bluuebunny/crossref_metadata_2025 huggingface dataset:
Changes in Skeletal Maturation and Mineralization in Children With Cerebral Palsy and Evaluation of Related Factors
Authors: Yardimci Ilikkan Demet , Yalçin Erdinç
Published: March 2008

Sixty-nine (28 females, 41 males) children with spastic cerebral palsy and 26 (13 females, 13 males) healthy children were included in the study. Total- and partial-body bone mineral content and bone mineral density values of patient and control subjects were measured by dual-energy x-ray absorptiometry. Left hand and wrist radiographs of all patients and right hand and wrist radiographs of 39 randomly selected patients were taken, and the bone ages of all radiographs were determined. In both female and male tetraplegics, bone mineralization values of lower extremities, where the mobility disorder and effects of absence of weight-bearing activity were maximal, were lower than those of controls and hemiplegics (P < .05). In 47 (68%) patients, left-side bone age values were below normal ranges for their ages, and the difference was statistically significant (P < .01). Our results indicate that motor function handicap affects skeletal mineralization adversely, and skeletal maturation is frequently delayed in children with cerebral palsy. We speculated that this delay might be a result of disrupted embryologic skeletal development due to hypoxic attack, which also causes the disease. (J Child Neurol 2001;16:425-430).
Seizure Recurrence in Children With Focal Seizures and Single Small Enhancing Computed Tomographic Lesions: Prognostic Factors on Long-Term Follow-up
Authors: Arun Kumar Baranwal  , Singhi Pratibha D. , Singhi Sunit C. , Khandelwal Niranjan
Published: March 2008

Single small enhancing computed tomographic (CT) lesions are common in children with focal seizures. There is a paucity of information regarding their long-term outcome and prognostic factors for seizure recurrence. The objective of this work was to study the frequency of seizure recurrence in children with single small enhancing computed tomographic lesions and to identify prognostic factors, if any, for seizure recurrence. A prospective long-term follow-up was conducted at the Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, an urban tertiary care teaching hospital. Sixty-three children between 2 and 12 years of age with focal seizures for less than 3 months and single small enhancing computed tomographic lesions were enrolled in a randomized, double-blind, placebo-controlled trial of albendazole therapy and followed up for 4 years. On long-term follow-up, the albendazole and placebo groups were left with 29 and 28 children, respectively. After several months of seizure-free period, antiepileptic drug was tapered off. Children with relapse underwent magnetic resonance imaging examination. All children were followed up for at least 18 months after stopping of the antiepileptic drug. Seizure recurrence was seen in three children each in both groups, after a mean interval of 6.4 weeks after stopping the antiepileptic drug. Magnetic resonance imaging revealed persistent chronic granuloma in 2 and calcified granuloma in 4 children. Residual lesions were significantly correlated with seizure recurrence. In children whose lesions completely disappeared, no seizure recurrence was seen even during shorter periods of antiepileptic drug treatment. Seizure recurrence was seen in a small number of children with focal seizures and single small enhancing computed tomographic lesions. It appears to be related to either a persistent or a calcified lesion. (J Child Neurol 2001;16:443-445).
Successful Withdrawal From Analgesic Abuse in a Group of Youngsters With Chronic Daily Headache
Authors: Hering-Hanit Rachel , Cohen Avner , Horev Zeev
Published: March 2008

Abuse of ergotamine and analgesics is common in adults. It coexists with headache and can also induce headaches. Ten to 15% of patients attending headache clinics and 1% of the general population suffer from chronic daily headache due to medication misuse. Indeed, this phenomenon was recently regarded as an epidemic. Nonetheless, analgesic-induced headache in children and adolescents was first reported in 1998. We report on our experience with children and adolescents with daily or almost-daily headache concomitant with daily or almost-daily analgesic intake. Over a period of 3 years, we evaluated 26 children (19 girls and 7 boys) with chronic daily or near-daily headache related to daily analgesic intake. The mean age of the group was 14.2 years (range, 12-18), and the mean headache history duration was 1.6 years (range, 3 months to 4½ years). The mean number of headache days per month was 28.1 (range, 19-31). All patients had no history of migraine prior to the chronic headache phase according to the International Headache Society criteria. They were using at least one dose of analgesic drug for each headache, whereas 16 were using analgesic drugs daily. The weekly analgesic intake averaged 28.1 tablets (range, 19-41). The majority abused simple analgesics. Twenty-one took acetaminophen alone. Five took a combination; four took a compound containing acetaminophen, caffeine, and codeine; and the fifth patient took a compound containing aspirin, caffeine, and codeine. All patients were informed about the phenomenon of medication-induced headache and were encouraged to achieve drug withdrawal. Withdrawal led to complete cessation of all headaches in 20 patients. In 5 patients, the daily headache resolved; however, they suffered from intermittent episodic migraine attacks, which were frequent enough in 3 to initiate prophylactic medication. One adolescent continued to have daily headache. Analgesic-induced headache does occur in adolescents. Successful withdrawal from the offending analgesics was achieved without hospitalization or significant interference with daily life and with complete disappearance of the induced chronic daily headache in 25 of 26 patients. (J Child Neurol 2001;16:448-449).
Heliox Use in the Treatment of Acute Hyperammonemia
Authors: Barr Joseph , Eshel Gideon , Chen-Levy Zehava , Lahat Eli
Published: March 2008

