Semra Saygi

Hashimoto's encephalopathy in children and adolescents.

Hashimotos encephalopathy is an underdiagnosed, steroid-responsive, progressive or relapsing encephalopathy associated with high titers of serum antithyroid antibodies. Although Hashimotos encephalopathy is well documented in adults, it is rarely observed or studied in children and adolescents. We describe the clinical and laboratory findings of four children (aged 9-15 years) with Hashimotos encephalopathy. The clinical features of two patients at presentation included epileptic seizures and confusion. The other presenting signs included breath-holding spells, behavioral problems, psychosis, and ataxia (one patient each). During their presentation, three patients were euthyroid, and one was hyperthyroid. All patients manifested increased antithyroid antibodies, and all improved with steroid treatment. Hashimotos encephalopathy is rarely suspected at presentation. Therefore, greater awareness of its signs by clinicians is necessary for proper diagnoses.

Juvenile Dermatomyositis Presenting With Anasarca

Generalized edema can occur during the course of juvenile dermatomyositis. In this article, a 4-year-old boy with generalized nonpitting edema and proximal weakness is reported. Characteristic cutaneous lesions, laboratory tests, results of electromyography, and magnetic resonance imaging findings resulted in a diagnosis of juvenile dermatomyositis. He was treated with corticosteroids and methotrexate. It is concluded that the generalized edema may appear as the presenting cutaneous manifestation of juvenile dermatomyositis.

Overview of Pediatric Peripheral Facial Nerve Paralysis: Analysis of 40 Patients

Peripheral facial nerve paralysis in children might be an alarming sign of serious disease such as malignancy, systemic disease, congenital anomalies, trauma, infection, middle ear surgery, and hypertension. The cases of 40 consecutive children and adolescents who were diagnosed with peripheral facial nerve paralysis at Baskent University Adana Hospital Pediatrics and Pediatric Neurology Unit between January 2010 and January 2013 were retrospectively evaluated. We determined that the most common cause was Bell palsy, followed by infection, tumor lesion, and suspected chemotherapy toxicity. We noted that younger patients had generally poorer outcome than older patients regardless of disease etiology. Peripheral facial nerve paralysis has been reported in many countries in America and Europe; however, knowledge about its clinical features, microbiology, neuroimaging, and treatment in Turkey is incomplete. The present study demonstrated that Bell palsy and infection were the most common etiologies of peripheral facial nerve paralysis.

Clinical characterization of gastroenteritis-related seizures in children: impact of fever and serum sodium levels.

Gastroenteritis-related seizures have increasingly gained attention in recent years. Most cases follow a brief, benign course with very few episodes of seizure recurrence and without development of epilepsy. Published reports usually do not make a distinction between febrile and afebrile patients, and most authors include only nonfebrile convulsions in their reported series. This study evaluated the impact of fever in children presenting with seizures during a mild gastroenteritis episode and found that the presence or absence of fever did not affect seizure characteristics or duration. However, mild hyponatremia affected some seizure features, particularly seizure duration, as hyponatremic children sustained more prolonged seizures than patients with normal serum sodium levels, irrespective of body temperature.

Acute transverse myelitis in a child with Lyme disease and a review of literature.

Acute transverse myelitis is a rare Borellia burgdorferi-related neurologic complication in childhood. We present a 12-year-old girl who was diagnosed with acute transverse myelitis associated with a borreliosis infection. We also review clinical features in all five cases of Borellia burgdorferi-related transverse myelitis in children. We describe here the sixth child with borreliosis-related transverse myelitis.

Stroke in Early Childhood Due to Homocystinuria

A previously healthy girl, age 3 years 9 months, presented with right-sided hemiparesis and seizures. Ischemic infarction was confirmed through magnetic resonance imaging and magnetic resonance angiography. Extensive evaluation to discover the underlying etiologies and risk factors predisposing this patient to stroke included coagulation defects, cardiac anomalies, congenital inborn metabolism deficiency, and infections and trauma. Based on the clinical and laboratory results, a diagnosis of homocystinuria was made. Homocystinuria is an inherited disorder that affects the metabolism of the amino acid methionine. Although homocystinuria is usually associated with ischemic strokes, the sudden onset of stroke as the initial clinical presentation of homocystinuria is very rare in early childhood. Based on this case, however, metabolic screening for hyperhomocystinemia is recommended in any child presenting with a stroke.

Prolonged neonatal complications after in utero exposure to fluoxetine.

This article presents the case of a female newborn with irritability, increased tonus, jitteriness, and eating difficulties who was referred to our institution. Her mother had been taking fluoxetine (20 mg daily) during the second and third trimesters of her pregnancy. The infants signs began on the first day after birth and persisted for 6 weeks. Extensive investigations excluded infective, genetic, and metabolic causes, and a provisional diagnosis of fluoxetine withdrawal was made. There have been few reports of neonatal complications following maternal fluoxetine hydrochloride treatment. According to these, signs occurred within a few days after birth and lasted up to 4 weeks. To our knowledge, our patient demonstrated the longest duration of signs reported to date. We recommend that physicians be aware of these complications in newborns after fetal exposure in utero to selective serotonin reuptake inhibitors. These neonates should be followed-up closely for adverse signs during the first days of life so that signs can be recognized and treated if necessary.

Superoxide Dismutase and Catalase Genotypes in Pediatric Migraine Patients

This study compared superoxide dismutase (SOD) and catalase (CAT) alleles in 97 consecutive children and adolescents with migraine to 96 healthy children and adolescents. Isolated genomic DNA was used as a template for SOD1 (35 A/C), SOD2 16 C/T, and CAT2 [(–262 C/T) and (–21 A/T)] allele genotyping. The SOD2 16 C/T genotype and C allele frequency differed significantly between controls and migraine (P = .047; P = .038). CAT –21 AA genotype and A allele frequency were significantly higher in both migraine with aura patients (P = .013; P = .004) and migraine without aura patients (P = .003; P = .001) compared to controls. To our knowledge, this is the first demonstration of differences in SOD and CAT genotypes between pediatric migraine patients and age-matched controls. Further studies on the functional implications of these genetic variants on neural antioxidant capacity and the use of antioxidant modulators for migraine treatment are warranted.

Acute Cerebellar Ataxia in a Pediatric Case of Lyme Disease and a Review of Literature

A broad range of neurologic disorders have been described in children with Lyme disease, of which peripheral facial nerve palsy and aseptic meningitis are among the most common. In contrast, there are few reports of cerebellar involvement in pediatric Lyme disease patients. We report the case of a 5-year-old girl seropositive for antibodies against the causative Lyme disease pathogen Borrelia burgdorferi presenting with severe acute cerebellar ataxia from the in southern coast of Anatolia (Mediterranean region).

Cerebellar Mutism Caused by Primary Varicella Infection in an Immunocompetent Child

Varicella (chickenpox) is a common childhood infection caused by the varicella-zoster virus, which is often self-limiting and usually benign. Although uncommon, neurologic complications of varicella have been documented that include postinfectious cerebellar ataxia, meningoencephalitis, Reye syndrome, myelitis, optic neuritis, stroke, Guillain-Barré syndrome, seventh cranial nerve palsy, and Ramsay-Hunt syndrome. In this case study, the authors describe a 7-year-old girl who presented with varicella skin rash with unsteady gait and anarthria on day 2, and her condition was attributed to cerebellar mutism. To date, this complication has never been reported in a child with primary varicella infection. Therefore, this case study documents a rare but serious complication of childhood chickenpox.