated since 2000 were included in this study. The collected variables were patients’ age at the time of diagnosis, gender, year of diagnosis, urban or rural residency, and province of residence. Additionally, age- specific incidence rates per 100,000 of the population were calculated.

This study was performed on 4544 cases of pediatric MS within 2000- 2019, of which 997 patients (21.9%) were male. The mean age of the patients with MS at the time of diagnosis was 14.3±4.6 years, and 4414 children (97.1%) lived in urban areas. The incidence rate of pediatric MS in Iran during 20 years increased from 0.26 per 100,000 of the population in 2000 to 1.53 in 2019.

 The incidence of pediatric MS in Iran is high, and the development of diagnostic practices in the past decade in Iran has contributed to the detection of this high incidence

Identifying the predictors of seizure recurrence is of great help in controlling the disease and preventing recurrence in patients. This study aimed to determine the risk factors for recurrence of seizures in children younger than 14 years of age.

In this cross-sectional analysis, we reviewed the medical records of patients admitted to pediatric neurology clinic in Tehran Imam Hussein Hospital between June 2016 and November 2017. Information including patients’ demographic and clinical data, and seizure recurrence and times were extracted. Data were analyzed in SPSS V23.

The participants were 210 patients (mean age: 62.40±46.79 months), including 53% males. Recurrence of seizures was observed in 81 patients. In 45 patients with recurrent seizures and 101 patients without recurrent seizure, the type of seizure was tonic-clonic. Abnormal developments were seen in 45.7% of patients with recurrent seizures and 15.5% of patients without recurrent seizures. Gestational age and abnormality in first electroencephalogram (EEG) were identified as predictors of seizure recurrence.

Preventive treatments are highly recommended in patients with low gestational age and disordered EEG to prevent subsequent seizures and their complications

Movement disorders are common neurologic disturbances in childhood. There are two major types movement disorders. Hypokinetic disorders are with paucity of voluntary movements and are very uncommon in pediatric age group. Hyperkinetic movement abnormalities are very common in children and defined as abnormal repetitive involuntary movements. Movement disorders in childhood and even in adolescents are different in etiology, timing, treatment and prognosis versus adulthood movement abnormalities. In this brief article, we reviewed common types of hyperkinetic abnormal movements in children and adolescents with emphasis on etiologies, new classifications and recent treatment strategies.

Neurometabolic disorder is one of the important groups of diseases that prominently has presentation early infantile period. In this study, we evaluated the result of metabolic screening of the patient with seizure, developmental delay and/or regression in development, demographic disease clinical and radiological findings on admitted and outpatient visited children.
Two-year retrospective review of 187 children with seizure, developmental delay and/or regression in the Mofid Children Hospital, Tehran, Iran was performed. The diagnosis was based on observation, findings of EEG and history of the patient, besides evaluation of patient milestones. The result of metabolic screening with Tandem mass spectrometry was evaluated using SPSS (ver.18.0) Statistical software.
Totally, 187 children with seizure, regression and/or developmental delay were evaluated by metabolic screening with tandem mass spectrometry method. The results of laboratory examination had no relationship between positive results of metabolic screening and the mentioned disease. The relations between positive results of metabolic screening and seizure, regression and/or developmental delay were not statistically meaningful.
Positive results of metabolic screening and seizure, regression and/or developmental delay were not statistically meaningful.

Approximately one-third of all children with epilepsy do not achieve complete seizure improvement. This study evaluated the efficacy of Vigabatrin in children with intractable epilepsy. From November 2011 to October 2012, 73 children with refractory epilepsy (failure of seizure control with the use of two or more anticonvulsant drugs) who were referred to the Children’s Medical Center and Mofid Children’s Hospital were included in the study. The patients were treated with Vigabatrin in addition to their previous medication, and followed-up after three to four weeks to determine the daily frequency, severity, and duration of seizures in addition to any reported side effects. Of the 67 children, 41 (61.2%) were males and 26 (38.8%) females, their age ranging from three months to 13 years with an average of 3.1 [standard deviation (SD), 2.6] years. The mean daily frequency of seizures at baseline was 6.61 (SD, 5.9) seizures per day. Vigabatrin reduced the seizure frequency ≤2.9 (SD, 5.2) (56% decline) and 3.0 (SD, 5.3) (54.5% decline) per day after three and six months of treatment, respectively. A significant difference was observed between seizure frequencies at three (P<0.001) and six months (P<0.001) after Vigabatrin initiation compared with the baseline. Somnolence [3 (4.5%)], horse laugh [1 (1.5%)], urinary stones [1 (1.5%)], increased appetite [1 (1.5%)], and abnormal electroretinographic pattern [3 (4.5%)] were the most common side effects in our patients. This study confirms the short-term efficacy and safety of Vigabatrin in children with refractory epilepsies.