The objective of this study was to evaluate a new method for the treatment of acute hyperammonemia with a helium-oxygen mixture (heliox). We conducted a prospective, randomized, controlled study of male Sprague-Dawley rats. Experimental hyperammonemia was induced by 7 days of a high-ammonia diet. Subsequently, the animals were randomly divided into two groups: the study group treated with heliox breathing for 24 hours and a control group breathing room air for 24 hours. A prospective, randomized, controlled laboratory animal study was conducted at an animal research facility. The baseline plasma ammonia level was 9.49 ± 10.96 μmol/L. After 7 days of a high-ammonia diet, the plasma ammonia level rose to 31.53 ± 8.86 μmol/L. There was a significant statistical difference between the plasma ammonia level following 24 hours of heliox therapy ( 23.14 ± 13.97 μmol/L) and the ammonia level in the control group (42.31 ± 24.25 μmol/L) (P < .05). Heliox breathing was found to be an efficient treatment modality for decreasing plasma ammonia levels in an animal model. Further studies are required to evaluate its potential application in the treatment of patients with hyperammonemia. (J Child Neurol 2001;16:456-458).
Idiopathic "Benign" Intracranial Hypertension: Case Series and Review
Authors: Salman Michael S. , Kirkham Fenella J. , MacGregor Daune L.
Published: March 2008

Idiopathic "benign" intracranial hypertension is an uncommon but important cause of headache that can lead to visual loss. This study was undertaken to review our experience in the diagnosis and management of idiopathic intracranial hypertension, giving special attention to treatments used. A retrospective chart review was conducted on 32 patients diagnosed with idiopathic intracranial hypertension between 1984 and 1995. Subjects included 23 females and ranged in age from 2 to 17.5 years. Headache was the most common symptom, followed by nausea and vomiting, double vision, and visual loss. Papilledema was the most common sign. Others were VIth cranial nerve palsy and compromised visual acuity at or within 3 months of presentation. Management included administration of acetazolamide or corticosteroids, lumboperitoneal shunt, optic nerve fenestration, and repeat lumbar puncture. Treatment combinations were used in 40% of cases. During follow-up, headache, papilledema, and decreased visual acuity persisted for longer than 10 months in a significant number of patients. We conclude that idiopathic intracranial hypertension causes significant short- and long-term morbidity with no proven effective treatment available. A prospective study is needed to establish the indications for treatment and the efficacy of the treatments used. (J Child Neurol 2001;16:465-470).
Childhood Guillain-Barré Syndrome: Clinical and Electrophysiologic Features Predictive of Outcome
Authors: Ammache Zakaria , Afifi Adel K. , Brown Carl Kice , Kimura Jun
Published: March 2008

The hospital records of 27 children with the diagnosis of Guillain-Barré syndrome were retrospectively reviewed. Based on the time to recovery, patients were divided into two groups: group 1 consisted of children whose times to complete or partial recovery extended beyond 2 months from onset of the disease; group 2 consisted of children who attained a full recovery within 2 months from onset of the disease. The clinical and electrophysiologic features of the two groups were statistically compared for their predictive value of outcome. Three clinical features (maximum disability score at presentation, intubation, and cranial nerve involvement) were predictive of poor outcome, whereas one electrophysiologic feature (conduction block) was predictive of favorable outcome. There was also a significant correlation between maximum disability score at presentation and the probability of intubation. (J Child Neurol 2001;16:477-483).
A5814G Mutation in Mitochondrial DNA Can Cause Mitochondrial Myopathy and Cardiomyopathy
Authors: Karadimas Charalampos , Tanji Kurenai , Geremek Maciej , Chronopoulou Penelope , Vu Tuan , Krishna Sindu , Sue Carolyn M. , Shanske Sara , Bonilla Eduardo , DiMauro Salvatore , Lipson Mark , Bachman Ronald
Published: March 2008

We describe a 5-year-old child with hypertrophic cardiomyopathy, mitochondrial myopathy, and lactic acidosis. Mitochondrial DNA analysis showed a heteroplasmic A5814G point mutation in the tRNACys gene. The mutational load was extremely high (>95%) in muscle, fibroblasts, and blood. This report expands the clinical heterogeneity of the A5814G mutation, which should be considered in the differential diagnosis of hypertrophic cardiomyopathy in childhood. (J Child Neurol 2001;16:531-533).
Acute Encephalopathy Associated With Nontyphoidal Salmonellosis
Authors: Arii Junko , Tanabe Yuzo , Miyake Masami , Noda Masatoshi , Takahashi Yoshihito , Hishiki Haruka , Kohno Yoichi
Published: March 2008

The importance of an acute encephalopathy associated with nontyphoidal salmonellosis has recently been recognized, but the disease entity has been poorly established. In this study, we describe two encephalopathic patients associated with nontyphoidal salmonellosis. The patients exhibited a rapid evolution of coma after the onset of lethargy or seizure. Fever and diarrhea due to salmonellosis preceded these events. Secondary factors inducing encephalopathies, such as severe dehydration, sepsis, meningitis, electrolyte or metabolic disturbances, acute renal failure, and multiple organ failure, were excluded in the differential diagnosis at the onset of encephalopathic features. These clinical findings and rapid development of encephalopathic features from localized intestinal infection without any significant abnormalities in a variety of blood tests may suggest a toxic etiology. However, endotoxin was not found in serum from both patients. From these results, we conclude that nontyphoidal salmonellosis can cause a toxic encephalopathy syndrome, like shigellosis or verocytotoxin-pro ducing Escherichia coli infection. (J Child Neurol 2001;16:539-540).
Absence of Hippocampal Sclerosis in Children With Multiple Daily Seizures Since Infancy
Authors: Kothare Sanjeev V. , Sotrel Ana , Duchowny Michael , Jayakar Prasanna , Marshall Paul C. , Smith Thomas W.
Published: March 2008

