Iranian Journal of Child Neurology

Photo-albums and video-clips are simple means for diagnosis of diverseneurologic disorders in children. Most families either own or can borrow a still or video camera. Even when a purchase is required, it is more costeffective than brain imaging as well as other sophisticated studies, and the family has something useful to show for expenditure. On the other hand many families have a photo-album which could be very informative for pediatric neurologists. These useful and simple means are invaluable in:

• Differentiation of progressive from static diseases of central nervous system in children.
• Helping in diagnosis of diverse types of seizures in pediatric epileptic patients.
• Differential diagnosis of epilepsy like disorders (e.g. sleep disorders) vs. epilepsy in children.
• Diagnosis as well as differential diagnosis of movement disorders in children.
• Therapeutic follow-up in many disorders (i.e. epilepsy, movement and sleep disorders) in children.

In my review article, I have indicated the importance of photo-albums and video-clips as invaluable means of diagnosis and  prediction in child neurology by giving simple examples in this regard.

Objective:

Mumps infection is endemic in Iran and mumps parotiditis is a common disease in Iranian children. There has been a dramatic decrease in the worldwide incidence of mumps since the introduction and use in 1968 of the very effective and inexpensive mumps vaccine.

In Iran probably due to a higher percentage of unvaccinated young persons <15yr, the incidence rate of mumps infection and its sequela are higher in comparison to corresponding data from developed countries prior to comprehensive vaccination programs. The aim of the study was to investigate the effects of the mumps virus on cochlear function and to determine the frequency of related Sensory Neural Hearing Loss (SNHL) in children.

Material& Methods:

This descriptive case-series study was conducted in 94children, aged less than 14 years,  hospitalized between 1999 and 2001, in the pediatric ward of the Hazrat Rasool Hospital in Tehran. All patients with documented mumps infection (specific mumps-IgM antibody) were evaluated twice for audiometeric function on the basis of diagnostic parameters for sensory neural hearing loss; the first evaluation was done on admission and the second three weeks later. 54 patients (age range 1-14y, mean age 4.83±3.93, male: female ratio30:24) were studied in two years.

Results:

The highest incidence of mumps was seen in winter (37%) and spring (28%) and the lowest in summer (13%). Specific IgM antibody for mumps virus was detected in 74 children. Comprehensive audiologic evaluation was done in 54 patients at admission and again 3 weeks later. SNHL was detected in 7.2% of patients; the 4.4% incidence of SNHL in this study was higher than in other studies in developed countries prior to comprehensive vaccination programs.

Conclusion:

Implementation of comprehensive vaccination programs in young Iranians could dramatically reduce the burden and costs imposed by the infection and its sequelae.

Keywords: Mumps infection ;SNLH(sensorineural hearing loss);Mumps vaccination

Objective:

Breath holding spells, very often misinterpreted as epileptic seizures, are most common in children aged 6 months to 6 years of age. In this investigation, we sought to prospectively document the natural history of breath holding spells (BHS) among children with cyanotic, pallid and mixed type BHS referred for neurological consultation.

Materials & Methods:

This was a cross-sectional study in which a total of 43 children (23 boys, 20 girls) with BHS, admitted to the out patient clinic of the Children's Hospital Medical Center, between Sept 1998 and June 1999, were enrolled. A structured interview was under taken at the time of initial consultation to confirm BHS and its type, associated phenomenon, family history, sex and age at initiation of spells. Laboratory, electroencephalographic and electrocardiographic tests were done.

Results:

Patients were between 1.7 and 42.8 months (mean age 18.4 months). In 76.8% of cases, BHS began during the first 12 months of age. Anger and pain were the most common triggering factors (65.1 %). A positive family history of BHS was identified in 51% and parental consanguinity was found in 30% of cases. The spells were cyanotic in 79.1% (34 children). 78% of cases were iron deficient and 53% of cases had iron deficiency anemia.

Conclusion:

The results of this study emphasize the role of genetic factors in BHS; measurement of hemoglobin and serum ferritin is recommended all such cases.

Key words: Breath-holding spells, Iron deficiency, Anemia, Serum ferritin

Objective :

Behavioral disorders of children and adolescents have long been a subject of discussion among researchers of pedology,   psychology, medicine and psychiatry and are also a commonly encountered complaint of patients referring to the pediatric neurology out patient clinics. The main purpose of the present study is to survey some organic disorders, e.g. temporal lobe epilepsy (non convulsive seizures) and to investigate the role of some trace element deficiencies, in particular iron deficiency on the development of behavioral disorders seen in children.

Materials & Methods:

In this study all patients referring to the Imam Khomeini pediatric neurology out patient clinic with the chief complaint of behavioral problems, between October 1996 and January 1998 were  enrolled; they were individually interviewed and underwent physical examinations, completing the relevant questionnaires.

Results:

Overall 139 patients (92boys and 47 girls) were enrolled and the data were analyzed using SPSS and Harvard's graphic package. Analysis revealed that the most common behavioral disorders documented were: Sleep problems, in 38 patients (27.3%), Irritability in 37(26.6%), aggressiveness in 28 (20.1%) staring in 27 (19.4%), altercation in 27(19.4%). Non psychiatric problem was seen in 77 patients (62.6%); neurological examinations in 36(25.9%) revealed abnormal findings; 103 patients (74-8%) had abnormal findings in other systems. 105 patients (76.6%) had abnormal EEG and 72 of them (51.9%) had abnormal findings in brain imaging. Iron deficiency was found in 88 (63.2%) of the patients.