Dravet syndrome or severe myoclonic epilepsy of infancy (SMEI) is a baleful epileptic encephalopathy that begins in the first year of life. This syndrome specified by febrile seizures followed by intractable epilepsy, disturbed psychomotor development, and ataxia. Clinical similarities between Dravet syndrome and generalized epilepsy with febrile seizure plus (GEFS+) includes occurrence of febrile seizures and joint molecular genetic etiology. Shared features of these two diseases support the idea that these two disorders represent a severity spectrum of the same illness. Nowadays, more than 60 heterozygous pattern SCN1A mutations, which many are de novo mutations, have been detected in Dravet syndrome. From May 2008 to August 2012, 35 patients who referred to Pediatric Neurology Clinic of Mofid Children Hospital in Tehran were enrolled in this study. Entrance criterion of this study was having equal or more than four criteria for Dravet syndrome. We compared clinical features and genetic findings of the patients diagnosed as Dravet syndrome or GEFS+. 35 patients (15 girls and 20 boys) underwent genetic testing. Mean age of them was 7.7 years (a range of 13 months to 15 years). Three criteria that were best evident in SCN1A mutation positive patients are as follows: Normal development before the onset of seizures, onset of seizure before age of one year, and psychomotor retardation after onset of seizures.Our genetic testing showed that 1 of 3 (33.3%) patients with clinical Dravet syndrome and 3 of 20 (15%) patients that diagnosed as GEFS+, had SCN1A mutation. In this study, normal development before seizure onset, seizures beginning before age of one year and psychomotor retardation after age of two years are the most significant criteria in SCN1A mutation positive patients.

Approximately one third of epileptic children do not achieve complete seizure improvement. Zonisamide is a new antiepileptic drug which is effective as adjunctive therapy in treatment of intractable partial seizures.The purpose of the current study was to evaluate the effectiveness, safety, and tolerability of Zonisamide in epileptic children. From November 2011 until October 2012, 68 children who referred to Children’s Medical Center and Mofid Children Hospital due to refractory epilepsy (failure of seizure control with the use of two or more anticonvulsant drugs) entered the study. The patients were treated with Zonisamide by dose of 2- 12 mg/kg daily in addition to the previous medication. We followed the children every three to four-weeks intervals based on daily frequency, severity and duration of seizures. During the follow-up equal and more than fifty percent reduction in seizurefrequency or severity known as response to the drug. In this study 68 patients were examined that 61 children reached the last stage.35 (57.4%) were male and 26 (42.6%) patients were female.After first and six months of Zonisamide administration daily seizure frequency decreased to 2.95±3.54 and 3.73±3.5 respectively. There was significant difference between seizure frequency in first and six month after Zonisamide toward initial attacks. After six months ZNS therapy a little side effects were created in 10 patients (16.4%) including stuttering(4.9%), decreased appetite (4.9%), hallucination (1.6%), dizziness(1.6%), blurred vision(1.6%) and suspiring(1.6%) as all of them eliminated later dosage reduction. This study confirms the short term efficacy and safety of Zonisamide in children with refractory epilepsies.

Multiple sclerosis (MS) is an autoimmune multifactorial degenerative disease with detrimental affliction on central nervous system. MHC class I chain- related geneA,B(MICA and MICB) are nonclassical human leukocyte antigens that can affect on some diseases and also on transplantation. The purpose of this study was to evaluate the MICA and MICB MRNA expression in multiple sclerosis patients. In this study, we evaluated MICA and MICB MRNA expression in the peripheral blood mononuclear cells by reverse transcryptase-polymerase chain reaction(RT-PCR) in MS patients and normal controls. The results of this study showed that 32.6% of patients with progressive clinical outcome over expressed MICB genes in comparison with controls (p=0.002). It is concluded that the high expression of MICB gene in MS patients is an important criterion of MS disease that it may be due to the interaction between MICB and its receptor on CD8+T or NK cells.

Chronic Hepatitis B virus (HBV) infection is a major liver disease worldwide and its clinical manifestations are linked to immune response. The purpose of this study was to evaluate the relationship between selenium, copper, and zinc in comparison with transaminase level in chronic HBV patients. Serum samples of the HBV infected patients were obtained from Tooba medical center, Sari, Iran. Sixty patients were enrolled in this study (36 men and 24 women), mean age: 39.6 ± 12.2 years. The concentration of zinc, selenium, copper and transaminases were determined using an autoanalyzer system. Concentrations of selenium (0.273 ±0.056 μg/dl) and zinc (2.1±0.037) was elevated in patients with low transaminase levels as were significantly different in comparison with patients with high transaminase level (P<0.05). Serum copper concentration was similar in two groups of patients. Elevated levels of transaminase concentrations were independently associated with low zinc and selenium concentrations in chronic HBV patients. It is concluded that serum zinc and selenium levels are associated with less hepatic damage in chronic HBV patients and might have a protective role during liver injury.