We report a series of nine children with multiple daily seizures since infancy who underwent functional hemispherectomy that included en bloc resection of the hippocampus and the temporal neocortex. In all cases, the hippocampi were normal by conventional histology despite the fact that these patients had suffered from recurrent seizures over a long period of time. This observation suggests that extremely frequent seizures in childhood are not invariably associated with the development of hippocampal sclerosis. (J Child Neurol 2001;16:562-564).
Should Children With Bell's Palsy Be Treated With Corticosteroids? A Systematic Review
Authors: Salman Michael S. , MacGregor Daune L.
Published: March 2008

The objective of this study was to evaluate the effect of corticosteroids in the treatment of pediatric Bell's palsy. A systematic review of trials that included pediatric (< 16 years old) cases with Bell's palsy and involved the use of steroids was conducted. Eight trials were identified, five of which were randomized, and prednisone was used in six trials, whereas corticotropin was used in the other two. The methods of randomization and allocation concealment of the treatments used were rarely reported. Only one trial was done exclusively in children; none of the other seven trials analyzed the pediatric cases separately. Four trials reported some benefit from steroids. The pediatric trial did not provide evidence for benefit from corticosteroids. There was substantial heterogeneity in the population and interventions used; hence a meta-analysis was not done. Based on this systematic review, we do not recommend the routine use of steroids in children with Bell's palsy. (J Child Neurol 2001;16:565-568).
Video-Assisted Thoracoscopic Thymectomy in Juvenile Myasthenia Gravis
Authors: Kolski Hanna K. , Kim Peter C.W. , Vajsar Jiri
Published: March 2008

As video-assisted thoracoscopic surgery for thymectomy has been reported to be as effective as traditional open surgical approaches in predominantly adult patients with myasthenia gravis, we applied this procedure to juvenile patients with this condition. Six patients underwent total thymectomy using the video-assisted technique (1997-98). Six patients in whom a median transsternal approach was used (1989-95) formed the comparison group. The two patient groups were similar in terms of age at thymectomy and preoperative clinical severity. There were no serious perioperative complications in either group. Minimum post-thymectomy duration of follow-up in the video-assisted thoracoscopic surgery patients was 2.3 years (mean 2.7 years), with all patients clinically improved over their baseline status. Preliminary results suggest that video-assisted thymectomies are comparably effective to transsternal procedures in treating generalized juvenile myasthenia gravis and can be safely performed in children as young as 20 months of age. In addition, video-assisted surgeries are less invasive than transstemal approaches, significantly shorten the postoperative hospital stay, and have superior cosmetic results. (J Child Neurol 2001;16:569-573).
Cerebral Venous Thrombosis in Children
Authors: Carvalho Karen S. , Bodensteiner John B. , Connolly Patrick J. , Garg Bhuwan P.
Published: March 2008

Cerebral venous thrombosis is an important cause of stroke in children. Understanding the natural history of the disease is essential for rational application of new interventions. We retrospectively identified 31 children with cerebral venous thrombosis confirmed by head computed tomography (4 patients) or by magnetic resonance imaging (27 patients). Risk factors, clinical and radiographic features, and neurologic outcomes were analyzed. There were 21 males and 10 females aged 1 day to 13 years (median 14 days). Nineteen (61%) were neonates. The most common risk factors included mastoiditis, persistent pulmonary hypertension, cardiac malformation, and dehydration. The chief clinical features were seizures, fever, respiratory distress, and lethargy. Fifteen patients had infarctions (8 hemorrhagic, 7 ischemic). Protein C and antithrombin III deficiency were the most common coagulopathies among 14 tested patients. On discharge, 11 patients were normal, 17 had residual deficits, and 2 patients died. Twenty-seven patients were followed from 1 month to 12 years (mean 22 months). At follow-up, 11 patients were normal, and 13 patients had development delay. One had residual hemiparesis and cortical visual impairment. Two had other deficits. Neonatal cerebral venous thrombosis is probably more common than previously thought, and outcomes are worse in this group. All children with cerebral venous thrombosis should be tested for coagulation disorders. (J Child Neurol 2001;16:574-580).
Medullomyoblastoma: Case Report
Authors: Cheema Zahid F. , Cannon Thomas C. , Leech Richard , Brennan James , Adesina Adekunle , Brumback Roger A.
Published: March 2008

This 7-year-old boy presented with a 2-week history of headache, nausea, vomiting, anorexia, lethargy, and unsteadiness of gait. Brain magnetic resonance imaging (MRI) revealed a cystic mass within the vermis of the cerebellum. A suboccipital craniectomy was performed to remove a tumor that contained primitive neuroectodermal cells with florid skeletal muscle differentiation. Immunohistochemical studies and electron microscopy confirmed the presence of both a primitive neuroectodermal component and rhabdomyoblastic differentiation, consistent with the diagnosis of medullomyoblastoma. This exceedingly rare tumor of the cerebellar vermis of children is characterized by two components: primitive neuroectodermal tumor cells and skeletal muscle. Although the histogenesis remains uncertain, advances in immunohistochemistry and electron microscopy suggest the origin of this tumor from a multipotential stem cell precursor. (J Child Neurol 2001 ; 16 :598-599).
Acetazolamide Treatment for Infantile Central Sleep Apnea
Authors: Philippi Heike , Bieber Imke , Reitter Bernd
Published: March 2008

Central sleep apnea is a common respiratory pattern in healthy neonates. Nevertheless, frequent central sleep apnea associated with drops in oxygen saturation may contribute to infantile morbidity. Recently, low-dose acetazolamide was shown to reduce symptomatic central sleep apnea in adults. We treated 12 infants, median conceptional age 42 weeks (range, 40-44 weeks), with central sleep apnea. In all cases, the central apnea index was >40/h total sleeping time (apnea ≥ 3 sec). The cumulative duration of drops in oxygen saturation below 90% was more than 3 min/h total sleeping time. All individuals received acetazolamide 7 mg/kg/day (orally, divided in three doses) for 11 weeks. Polysomnography was begun 10 hours before the first dose and continued for 10 hours after the third dose. Polysomnography was repeated after 6 weeks of treatment and 1 week after acetazolamide therapy was discontinued. Comparison of the respiratory patterns before and after treatment (10-hour recording after the third dose) showed a decrease in the median central apnea index from 74/h (range, 42-152/h) to 13/h (range, 6-49/h). The median of the cumulative duration of drops in oxygen saturation below 90% decreased from 3.6 min/h (range, 3.1-9.2 min/h) to 0.07 min/h (range, 0-0.5 min/h). Basal oxygen saturation increased from 95 (92-97%) to 98% (96-99%). This improvement was maintained in the final polysomnography (12 weeks after therapy was begun and 1 week after completion of the 11-week course). No adverse effects were noted. We conclude that low-dose acetazolamide treatment may be useful for the treatment of central infantile sleep apnea associated with hypoxemia. (J Child Neurol 2001;16:600-603).
Neonate With Spinal Hypoplasia on T12 and a Localized Vertebral Malformation on L4
Authors: Weber Astrid , Maier Rolf F , Felderhoff-Mueser Ursula , Lehmann Rüdiger , Stöver Brigitte , Obladen Michael
Published: March 2008

We report a case of a neonate with sectional narrowing of the spinal cord on the level of T12 to L2 and a deformed vertebral body on a different level, L4. In previously described cases of sectional spinal dysgenesis, the vertebral and spinal cord malformations are usually found on the same level. Our case may represent a new variant of spinal dysgenesis. (J Child Neurol 2001;16:619-621).
The Ketogenic Diet: An Effective Medical Therapy With Side Effects
Authors: Wheless James W.
Published: March 2008

Attention is directed to the fact that the ketogenic diet is much less toxic than some of the medications currently used in the treatment of epilepsy. (Dr Samuel Livingston, 1972) 1

The ketogenic diet... is an effective and safe medical treatment for epilepsy, but it must be judiciously applied and carefully monitored. (Drs Doug R. Nordli and Daryl C. DeVivo, 2001)2
Carnitine-Deficient Myopathy as a Presentation of Tyrosinemia Type I
Authors: Nissenkom Andrea , Korman Stanley H. , Vardi Orna , Levine Arie , Katzir Zeev , Ballin Ami , Lerman-Sagie Tally
Published: March 2008

Carnitine deficiency secondary to renal Fanconi's tubulopathy has been described in only a few inborn errors of metabolism : cystinosis, galactosemia, and Fanconi-Bieckel syndrome. We report a 27-month-old infant who presented with a sudden change in gait owing to proximal muscle weakness. The laboratory evaluation showed carnitine deficiency associated with Fanconi's tubulopathy. Eventually, tyrosinemia type I was diagnosed. Carnitine deficiency can contribute to the clinical picture of hepatorenal tyrosinemia and should therefore be evaluated and treated. ( J Child Neurol 2001;16:642-644).
Acute Pancreatitis Causing Death in a Child on the Ketogenic Diet
Authors: Stewart Wendy A. , Gordon Kevin , Camfield Peter
Published: March 2008

The ketogenic diet has demonstrated good efficacy in children with pharmacologically resistant seizures. Relatively few serious complications have been reported in the more than 70 years in which the diet has been used. We report a child who developed acute pancreatitis and died. A 9-year-old girl had a seizure disorder with associated developmental delay owing to glucose transport protein deficiency. The ketogenic diet with medium chain triglyceride oil had been initiated shortly after diagnosis in infancy. She was not on anticonvulsants. She presented in coma with decreased respiratory effort and shock, requiring resuscitation. Investigations were consistent with pancreatitis. Despite fluid resuscitation and inotropic support, she had prolonged hypotension and acidosis. She subsequently had a cardiac arrest and died. A postmortem examination confirmed hemorrhagic pancreatitis. Hypertriglyceridemia is a risk factor for developing acute pancreatitis. The high fat content of the ketogenic diet often causes hyperlipidemia. The outcome for this patient raises concern regarding a potential consequence of the ketogenic diet. (J Child Neurol 2001;16:682).
Homocystinuria With Transverse Sinus Thrombosis
Authors: Buoni Sabrina , Molinelli Massimo , Mariottini Aldo , Rango Caterina , Medaglini Sergio , Pieri Sergio , Strambi Mirella , Fois Alberto
Published: March 2008

A case of cerebral venous thrombosis caused by undiagnosed homocystinuria is reported. The pitfalls regarding the diagnosis of a potentially medically treatable condition are discussed. Cerebral venous thrombosis in children has a variable type of onset and a multiplicity of causes. This type of pathology, although not frequent, is more common than previously thought. Among the different etiologies, undiagnosed homocystinuria is not routinely considered. We report a case of venous thrombosis of the left transverse cerebral sinus in a girl with drug-resistant partial epilepsy and homocystinuria. This diagnosis was considered and confirmed after the appearance of acute cerebral symptoms caused by venous thrombosis. (J Child Neurol 2001;16:688-690).
Optic Neuropathy in Primary Antiphospholipid Syndrome in Childhood
Authors: Beşbaş Nesrin , Anlar Banu , Apak Anil , Kansu Tulay
Published: March 2008

Neurologic symptoms associated with antiphospholipid antibodies in children include thrombotic events, unilateral movement disorders, or migraine. We present a 7-year-old girl with bilateral optic neuropathy, cerebral white-matter lesions, and antiphospholipid IgM that responded to prednisone and tended to relapse when it was stopped. Remission was obtained under maintenance corticosteroid therapy, and the antiphospholipid antibodies disappeared.This case suggests a role for antiphospholipid antibodies in the pathogenesis of optic neuropathy in childhood. (J Child Neurol 2001;16:690-693).
Visual Agnosia After Treatment of a Posterior Fossa Ependymoma in a 16-Month-Old Girl
Authors: Kieffer-Renaux Virginie , Bulteau Christine , Grill Jacques , Levy-Piebois Christine , Couanet Dominique , Pierre-Kahn Alain , Hartmann Olivier , Kalifa Chantal
Published: March 2008

We present the clinical observation of a 16-month-old girl treated for a posterior fossa ependymoma who experienced severe and delayed visual dysfunction. She was initially treated by surgery and conventional chemotherapy. When she relapsed at age 3 years, the salvage treatment combined high-dose chemotherapy, second surgery, and local irradiation. At age 4 years, disturbed gait and dysarthric speech appeared rapidly, and she became unable to recognize objects and people. Computed tomography revealed bilateral calcifications in the cerebellum and temporal and occipital lobes but no relapse. The neuropsychologic evaluations revealed signs of visual agnosia and marked intellectual impairment. The role of the different treatment modalities in the pathogenesis of this unusual syndrome is discussed. ( J Child Neurol 2001;16:698-704).
Concentrations of Nucleotides, Nucleosides, Purine Bases, Oxypurines, Uric Acid, and Neuron-Specific Enolase in the Cerebrospinal Fluid of Children With Sepsis
Authors: Rodríguez-Núñez Antonio , Cid Elena , Rodríguez-García Javier , Camifia Félix , Rodriguez-Segade Santiago , Castro-Gago Manuel
Published: March 2008

To determine the effects of sepsis on cerebral energy metabolism, the cerebrospinal fluid adenosine monophosphate, inosine monophosphate, inosine, adenosine, guanosine, hypoxanthine, xanthine, and urate concentrations were determined by high-performance liquid chromatography and the neuron-specific enolase levels by means of an enzyme immunoassay method in 32 children with sepsis, without meningitis, aged between 2 months and 13 years, and in 160 age-matched controls. The septic group had significantly higher cerebrospinal fluid concentrations of inosine, adenosine, xanthine, and urate than controls. These results suggest that sepsis could provoke some degree of neuronal hypoxia and significant alterations of cerebral energy metabolism homeostasis. (J Child Neurol 2001;16:704-706).
Dizziness and Headache: A Common Association in Children and Adolescents
Authors: Weisleder Pedro , Fife Terry D.
Published: March 2008

Vertigo has long been recognized by the clinician as a frequent accompanying symptom of the adult migraine syndrome. This association has not been so readily identified in the pediatric population, and, as a consequence, children undergo unnecessary evaluations. We reviewed the charts of all children and adolescents referred for vestibular function testing to the Balance Center at the Barrow Neurological Institute between July 1994 and July 2000 (N = 31). Items analyzed included age, gender, symptoms that prompted the referral, test outcomes, family medical history, and final diagnosis. The most common justification for vestibular testing referral was the combination of dizziness and headache. Other less common reasons were "passing out" episodes, poor balance, and blurred vision. Normal test results were obtained from 70% of patients ( n = 22). The most common abnormal test outcome was unilateral vestibular dysfunction (n = 5). Bilateral peripheral vestibular dysfunction was present in three patients. One patient had central vestibular dysfunction. The final diagnoses were vestibular migraine (n = 11), benign paroxysmal vertigo of childhood (n = 6), anxiety attacks (n = 3), Meniere's disease (n = 2), idiopathic sudden-onset sensorineural hearing loss (n = 1), vertigo not otherwise specified ( n = 1), familial vertigo/ataxia syndrome (n = 1), and malingering (n = 1); in five patients, no definitive diagnosis was established. The stereotypical patient with vestibular migraine was a teenage female with repeated episodes of headache and dizziness, a past history of carsickness, a family history of migraine, and a normal neurologic examination. Patients who fit this profile are likely to have migrainous vertigo. Consequently, a trial of prophylactic migraine medication should be considered for both diagnostic and therapeutic purposes. Brain imaging and other tests are appropriate for patients whose symptoms deviate from this profile. (J Child Neurol 2001;16:727-730).
Cerebrovascular Disorders in Children With the Factor V Leiden Mutation
Authors: Lynch John Kylan , Nelson Karin B. , Curry Cynthia J. , Grether Judith K.
Published: March 2008

