bluuebunny/crossref_metadata_2025 huggingface dataset:
Encephalocraniocutaneous Lipomatosis
Authors: Fishman Marvin A.
Published: June 2008

Encephalocraniocutaneous lipomatosis is a distinct clinical syndrome characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. The cutaneous lesions are confined to the head. The central nervous system lesions consist in part of cerebral hemiatrophy, porencephaly, and defective opercularization of the insula. The intracranial lesions and clinical symptomatology are progressive, and appear, in part, to have a vascular pathogenesis. This suggests that a primary defect in the formation of tissues derived from embryonic mesenchyme may be responsible for the syndrome and that tissues derived from ectoderm may be secondarily affected. ( J Child Neurol 1987;2:186-193).
Computed Tomography of the Brain in the Smith-Lemli-Opitz Syndrome
Authors: Marion Robert W. , Alvarez Luis A. , Marans Zvi S. , Lantos George , Chitayat David
Published: June 2008

Computed tomographic (CT) scans of the brain in a child with Smith-Lemli-Opitz syndrome revealed enlargement of the ventricular system, hypoplasia of the cerebellum, and abnormal thickening of the gray matter, consistent with pachygyria. These findings have been previously noted in autopsies performed on patients with this disorder. We conclude that CT scanning is a valuable tool in the evaluation of children suspected of having the Smith-Lemli-Opitz syndrome. (J Child Neurol 1987;2:198-200).
Abnormal Cerebral Cortical Neurons in a Child With Maternal PKU Syndrome
Authors: Lacey Daniel J. , Terplan Kornel
Published: June 2008

A child born to a phenylketonuric (PKU) woman not on dietary treatment was microcephalic and had congenital heart and other physical anomalies consistent with the maternal PKU syndrome. After a repeat cardiac catherization at 3 months of age, he suffered an embolic left cerebral infarct and seizures. He was found by his mother dead in bed at 4 months of age; general autopsy revealed Klebsiella sepsis and pneumonia. Neuropathologic studies of the right cerebral hemisphere showed neuronal loss of the third cortical layer; Golgi studies revealed persistently immature cortical pyramidal cell somata and dendritic spines. This is the first report of specific neuronal abnormalities in a child with the maternal PKU syndrome and may, in part, account for the clinical features of microcephaly, mental retardation, and seizures seen in affected children. (J Child Neurol 1987;2:201-204).
Ultrasound Imaging and Directed Needle Biopsy in the Diagnosis of Selective Involvement in Muscle Disease
Authors: Heckmatt J.Z. , Dubowitz V.
Published: June 2008

Four children investigated for neuromuscular disorder by routine ultrasound imaging showed selective involvement within the quadriceps femoris muscle, with involvement of the vasti and sparing of the rectus femoris. This was confirmed by concurrent needle biopsy of the two muscles. Real-time ultrasound imaging is quick, noninvasive, readily accepted by children, and has the advantage over CT scans of being practical for routine outpatient use. Needle biopsy is relatively atraumatic and enables one to select specific superficial and deep muscles for concurrent biopsy. ( J Child Neurol 1987; 2:205-213).
Brain Tumors Presenting as a Seizure Disorder in Infants
Authors: Rutledge S. Lane , Carter Snead O. , Morawetz Richard , Chandra-Sekar B.
Published: June 2008

Seizures occur in 25% to 40% of children with supratentorial tumors and are the presenting complaint in 10% to 15%. However, when divided by age, only 2% of children with seizures as the presenting complaint of brain tumors were less than 1 year of age. Three children, ranging in age from 20 days to 7 months and seen within the past 2 years, form the basis of this report. The presenting complaint in all children was seizures. Computed tomographic (CT) scan was indicated in all children because of intractability to anticonvulsant drug therapy (one patient) and focal electroencephalographic (EEG) abnormality with clinical evidence of complex partial seizure activity (two patients). CT scan showed a contrast-enhancing mass in the medial temporal lobe in all patients. At surgery, a temporal lobe tumor was found and resected in all children. Histopathologic examination revealed a ganglioglioma, a fibrillary astrocytoma, and an anaplastic astrocytoma. All children did well postoperatively and are seizure free to date. Our experience suggests that supratentorial tumors should be considered as a cause of intractable and/or focal seizures in children under 1 year of age, and that such tumors should be attacked aggressively neurosurgically. Our experience is also in agreement with that of Tadmor et al, who have suggested that with the advent of CT scanning supratentorial tumors in this age group have been found to be more common than previously realized. ( Child Neurol 1987;2:214-219)
Hypothalamo-Pituitary-Adrenal Function in Infantile Spasms: Effects of ACTH Therapy
Authors: Rao Jayashree K. , Willis John
Published: June 2008

