Acute flaccid myelitis outbreak through 2016-2018: A multicenter experience from Turkey.

AIM: We aim to describe the demographic characteristics, etiology, neurophysiology, imaging findings, treatment, prognosis, and prognostic factors of acute flaccid myelitis. METHODS: The clinical data, laboratory test and, magnetic resonance imaging (MRI) results of pediatric patients diagnosed with acute flaccid myelitis according to the Centers for Disease Control criteria between August 1, 2016, and December 31, 2018, from 13 centers in Turkey were reviewed. RESULTS: Of the 34 cases identified, 31 were confirmed (91.2%). Eighteen patients (55.9%) were boys. The median patient age was 4 years (interquartile range 2.5-6.9 years). Most of the patients were admitted in 2018 (n = 27). A preceding history of a febrile illness was reported in all patients, with a median of 4 days (interquartile range 3-7 days) before symptom onset. Thirty-one patients had T2 hyperintensity on spinal MRI, and 18 patients had cerebrospinal fluid pleocytosis. The most common infectious agents were entero/rhinoviruses (n = 5) in respiratory specimens. All patients except one received immunotherapy either alone or in combination. Among 27 patients with follow-up data 24 had persistent weakness. Involvement of four limbs together with an abnormal brain MRI at onset were associated with a poor prognosis. CONCLUSION: The number of patients with acute flaccid myelitis increased since 2012, spiking with every 2-year interval, largely in the pediatric population. The median age decreases with every outbreak. Clinicians should be aware of the clinical picture for early collection of specimens and early start of rehabilitation programs. Further studies are needed to better characterize the etiology, pathogenesis, risk factors, and treatment of this rare condition.

A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) and of immunoglobulins has been reported repeatedly, optimum application regimen of these drugs has not been established. This is partly due to the absence of common diagnostic and clinical standards focusing on neurological and psychosocial aspects. Carbamazepine, levetiracetam, and clobazam are the drugs most frequently used to control myoclonic jerks. We have established a consensus on essential laboratory and clinical parameters that should facilitate collaborative studies. Those are urgently needed to improve outcome.

Acute forearm compartment syndrome in a newborn caused by reperfusion after spontaneous axillary artery thrombosis.

Acute compartment syndrome of the forearm in newborns is often misdiagnosed and can be disastrous if left untreated. Here, we report a full-term infant of a diabetic mother with underlying heterozygosity for MTHFR C677T and A1298C alleles. A spontaneous thrombosis occurred in the left axillary artery immediately after birth. The patient responded well to anticoagulant (heparin) and thrombolytic (tissue plasminogen activator) agents. After reperfusion of the extremity, acute compartment syndrome developed. Emergent fasciotomy was performed. In this case, effective collaboration between pediatricians and orthopedic surgeons resulted in salvage of the extremity, with good clinical and functional results.

Ophthalmological Findings of Turkish Children With Muscular Dystrophies.

PURPOSE: To present the results of ophthalmological examinations in children with muscular dystrophies and highlight the importance of their ophthalmological evaluation. METHODS: Retrospective analysis of the ophthalmological examination records in 74 children with a type of muscular dystrophy, examined between January 2011 and January 2015, was performed. RESULTS: The most common type of muscular dystrophy observed in our patients was Duchenne muscular dystrophy (67.5%), followed by Becker muscular dystrophy (9.4%), myotonic dystrophy (8%), limb-girdle muscular dystrophy (6.7%), merosin-negative muscular dystrophy (4%), and Ullrich muscular dystrophy (4%). Ten cases of Duchenne muscular dystrophy had both macular and retinal pigmentary changes (20%) and 9 had abnormal electroretinographies with decreased photopic and scotopic responses. Ptosis was the most common finding (83.3%). No abnormalities of light reflexes, pupil size, or saccadic and smooth pursuit movements were seen among cases with myotonic dystrophy. CONCLUSIONS: Ophthalmological problems are commonly seen in children with muscular dystrophies. Simple ophthalmological screening and early intervention can improve their communication skills by way of increasing their visual talents.

Two infants with infantile spasms associated with vitamin B12 deficiency.

BACKGROUND: In developing countries, nutritional vitamin B12 deficiency in infants because of maternal deficiency often causes hematological and neurological disorders. However, epilepsy is a rare manifestation of vitamin B12 deficiency. The biological basis for the observed neurological symptoms of infantile vitamin B12 deficiency remains uncertain. There are only a few reports in the English literature regarding the relationship between infantile spasms and vitamin B12 deficiency. PATIENTS: We report two unrelated infants having infantile spasms associated with vitamin B12 deficiency related to maternal nutritional deficiency. RESULTS: During the first month of adrenocorticotropic hormone (ACTH), phenobarbital, and vitamin B12 treatments, both infants' abnormalities resolved. After 3 months, electroencephaography was completely normal. ACTH and phenobarbital treatments were ended. The children are disease-free 9 months after the treatment. CONCLUSIONS: We suggest that vitamin B12 deficiency should be considered in the differential diagnosis of infantile spasms as a treatable cause, especially with a history of maternal nutritional deficiency.

Bilateral optic neuritis--the only ocular finding in a case of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a rare disease of central nervous system caused by defective measles virus. Chorioretinitis with macular involvement is the mostly observed ocular finding in the disease. Other reported ocular findings in the disease are cortical blindness, hemianopsia, nystagmus, extraocular muscle paresis and optic atrophy. We present a rare case of subacute sclerosing panencephalitis with isolated bilateral optic neuritis as the only ocular finding without macular involvement.

Transient neonatal hyperparathyroidism: a presenting feature of sialidosis type II.

Sialidosis is a lysosomal storage disease caused by deficiency of α-N-acetyl neuraminidase-1. Sialidosis is classified into two main clinical variants: type I, the milder form of the disease, and type II, which can in turn be subdivided into three forms: congenital, infantile, and juvenile. We report a female patient with sialidosis type II, presenting with the congenital form of the disease with thrombocytopenia, pulmonary interstitial thickening, and transient secondary neonatal hyperparathyroidism.

Acute disseminated encephalomyelitis associated with acute Toxoplasma gondii Infection.

Acute disseminated encephalomyelitis is an acute demyelinating disorder of the central nervous system, which principally affects the brain and spinal cord. It usually follows a benign infection or vaccination in children. Although a number of infectious agents have been implicated in acute disseminated encephalomyelitis, Toxoplasma gondii infection has not been described previously in children. Acquired T. gondii infection presents with lymphadenopathy and fever and usually spontaneously resolves in immunocompetent patients. We describe a previously healthy 10-year-old boy with acute disseminated encephalomyelitis associated with acute acquired Toxoplasma gondii infection, the symptoms of which initially began with nuchal stiffness, difficulty in walking, and urinary and stool incontinence; he later had development of motor and sensory impairment in both lower extremities and classical magnetic resonance imaging lesions suggestive of the disease. The patient recovered completely after the specific therapy for acquired T. gondii infection and pulse prednisolone. Although acute acquired Toxoplasma gondii infection has not been reported previously in association with acute disseminated encephalomyelitis, clinicians should keep in mind this uncommon cause of a common disease when evaluating a patient with acute disseminated encephalomyelitis.

The socioeconomic and biological risk factors for developmental delay in early childhood.

UNLABELLED: To investigate the biological and socioeconomic factors associated with developmental attainment in socioeconomically disadvantaged children. This study was performed at the Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital, between January and December 2010. The effects of biological, socioeconomic risk factors on developmental delay were investigated in 692 children (3 months-5 years) using the Denver II. Low-level maternal education (odds ratio [OR], 11.118; 95 % CI, 4.211-29.351), low-level paternal education (OR, 2.107; 95 % CI, 1.333-3.331), low-level household income (OR, 2.673; 95 % CI, 1.098-2.549), and ≥ 3 children in the family (OR, 1.871; 95 % CI, 1.206-2.903) were strongly associated with abnormal on Denver II; biological risk factors, including birth weight, gestational age at birth, and maternal age at birth <20 years, were correlated with suspect on Denver II results based on univariate analysis. Low-level maternal education (OR, 6.281; 95 % CI, 2.193-17.989), premature birth (32-36 weeks of gestation; OR, 0.535; 95 % CI, 0.290-0.989) were strongly associated with abnormal on Denver II results, and low-level paternal education (OR, 3.088; 95 % CI, 1.521-6.268), low-level household income (OR, 1.813; 95 % CI, 1.069-3.077), low birth weight (<1,500 g; OR, 3.003; 95 % CI, 1.316-6.854), premature birth (27-31 weeks of gestation; OR, 2.612; 95 % CI, 1.086-6.286), and maternal age at birth <20 years (OR, 3.518; 95 % CI, 1.173-10.547) were strongly associated with suspect on Denver II results based on multivariate analysis. CONCLUSION: Socioeconomic risk factors were observed to be as important as biological risk factors in the development of children aged 3 months-5 years.

The association of anti-glutamic acid decarboxylase antibodies with different neurological findings in childhood.

Glutamic acid decarboxylase antibodies can rarely be associated with various neurological syndromes, which are usually present in adults. Here, we present 2 affected children. Our first patient had a diagnosis of epilepsy and presented with continuous involuntary movements and multifocal myoclonic seizures following an infection at the age of 9 months. Anti-glutamic acid decarboxylase antibodies were found in the serum and cerebrospinal fluid. A partial response was obtained from intravenous immunoglobulin, steroid, and plasmapheresis treatment. The other patient presented with a clinical picture of acute cerebellar ataxia and mutism at the age of 6 years and recovered fully following intravenous immunoglobulin treatment. Neurological findings due to anti-glutamic acid decarboxylase antibodies may be more common in children than previously thought, and achieving an early diagnosis can be important for prompt treatment.

Seizure due to somatostatin analog discontinuation in a case diagnosed as congenital hyperinsulinism novel mutation.

The most common reason for refractory hypoglycemia in newborns is congenital hyperinsulinism. We report a girl with congenital hyperinsulinism due to novel homozygous mutation (c.2041-25 G>A; aberrant splicing mutation) in the ABCC8 gene encoding SUR1 and during somatostatin analog (octreotide) discontinuation developed by nonhypoglycemic seizures. The newborn (birth weight of 3,750 g) was referred to our clinic because of hypoglycemic seizures at 4 h postnatal. On admission, blood glucose was 24 mg/dL and intravenous glucose infusion was started. The patient's insulin level was 27 mIU/mL during the hypoglycemic period. Phenobarbital (5 mg/ kg/day) was added because of short-acting generalized clonic seizures. Although the patient received high doses of diazoxide, esidrex, and octreotide approximately for 2 months, hypoglycemic episodes continued. Then the patient had near-total pancreatectomy, and pathology confirmed a diffuse form of congenital hyperinsulinism. There was homozygous mutation in the ABCC8 gene encoding SUR1, which confirmed the diagnosis of autosomal recessive congenital hyperinsulinism. During octreotide discontinuation, the patient developed non-hypoglycemic seizures, which were controlled by restarting the previous doses. In the light of in vitro and in vivo studies on antiepileptic effects of somatostatin, we believe that seizures in our case have developed secondary octreotide discontinuity.

Electroencephalogram variations in pediatric migraines and tension-type headaches.

This study evaluates specific electroencephalogram abnormalities in pediatric migraine and tension-type headaches, and demonstrates the clinical value of these abnormalities. We studied 50 migraine patients and 50 tension-type headache patients. Their mean age ± SD was 10.62 ± 3.21 (range, 5-16) years in the migraine group, and 13.00 ± 2.37 (7-16) years in the tension-type headache group. Diagnoses were rendered according to the International Classification of Headache Disorders, 2nd Edition, First Revision, of the International Headache Society. All patients underwent two waking-state electroencephalograms, one during a headache, and the other when headache-free. Thirty-six percent (18/50) of migraine patients and 12% (6/50) of tension-type headache patients revealed specific electroencephalogram abnormalities in headache attack electroencephalograms (P < 0.05). In headache-free period electroencephalograms, 16% (8/50) of the migraine group and 2% (1/50) of the tension-type headache group revealed abnormalities (P < 0.05). Our results indicate that electroencephalogram abnormalities are particularly prevalent in migraines, especially during headache attacks. This study is the first, to the best of our knowledge, on electroencephalographic evaluation of pediatric migraine and tension-type headache patients during both headache attacks and headache-free periods.

Transient acute flaccid paralysis and seizures associated with rotavirus gastroenteritis in a child.

Rotavirus is a common cause of acute gastroenteritis in young children. Neurological complications including seizures are known to accompany rotavirus gastroenteritis. Acute flaccid paralysis (AFP) associated with rotavirus has not been reported previously except for one report. Herein, we describe a case of transient AFP and seizures associated with rotavirus gastroenteritis. We think that transient AFP can be seen during mild rotavirus gastroenteritis in children, but further studies may be necessary to understand the role of rotavirus as a cause of AFP in children.

Neurologic manifestations of novel influenza A (H1N1) virus infection in childhood.

The neurologic manifestations and prognoses of a novel influenza A (H1N1) virus infection in previously healthy children were evaluated. Nose and throat swabs were retrieved from all patients who met the criteria of influenza-like illness. A real time reverse-transcriptase polymerase chain reaction assay was used to confirm the novel influenza A (H1N1) virus. This viral infection was evident in 240 children between October 10 and December 22, 2009. Neurologic findings were evident in 17 (7.08%) patients, aged between 4 months and 8 years. Nine were boys. Five patients manifested simple febrile seizures, seven manifested complex febrile seizures or additional afebrile seizures, and three manifested encephalopathy. Febrile status epilepticus and flaccid paralysis were diagnosed in one patient each. All were treated with oseltamivir. Fifteen of 17 patients demonstrated complete recovery. One undergoing follow-up with a diagnosis of Guillain-Barré syndrome manifested sequelae. One patient died because of septic shock and disseminated intravascular coagulation. We suggest that neurologic manifestations occur quite often in children aged less than 5 years with novel influenza A (H1N1) virus infection. Most infections were benign, although a severe course is possible, and sequelae may be encountered.