Since 1995, at least 128 children with a cerebrovascular disorder, cerebral palsy, or both and the factor V Leiden mutation have been reported. The majority of these strokes were in the first year of life, many of them in the perinatal period. Two thirds had an additional exogenous risk factor for thrombosis, and 42% had another recognized endogenous prothrombotic risk factor in combination with the mutation. We review the association of the factor V Leiden mutation and a cerebrovascular disorder in children younger than 16 years of age and describe the clinical features of 8 children with cerebral palsy and the Leiden mutation. This mutation should be considered in the evaluation of children with a stroke or its sequelae, including infants with perinatal stroke. (J Child Neurol 2001;16:735-744).
Aicardi-Goutieres Syndrome in Siblings
Authors: Koul Roshan , Chacko Alexander , Joshi Surendranath , Sankhla Dilip
Published: March 2008

Two siblings with familial encephalopathy, calcification of the basal ganglia, and cerebrospinal fluid lymphocytosis, constituting the triad of Aicardi-Goutieres syndrome, are reported. This syndrome resembles congenital intrauterine infections, which must be meticulously excluded. Aicardi-Goutieres syndrome is extremely rare and is being reported from the Arab world for the first time to our knowledge. (J Child Neurol 2001;16:759-761).
Multiple Neuroendocrine Disorder in Salla Disease
Authors: Grosso Salvatore , Berardi Rosario , Farnetani Maria Angela , Margollicci Maria , Mancini Maria Grazia , Morgese Guido , Balestri Paolo
Published: March 2008

Salla disease represents the slowly progressive adult form of the sialic acid storage diseases, a group of autosomal-recessive neurodegenerative disorders in which psychomotor development, ataxia, axial hypotonia, and spasticity in the lower limbs occur. No skeletal dysostosis or organomegaly is present, and life expectancy is normal. Short stature can also be observed. Progressive cerebral and cerebellar atrophy associated with dysmyelination and corpus callosum hypoplasia have been shown by magnetic resonance imaging studies. We report the first patient with Salla disease in whom combined growth hormone and gonadotropin deficiencies, hypothalamic pituitary in origin, have been demonstrated by neuroendocrine studies. We believe that the multiple neuroendocrine disorder may be the consequence of the abnormalities of common neuronal pathways regulating growth hormone and gonadotropin synthesis or secretion related to the brain storage of free sialic acid and/or to the neurodegenerative process occurring in Salla disease. Therefore, a complete endocrinologic evaluation of these patients is both warranted and useful. (J Child Neurol 2001;16:775-777).
Subjective Reactions of Children to Single-Pulse Transcranial Magnetic Stimulation
Authors: Garvey Marjorie A. , Kaczynski Karen J. , Becker Danielle A. , Bartko John J.
Published: March 2008

Single-pulse transcranial magnetic stimulation is a useful tool to investigate cortical function in childhood neuropsychiatric disorders. Magnetic stimulation is associated with a shock-like sensation that is considered painless in adults. Little is known about how children perceive the procedure. We used a self-report questionnaire to assess children's subjective experience with transcranial magnetic stimulation. Normal children and children with attention-deficit hyperactivity disorder (ADHD) underwent transcranial magnetic stimulation in a study of cortical function in ADHD. Subjects were asked to rate transcranial magnetic stimulation on a 1 to 10 scale (most disagreeable = 1, most enjoyable = 10) and to rank it among common childhood events. Thirty-eight subjects completed transcranial magnetic stimulation; 34 said that they would repeat it. The overall rating for transcranial magnetic stimulation was 6.13, and transcranial magnetic stimulation was ranked fourth highest among the common childhood events. These results suggest that although a few children find transcranial magnetic stimulation uncomfortable, most consider transcranial magnetic stimulation painless. Further studies are necessary to confirm these findings. (J Child Neurol 2001;16:891-894).
Holoprosencephaly (Lobar Form) Associated With Bilateral Vocal Cord Palsy
Authors: Smilari Pierliugi , Serra Agostino , La Mantia Ignazio , Parano Enrico , Pavone Piero
Published: March 2008

There have been many causes associated with bilateral vocal cord palsy, both congenital and perinatal. Until now, the main congenital causes of bilateral vocal cord palsy have been associated with meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. We report a patient with bilateral vocal cord palsy associated with the lobar form of holoprosencephaly. In this case, bilateral vocal cord palsy was caused by the disruption of the cortical laryngeal motoneurons. Neonatal stridor must be carefully evaluated by the neonatologist to exclude severe cerebral anomalies. (J Child Neurol 2001;16:932-934).
Attenuation of Somatosensory Evoked Potentials During Positioning in a Patient Undergoing Suboccipital Craniectomy for Chiari I Malformation With Syringomyelia
Authors: Anderson Richard C. , Emerson Ronald G. , Dowling Kathryn C. , Feldstein Neil A.
Published: March 2008

Intraoperative electrophysiologic monitoring can diminish the risk of neurologic injury by enabling the detection of injury at a time when it can be reversed or minimized. This report describes a 14-year-old girl with a Chiari's malformation type I and syringomyelia who underwent a suboccipital decompression and dural patch grafting with concurrent somatosensory evoked potentials. When the patient was turned into the prone position and the neck was flexed, the left-sided somatosensory evoked potential deteriorated. After the patient's neck was repositioned, the left median nerve potential improved but did not return to baseline. Postoperatively, the patient had decreased proprioception of her left arm, which completely resolved at 2-week follow-up. This single case report does not establish the need for routine somatosensory evoked potential monitoring. Nevertheless, deterioration of the potential in this case led directly to a change in the surgical positioning, which may have significantly reduced the chances of a permanent neurologic injury. (J Child Neurol 2001;16:936-939).
Topical Review : The Cerebellum: It's About Time! But Timing Is Not Everything-New Insights Into the Role of the Cerebellum in Timing Motor and Cognitive Tasks
Authors: Salman Michael S.
Published: March 2008

Converging evidence from different research studies supports a role for the cerebellum in timing neural processes. The cerebellum is part of a distributed system for motor control. The timing hypothesis provides a specific functional role for the unique contribution of the cerebellum. The timing capabilities of the cerebellum appear to extend beyond motor control into tasks focusing on perceptual processing that require the precise representation of temporal information and sensorimotor learning. Behavioral and modeling studies suggest that the cerebellar timing system is best characterized as providing a near-infinite set of interval-type timers rather than as a single clock with pacemaker or oscillatory properties, but this is controversial. In addition to learning precisely timed motor responses, the cerebellum is involved in on-line processing using feed-forward systems for which sensory input is used prior to movement execution to improve movement accuracy. This would be a mechanism for triggering accurate "time." The cerebellum continues to fascinate scientists, and although survival is possible without the cerebellum, the resultant quality of life is significantly compromised with clumsiness, ataxia, hypotonia, dysarthria, slowing of various cognitive perceptual processes, and impaired fine motor and ocular-motor coordination. The last three decades have seen the development of research that has focused on how the cerebellum functions. Further neurophysiologic research in cerebellar cortical neurotransmission is likely to further our understanding of the cerebellar contribution to timing sensorimotor processes. (J Child Neurol 2002; 17:1-9).
Subacute Sclerosing Panencephalitis: Clinical and Magnetic Resonance Imaging Evaluation of 36 Patients
Authors: Öztürk Aylin , Gürses Candan , Baykan Betül , Gökyiğit Ayşen , Eraksoy Mefkure
Published: March 2008

We studied 36 patients (24 males, 12 females), all of whom had definite subacute sclerosing panencephalitis with typical periodic complexes in their electroencephalograms and increased titers of measles antibody in serum and cerebrospinal fluid. Their clinical and laboratory findings on admission were reviewed retrospectively. The age at onset of symptoms varied from 4 to 23 years. The average age at onset of disease was 13.1 ± 4.18 years. The mean of the duration from the infection to the onset of subacute sclerosing panencephalitis was 9 years. Unusual symptoms, especially in the early periods of disease, included hemiparesis (7 patients), headache (3), generalized tonic-clonic seizures (6), absence seizure (1), nausea (3), and vomiting (3). Twenty-six cranial magnetic resonance imaging (MRI) and 12 computed tomography examinations were performed. Nine patients had normal MRI. In the early stages, lesions usually involved parieto-occipital corticosubcortical regions asymmetrically. In time, symmetric periventricular white-matter changes became more prominent. In addition to the common clinical findings in cases of subacute sclerosing panencephalitis reported in the literature, there were some different clinical features of the disease. Eventually, we concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings. (J Child Neurol 2002;17:25-29).
Diffuse Magnetic Resonance Imaging White-Matter Changes in a 15-Year-Old Boy With Mitochondrial Encephalomyopathy
Authors: Weinstock Arie , Giglio Pierre , Cohen Michael E. , Bakshi Rohit , Januario John , Balos Lucy
Published: March 2008

We report a 15-year-old boy who presented with recurrent headaches associated with numbness, confusion, and speech difficulty. Brain magnetic resonance imaging (MRI) revealed diffuse bilateral white-matter hyperintensity on fluid-attenuated inversion-recovery and T2-weighted images in the internal capsule, periventricular and subcortical white matter, base of the pons, and middle cerebellar peduncles. Lesions were isointense on T1weighted images and nonenhancing. Muscle biopsy showed changes consistent with a mitochondrial myopathy. Mitochondrial respiratory chain enzyme activity analysis revealed a significant reduction in complex II activity. Genetic testing was negative. We conclude that mitochondrial encephalomyopathy may present with unusual diffuse supratentorial and infratentorial white-matter changes on MRI. (J Child Neurol 2002;17:47-49).
Cavanagh's Syndrome (Congenital Thenar Hypoplasia)
Authors: Sonel Birkan , Senbil Nesrin , Gurer YK. Yavuz , Evcik Deniz
Published: March 2008

Thenar hypoplasia can be an isolated defect, as in Cavanagh's syndrome, can be present with cardiac (Holt-Oram syndrome) or eye (Okihiro's syndrome) disorders, or can be associated with hand anomaly, as in Haas's malformation. Vascular abnormality may be associated with thenar hypoplasia, which has been demonstrated in Okihiro's syndrome. Cavanagh's syndrome is a rare anomaly of the upper extremities that presents with unilateral or bilateral hypoplasia of the thenar eminence. Typical clinical, radiographic, and electrophysiologic findings emphasize the diagnosis. Differentiation from carpal tunnel syndrome is important to prevent unnecessary intervention. Electrophysiologic and radiographic findings are necessary tools for the physician to establish a correct diagnosis and make an appropriate referral. Because of its rarity, we present the case of an 8-year-old girl with this syndrome. (J Child Neurol 2002;17:51-54).
Syndrome of Cerebrospinal Fluid Hypovolemia Following Lumbar Puncture Cerebrospinal Fluid Leak in a Patient With Idiopathic Intracranial Hypertension
Authors: Koul Roshan , Chacko Alexander , Javed Hashim , Jain Rajeev , Ganesh Anuradha , Srinivasan Sinnakirouchanana
Published: March 2008

An 11-year-old girl presented with headache of 3 months' duration. There was bilateral disc edema. The cerebrospinal fluid pressure was 50 cm of water with normal cerebrospinal fluid cytology and biochemistry. She developed severe headache (different and disabling), dizziness, vomiting, and backache on sitting up 6 hours after lumbar puncture, and lying supine relieved all of her symptoms. Intravenous fluids, analgesics, and complete bed rest did not relieve her symptoms over the next 72 hours. She was completely relieved of her symptoms on receiving two tablets of Caffergot containing 200 mg of caffeine and 2 mg of ergotamine 72 hours after lumbar puncture. The symptoms recurred 48 hours later, and a repeat dose of Caffergot was required. Magnetic resonance imaging (MRI) done 96 hours after lumbar puncture revealed the entire dura overlying the brain, including the posterior fossa, showing intense enhancement on contrast injection with leak at the lumbar puncture site. Oral caffeine (coffee, three times a day) was advised over 1 week. The patient remained asymptomatic, and a repeat MRI scan after 10 days showed complete clearing of the cerebrospinal fluid leak with no dural enhancement. The syndrome of cerebrospinal fluid hypovolemia following lumbar puncture is reported in a girl with idiopathic intracranial hypertension. (J Child Neurol 2002;17:77-79).
White-Matter Alterations and Callosal Abnormalities in Syndromic Patients With Mental Retardation
Authors: Gabrielli Orazio , Bruni Stefano , Coppa Giovanni V. , Carloni Ines , Polonara Gabriele , Regnicolo Luana , Salvolini Simone , Salvolini Ugo
Published: March 2008

The aim of this study was to evaluate the frequency of callosal abnormalities and white matter alterations in syndromic patients. The authors report on the cerebral magnetic resonance imaging (MRI) morphologic analysis of the corpus callosum and white matter in 73 normal subjects and 61 syndromic patients. The study of the corpus callosum was carried out by MRI using different morphometric methods: measurement of the dimensions of length and thickness of genu, body, and splenium; measurement of angles obtained using the sagittal plane; and application of the proportional grid of Talairach. The evaluation of the white matter was carried out by applying a subjective grading scale. Abnormalities of the corpus callosum were found in about 50% of the syndromic subjects; in half of these cases, the abnormalities were associated with white matter alterations. In five syndromic patients (8.2%), the white matter alterations were not associated with corpus callosum abnormalities. This study shows that corpus callosum abnormalities are frequent in syndromology regardless of the syndrome type. (J Child Neurol 2002;17:164-168).
Rapid Eye Movement Sleep Behavior Disorder in Children With Autism
Authors: Thirumalai Shanti S. , Shubin Richard A. , Robinson Ricki
Published: March 2008

We performed nocturnal polysomnography on 11 children with autism who had symptoms of disrupted sleep and nocturnal awakenings. We identified rapid eye movement (REM) sleep behavior disorder in 5 of these 11 patients. Since REM sleep behavior disorder typically affects elderly males with neurodegenerative diseases, the identification of this phenomenon in autistic children could have profound implications for our understanding of the neurochemical and neurophysiologic bases of autism. Further, accurate diagnosis of REM sleep behavior disorder would enable specific treatment with clonazepam and help the family and the child consolidate sleep and improve daytime performance. (J Child Neurol 2002;17:173-178).
Is a Fast Necessary When Initiating the Ketogenic Diet?
Authors: Wirrell Elaine C. , Darwish Husam Z. , Williams-Dyjur Christine , Blackman Marlene , Lange Valerie
Published: March 2008

The purpose of this study was to determine time of onset of ketosis and efficacy when the classic ketogenic diet is initiated at full calories without a prior fast in children with epilepsy. A retrospective hospital and neurology clinic chart review was done of all 14 children commenced on the classic ketogenic diet at full calories without a prior fast between January 1, 1997, and May 31, 2001, to determine time to ketosis, time to good ketosis (urine ketones ≥80 mg/dL), and success of the ketogenic diet. Median age at diet initiation was 63 months (25th-75th percentile 47-149 months). There were 7 girls and 7 boys. Four had symptomatic generalized epilepsy, whereas the remainder had partial seizures ± secondary generalization. Twelve of 14 children suffered seizures on a daily basis prior to the ketogenic diet. Six were commenced on the diet as outpatients, whereas 8 were admitted to hospital. No patients were fasted. All admitted patients were started on a 1:1 ketogenic ratio at full calories for the first 24 hours and advanced to a 3:1 or 4:1 ratio over 3 to 4 days, while outpatients were started on a 1:1 or 2:1 ratio and similarly advanced. Thirteen of 14 patients were successfully started on the diet, with 1 developing vomiting and food refusal during the initial hospitalization but after ketosis was established. One child was lost to follow-up after initial hospital discharge. Information regarding time to ketosis was determined for all inpatients. Mean time to onset of ketosis was 33 hours (range 17 to 48) and to good ketosis, 58 hours (range 40 to 84). Five of 12 children (42%) experienced success with the ketogenic diet, and all of these had their antiepileptic medications either withdrawn (n = 3) or decreased (n = 2). The ketogenic diet can be effectively initiated without a fast in children with epilepsy. Time to ketosis and diet efficacy are similar to protocols that use a fast. (J Child Neurol 2002;17:179-182).