Conclusion:

According to these findings, treatment of organic disorders in patients with behavioral problems, can lead to partial or total control of their problems, which could otherwise result in major disturbances and disruptions in their own and in their family's lives.

Key words:

behavioral problems, EEG, Imaging, Iron deficiency

Objective:

Infantile spasms (IS) is an age specific epileptic syndrome. Due to poor response of IS to conventional antiepileptic drugs, scientists are always on the lookout for newer, more effective drugs to treat the condition.

Materials & Methods:

In this study, 50 infants with IS symptoms, aged between 2-24 months, were randomly divided into two equal groups, each treated either with Vigabatrin or ACTH (long acting   Tetracosectide); the results for clinical efficacy of medication and drug side effects were compared.

Results:

After the sixth week of treatment, in the Vigabatrin group, 28% complete remission and 40% reduction in seizure frequencies was seen, while in the Tetracosectide group there were 40.9%  complete remission and 45.5% reduction in seizure frequencies, showing no significant difference between the clinical responses found in the two groups (P=0.44).

Conclusion:

Vigabatrin effeciacy is similar to Tetracosectide, but since it is impossible to evaluate visual field constriction, a probable irreversible side effect of Vigabatrin , It's better to use Tetracosectide for the first line in the treatment of IS .

Keywords:

Infantile spasms,Hypsarrhythmia, Vigabatrin,Tetracosectide

Objective:

The term "Ataxia" refers to disturbances of body posture and movement that are normally controlled by the cerebellum, frontal lobes and the posterior columns of the spinal cord. The primary symptom and the most prominent feature of ataxia is abnormal gait which is characterized by lurching and wide base walking. 

Ataxia was considered acute, if it had occurred within the two preceding weeks. Knowing how frightening acute-onset Ataxia is for the family is not surprising that the condition prompts an immediate visit to the physician.

Material & Methods:

In view of the lack of information in our country, on the etiology of sudden-onset Ataxia, the authors enrolled 100 children with the chief complaint of acute loss of equilibrium, who came to the attention of the Pediatric Neurology Department over a two year duration (Sept.2001-Sept 2003); they were admitted to the Mofid Childrens' Hospital and all necessary investigations were carried out.

Results & Conclusion:

The results revealed that Acute Cerebellar Ataxia was the most common cause of the problem, the second most frequent being drug intoxication, which most commonly occurred in patients, 2- 4years old. The remaining causative factors in order of descending frequency consisted of infectious polyneuropathy, migraine, opsoclonus-myoclonus, brain tumor, acute disseminated encephalomyelitis, multiple sclerosis, and epilepsy.

Key words:

Objective:

Pseudotumor Cerbri (PTC) is a clinical syndrome characterized by increased Intra-Cranial Pressure (ICP) without any evidence of a mass lesion or any obstructive process. The incidence is 0.9-2 per 100.000 people. It is more frequent in adults in the 20-30 years age group, especially obese women, and less common in pediatric age groups; 11-16 years old children may however suffer from PTC, with no difference in the rates of occurrence in either sex.

Material & Methods:

In this descriptive retrospective study we reviewed the files of 32 children with diagnosis of PTC admitted during the past 15 years in the neurology ward of the Mofid Children's Hospital (25) and Ali Asghar Children Hospitals (7) between the years 1988 and 2003.

Results:

The results of this study revealed that children in the 5-10 years age group, girls in particular, are more vulnerable.

Conclusion:

The most frequent complaints that brought these patients to physician included headache, vomiting and strabismus. Common findings of neurological examination were papilledema, abducent nerve palsy and ataxic gait. While all cases recovered with medical treatment, one did need surgical intervention. Abbreviations; Pseudotumor Cerbri = PTC; Intra Cranial pressure = ICP.

Keywords :

Objective:

Torticollis is a symptom that can be related to different pathological mechanisms ranging from simple to life-threatening conditions. Here we report a child with torticollis caused by a neuroenteric cyst in the upper cervical region; this is a very rare condition in childhood and  in this case, it was successfully resolved by surgery.

Clinical presentation:

A 2.5 year old boy presented with a 2 month-history of torticollis, he had developed paraparesia 2 weeks before admission. At examination he was found to be quadriparetic. Radiographic study of  the cervical spine revealed widening of the cervical canal. Brain and spinal magnetic resonance imaging revealed a hypointense lesion on the T1 at the craniovertebral junction having a  compressive effect on the anterior aspect of the brain stem and spinal cord.

Intervention:

The patient underwent surgery. After craniotomy and opening of the dura, a cystic lesion was seen; clear fluid was aspirated and the cyst wall was removed.

Conclusion:

Considering the quadriparesis and torticollis, the patient improved significantly within the first few days after surgery. No relapse of symptoms occurred during the follow up period. This is the first case report of a child in whom torticollis was due to a neuroenteric cyst of the upper cervical intradural region.

Keywords: