Background The occult Neurological disorders are an important cause of constipation in children. This study aimed to evaluate the spinal canal and lumbosacral by ultrasound in pediatric constipation to better identifying Neurological causes of constipation. Materials and Methods In this case-control study, 100 children with constipation (age range 1 to 14 years) without previously known chronic illness referred to the Radiology Department of the Mashhad Pediatric Dr. Sheikh Hospital were selected. After recording clinical data, the patients were undergone sacral and spinal cord ultrasound examinations and the results were compared with the control group (healthy children with transient illness (otherwise constipation or urinary disorders) who had referred to radiology department for sonographic examination). Results The mean age of patients was 6 ± 3. 3 years old. The tethered cord and occult intrasacral meningocele were observed in 2% of patients group. Spina bifida was found in 64% patients, and 31% control subjects with significant difference (P = 0. 009). About one third of cases with spina bifida were found in lumbar L5 vertebra, and another two third were in high sacral vertebrae (S1 or S2). Various degrees of caudal regression were observed in 8% patients suffering from constipation and in control group, 2% children had coccyx hypoplasia. No significant correlation was found between case and control groups regarding the location of spina bifida and non-formation of the posterior arch of the sacrum, the mean coccyx length, dural diameter and the ossification age of first vertebra of coccyx. Conclusion In pediatric age, lumbosacral spinal anomalies can easily evaluate with ultrasound. The prevalence of spina bifida and caudal regression in children with constipation was significantly higher compared with normal control group.

Inborn errors of organic acid metabolism are relatively recently recognized diseases with a wide spectrum of clinical signs and symptoms: ranging from asymptomatic, normal appearing children to death during first few days of life. In my presentation I will try to explain some of the most common clinical presentation of these disorder with stress on Neurologic findings. Organic acidemia usually have three clinical manifestations Severe neonatal form, Intermittent late-onset form and chronic progressive form. Recurrent coma, The main feature of these disorders is due to accumulation of toxic metabolites in Central Nervous system with direct effect on the function, while chronic accumulation of these materials may interfere with CNS development or cerebral metabolism leading to developmental delay. Severe neonatal forms Following a symptom free interval of a few days from birth, poor sucking and difficult feeding appears in the newborn, followed by unexplained and progressive coma. Seizures may appear during the course of the disease and EEG may show a burst-suppression pattern. During this stage most infants have axial hypotonia with peripheral dystonia, choreoathetosis, episodic opisthotonus and some repetitive bicycling and boxing movements. Associated biochemical abnormalities including metabolic acidosis, ketonuria and hyperammonemia also is usually present. The overall short-term prognosis with recent advances in medical care is improving. But later in life acute intercurrent episodes triggered by a stress often occur, which can be occasionally fatal. bulging fontanelle and cerebral edema may mimic CNS infection in these babies. Intermittent late-onset forms Recurrent attacks of coma or lethargy with ataxia can occur in childhood or even in adolescence or adulthood. These episodes may be frequent, though in between these the child is entirely normal. These attacks are precipitated by conditions that enhance protein catabolism (trauma, infection etc). Sometimes these episodes can lead to death or severe sequel. Seizure disorder is one of these sequels which is generalized in type with myoclonic seizure in infancy and childhood and later tonic-clonic and atypical absence seizures predominate.Also many of the survivors have acute or progressive extra pyramidal syndrome due to bilateral necrosis of basal ganglia. Chronic progressive forms Non specific Developmental delay, hypotonia, muscular weakness, microcephaly and seizures are rarely the only revealing signs in organic acidemia without any acute presentation. Seizures may become refractory to Anti Epileptic Drugs. In addition many asymptomatic or minimally symptomatic infants have been identified during tandem mass spectrometry newborn screening program. Cognitive deterioration associated with movement disorder such as dystonia or chorea may be caused by any form of organic aciduria.

Background: Behcet disease is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its Neurologic manifestations (neuro-Behcet disease) are relatively rare, but they must be thoroughly investigated due to their grave prognosis.Review Summary: The frequency of Neurologic manifestations, more common in tnale Behcet patients, is between 5% and 30%. Both the central and peripheral nervous systems can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/ aneurysms. Peripheral neuropathy and myopathy are relatively rare. Cerebrospinal fluid analysis reveals pleocytosis and elevated protein levels. Magnetic resonance imaging is the investigation of choice which often reveals iso-/hypointense lesions in T1-weighted images and hyper intense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts the brainstem. Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal manifestations, and corticosteroids and anticoagulants are used for treatment of dural sinus thrombosis.Conclusion: Neuro-Behcet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other Neurologic syndromes.

Background: Canine distemper (CD) is the dogs’ most contagious and lethal viral disease. Despite the widespread use of vaccines to control CD, the prevalence of the CD virus (CDV) has increased at an alarming rate in recent years. Objectives: To identify the genotypes responsible for the Neurological and non-Neurological clinical forms of CD and to investigate the presence of the virus in the Neurological and non-Neurological forms of the disease. Methods: In this descriptive-analytical study, the samples were collected from 70 CD suspected unvaccinated dogs with clinical signs of distemper. All cases were tested with rapid tests and separated into 3 groups based on clinical signs and symptoms. Cerebral spinal fluid (CSF), respiratory secretion, and fecal samples of allall 70 cases were examined for reverse transcription polymerase chain reaction (RT-PCR). After sequencing the hemagglutinin gene (H gene), phylogenetic analysis of the gene isolated from CDVs was carried out using MEGA software, version 7. Results: The RT-PCR results showed that the respiratory secretion sample in the non-Neurological CDV group (85%) and the Neurological CDV group (80%) had the highest level of virus contamination. However, in the non-Neurological CDV group, the CSF sample (40%) had a high level of infection. In neurotic groups, cases older than 12 months showed the highest percentage of distemper contamination, and in the non-Neurologic CDV group, those between 3 and 6 months were more involved. Sequencing and phylogenetic analysis of the H gene revealed the CDV as a member of the endemic Arctic-like genetic lineage. Conclusion: The genotypic examination of the hemagglutinin gene of the distemper virus reveals that the recent isolates of Neurologic and non-Neurologic clinical forms of CDV in Iran are similar. In positive rapid test cases, the PCR test of respiratory secretions for virus detection ranks with the highest sensitivity. In Neurologic cases with negative rapid test results, PCR of CSF had the highest sensitivity, so it may serve as a diagnostic criterion.

Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of Neurological manifestations in children with rheumatologic disorders is limited. This review describes the Neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally.