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 seizure frequency 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.
Background: Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system. Case presentation: We describe a 12-year-old boy with ...progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain--Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later. Conclusion: SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment. Keywords: Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, systemic lupus erythematosus, nerve disorders
Pulmonary Involvement in Neuromuscular Diseases Khanbabaee, Ghamartaj; Zadeh, Parvaneh Karim; Nasehi, Mohammad Mahdi ...
Iranian journal of child neurology,
04/2023, Letnik:
17, Številka:
2
Journal Article
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.
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.
Pulmonary Involvement in Neuromuscular Diseases: A Review Khanbabaee, Ghamartaj; Karim Zadeh, Parvaneh; Nasehi, Mohammad Mahdi ...
Iranian journal of child neurology,
2023-Spring, 20230101, Letnik:
17, Številka:
2
Journal Article
Odprti dostop
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.
Overall, 2%-5% of patients with multiple sclerosis (MS) experienced the first episode of disease before the age 18 years old. Since the age of onset among children is not similar to that in general ...population, clinicians often fail to early diagnose the disease. This study aimed to determine the epidemiological and clinical patterns of MS among Iranian children.
In this cross-sectional study carried out in Iran in 2014-2015, information was collected using a checklist with approved reliability and validity. Method sampling was consensus. Data were analyzed using frequency, mean and standard deviation indices by means of SPSS ver. 20 software.
Totally, 177 MS children were investigated. 75.7% of them were female. Mean (SD), minimum and maximum age of subjects were 15.9 (2), 7 and 18 yr, respectively. The most reported symptoms were sensory (28.2%), motor (29.4%), diplopia (20.3%) and visual (32.8%). Primary MRI results showed 91.5% and 53.1% periventricular and spinal cord lesions, respectively.
MS is significantly more common among women. The most common age of onset is during the second decades. Sensory and motor problems are the most symptoms, while, periventricular and spinal cord lesions are the most MRI results.
This study was conducted to predict the response to treatment in patients treated with anti-epilepsy drugs.
This analytical questionnaire-based study was conducted in 2014 among 128 patients with ...epilepsy admitted to Mofid Children's Hospital, Tehran, Iran. The inclusion criteria were children 2 months to 12 yr of age with epilepsy and patients who experienced fever and seizure attacks at least once were excluded from the study. Patients were followed up for 6 months and the response to their treatment was recorded. The good response to treatment was defined as the absence of seizure with two drugs during follow up.
Seventy-two patients (56.3%) were boys. The age of the first seizure was under 2 yr old in 90 patients (70.3%). History of febrile convulsion, family history of epilepsy and history of asphyxia was found in 16 (12.5%), 41 (32%), and 27 (21.1%) patients, respectively. Seizure etiology was idiopathic in 90 patients (70.3%), and the number of seizures was 1-2 in 36 patients (28.1%). Overall, 57 patients (44.5%) had cerebral lesion according to CT scan or MRI, and EEG was abnormal in 101 patients (78.9%). In 6-month follow-up, 40 patients (31.3%) responded well to the treatment and 88 patients (68.8%) responded poorly to the treatment. History of asphyxia (OR = 6.82), neonatal jaundice (OR = 2.81) and abnormal EEG (OR = 0.19) were effective factors in response to treatment.
Abnormal EEG is an effective factor in treatment response in the children studied.