The metyrapone test was used to study the hypothalamo-pituitary-adrenal function in ten children with infantile spasms, before and after ACTH treatment. The hypothalamo-pituitary-adrenal response was normal before ACTH treatment in almost all children. After ACTH, the responses of two children were suggestive of a diminished pituitary reserve; three were suggestive of decreased adrenal as well as decreased pituitary reserve, and one suggested either adrenal hyperplasia with normal pituitary reserve, or appropriate response to a developing medical stress. We suggest that, in children being treated with ACTH, the dosage of ACTH should be gradually tapered, AM cortisol levels should be monitored, and high-dose steroids should be included when treating medical stress. ( J Child Neurol 1987;2:220-223).
Midline Foci of Epileptiform Activity in Children and Neonates
Authors: Fischer Richard A. , Clancy Robert R.
Published: June 2008

We report the clinical and electroencephalographic (EEG) correlates of focal potentially epileptogenic discharge arising from midline sagittal vertices (Fz, Cz , Pz) in 21 children prospectively identified from among 7,051 consecutive EEGs recorded during a 27-month period. The patients range in age from neonates to 13 years. EEGs were obtained during evaluations for solitary or recurrent seizures (17/21), attention deficit disorder (2/21), psychomotor retardation (1/21), and headache (1/21). Clinical seizure types were diverse and included simple partial (5/17), complex partial (2/17), generalized tonic-clonic (4/17), mixed (2/17), and neonatal (4/17). The majority (13/21) of patients had an idetifiable etiology for their disorder; CT scans verified mass lesions in two patients. Midline epileptogenic foci were present during wakefulness in 14 of 17 older children and restricted to sleep in the others. Sleep states were indeterminate in the four neonates. Midline foci were exquisitely confined to Fz, Cz, and/or Pz in 6 of 17 older children and would have been entirely missed by a recording montage that did not include vertex electrodes. In five other children, midline foci spread preferentially to the adjacent central-parietal regions and closely resembled the appearance of benign rolandic foci in the longitudinal EEG montages, a potentially serious cause of EEG misinterpretation in view of the high incidence of neuropathology in this patient group. ( J Child Neurol 1987;2:224-228).
Review Article: Epilepsy Surgery in Children
Authors: Green Robert C. , Adler John R. , Erba Giuseppe
Published: June 2008

Neurosurgical procedures of established value in the treatment of the medically intractable epilepsies include temporal lobectomy, extratemporal cortical excision, hemispherectomy, and corpus callosotomy. The clinical decision to consider surgery in children with epilepsy requires an understanding of the natural history of pediatric seizures, the constraints of the presurgical evaluation, and the relationship between surgical outcome and tissue pathology. This article presents an overview of the indications, risks, and benefits of epilepsy surgery in the pediatric population. (J Child Neurol 2988;3:255-266).
Status Epilepticus in Children: Etiology, Clinical Features, and Outcome
Authors: Dunn David W.
Published: June 2008

Between August 1984 and September 1986, data were gathered prospectively on 114 episodes of convulsive status epilepticus, defined as seizure duration longer than 30 minutes, affecting 97 children. Status epilepticus was symptomatic in 72% (chronic 59%, acute 13%) and idiopathic or febrile in 28%. We identified precipitating factors in 63% of episodes. The most common factors were inadequate blood levels of anticonvulsants (32 of 60 episodes in children with prior seizures) and febrile illnesses, excluding meningitis or encephalitis (38 of 114 episodes). There was an elevated peripheral white blood cell count in 60%, acidosis with a pH of <7.0 in 12.5%, and cerebrospinal fluid pleocytosis not due to meningitis or encephalitis in 8 of 64 episodes. Eight children died, three with severe pre-existing brain damage, two with meningitis, and two with a poorly defined encephalopathy. Eighteen children developed a new neurologic deficit. Outcome was associated with the etiology and duration of status epilepticus, with age at the time of status a minor factor. A permanent deficit occurred in only five children with idiopathic or febrile status epilepticus. (J Child Neurol 1988;3:167-173).
Sciatic Neuropathies in Childhood: A Report of Ten Cases and Review of the Literature
Authors: Jones H. Royden , Gianturco Laurie E. , Gross Paul T. , Buchhalter Jeffrey
Published: June 2008

Primary lesions affecting the sciatic nerve are uncommon, especially in children. Isolated sciatic nerve involvement was found in ten patients during an 81/2-year period at a metropolitan children's hospital. Etiologic mechanisms included three with compression and one each with stretch injury after operation using the lithotomy position, stretch injury after closed reduction of hip dislocation, puncture wound, lymphoma, hypersensitivity vasculitis associated with hypereosinophilia, indeterminate lesion associated with transverse myelitis, and idiopathic progressive lesion with negative findings on exploration. Neonatal injuries associated with breech delivery or intragluteal injections were not causative factors in this series. Children with sciatic neuropathies have a variable prognosis depending on the etiology. Compression was the only potentially preventable pathophysiologic mechanism. (J Child Neurol 1988;3:193-199).
Cerebral Involvement in Children with Acute and Relapsing Inflammatory Polyneuropathy
Authors: Willis John , Van den Bergh Peter
Published: June 2008

We present two children with seizures and other signs of cerebral involvement at the outset of acute inflammatory demyelinating polyneuropathy, consistent with a diagnosis of encephalomyeloradiculoneuropathy. One child had a recurrence associated with mild central dysfunction and improved during therapy with corticosteroids. Both children recovered completely. Both acute and relapsing inflammatory demyelinating neuropathy may be accompanied by cerebral dysfunction, expanding the clinical spectrum of encephalomyeloradiculoneuropathy. ( Child Neurol 1988;3:200-204).
Parainflammator Leukoencephalomyelitis: Clinical and Magnetic Resonance Imaging Findings
Authors: Marks Warren A. , Bodensteiner John B. , Bobele Gary B. , Hamza Mohsen , Wilson Don A.
Published: June 2008

Parainflammatory leukoencephalomyelitis is a broad term used to include the spectrum of disorders that affect the central nervous system following infection, immunization, or other noxious stimuli. There is a wide range of clinical and pathologic severity, ranging from acute cerebellar ataxia to acute hemorrhagic leukoencephalopathy. With the improved survival of these patients, magnetic resonance imaging provides a window to the pathologic process, which can aid in the long-term management of these patients. Although lesions of the brainstem and spinal cord correlate well to clinical symptoms, multiple cortical lesions may be present without specific localizing signs. The distribution of magnetic resonance lesions is different from that commonly seen in multiple sclerosis. In some cases, prolonged immunosuppression may be required to prevent recrudescence of the inflammatory response. (J Child Neurol 1988;3:205-213).
Review Article: The Use of Somatosensory Evoked Potentials in Infants and Children
Authors: Gilmore Robin
Published: June 2008

Somatosensory evoked potentials (SSEPs) are a useful, reliable means of assessing function of the somatosensory system. Complex maturational changes of the CNS such as synaptogenesis and myelination, as well as body growth, complicate interpretation of SSEPs. An understanding of these factors enhances clinical interpretation in infants and children. (J Child Neurol 1989 ;4 :3-19).
Intraventricular Interferon Treatment for Subacute Sclerosing Panencephalitis
Authors: Steiner I. , Wirguin I. , Morag A. , Abramsky O. , Dyken Paul R.
Published: June 2008

Three children with subacute sclerosing panencephalitis (SSPE) were treated with alpha-interferon. Medication was administered via an Ommaya reservoir into the cerebral ventricular system over a period of 13 to 24 months in increasing amounts up to 1.0 X 106 IU twice weekly. Mild clinical improvement in two children and a stable course in the third were associated with improvement of electroencephalographic recordings in all patients and a decrease in antimeasles antibodies in the cerebrospinal fluid of two. The small number of patients does not enable us to determine whether this course was the natural history of SSPE in our patients or whether it was induced by treatment, yet it seems that intraventricular interferon treatment can modify the course of SSPE. ( J Child Neurol 1989;4:20-23).
Immune Function in Patients Treated With Phenytoin
Authors: Burks A. Wesley , Charlton Ronald , Casey Peggy , Poindexter Ann , Steele Russell
Published: June 2008

Multiple immunologic side effects have been ascribed to phenytoin. Numerous reports in the literature discuss the possible cellular and humoral abnormalities that appear to be present in patients given phenytoin. The most consistent finding is a reduction in serum IgA found in up to 20% of patients. To resolve some of the conflicting studies on cellular immune status, 191 patients taking phenytoin were evaluated initially with an serum IgA determination, and then further immune studies were done on the 11% with IgA values lower than two standard deviations below the mean. Data collected included total lymphocyte counts, lymphocyte population studies, and responses to in vitro mitogen stimulation. Only 2 of 191 patients had serum IgA values less than 5 mg/dL, which is an incidence not significantly different than that in the population at large. The patients with decreased serum IgA values did not have an increased incidence of autoimmune phenomena, allergic disorders, gastrointestinal manifestations, or recurrent upper respiratory tract infections. Their cellular immune status showed no significant variations from control values. Thus it appears that routine monitoring of patients on phenytoin with serum IgA determinations is of limited value, and the immunologic side effects of phenytoin are not expressed as a cellular abnormality. ( Child Neurol 1989;4:25-29).
Megalencephaly in Sudden Infant Death Syndrome
Authors: Shaw Cheng-Mei , Siebert Joseph R. , Haas Joel E. , Alvord Ellsworth C.
Published: June 2008

The fresh brain weights of 79 infants with sudden infant death syndrome (SIDS) were tabulated and compared with expected "normal" brain weights. The series included 47 males and 32 females. Their ages ranged from 8 days to 16 months with a median of 2.5 months. The weights of all these brains were above the 50th percentile for age, and 64 of 79 brains (81%) were above the 95th percentile, including 15 (19%) at or above the 99.9th percentile. The ratio of the brain stem plus cerebellum weight to the whole brain weight of 93 formalin-fixed brains from victims of SIDS showed that the cerebrum was disproportionately heavier in most cases. No gross or microscopic evidence of cerebral edema was noted to account for the heavier weight. The significance of these findings is debatable. The increase in brain weight was less obvious in infants younger than 1 month of age but accelerated after that age. The brain weights of infants with SIDS may represent the really "normal" weights, since these patients were not known to be ill prior to death. If the brain weights of SIDS infants are really heavier than "normal," the possibility must be considered that disproportionately rapid growth of the brain during early infancy may be detrimental to the neural control of the cardiorespiratory system. (J Child Neurol 1989;4:39-42).
Benign Nocturnal Childhood Occipital Epilepsy: A New Syndrome with Nocturnal Seizures, Tonic Deviation of the Eyes, and Vomiting
Authors: Panayiotopoulos Chrysostomos P. , Aicardi Jean
Published: June 2008

An epileptic syndrome of benign nocturnal childhood occipital epilepsy with excellent prognosis is described. The syndrome is characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. There may be marching to involve the head and limbs, ending with a generalized tonic-clonic seizure. Consciousness is usually, but not invariably, disturbed. Infrequent daytime fits may develop one to two years after remission of the nocturnal seizures. Age of onset is usually from 3 to 5 years. Both sexes are involved. There is no family history of epilepsy or migraine. No definite causative factor was detected. The frequency of the seizures is very low with two children having only solitary ones. The interictal electroencephalographic features consist of repetitive occipital spike and slow wave complexes that are induced by closed eyes and darkness and are inhibited by open eyes and fixation with visual cues. It is proposed that this is a new idiopathic age-related-onset syndrome of the localization-related epilepsies. (J Child Neurol 1989;4:43-48).
Eclampsia: A Pediatric Neurologic Problem
Authors: Elliott Debra , Haller Jerome S.
Published: June 2008

Eclampsia is a syndrome of hypertension, proteinuria, edema, and neurologic manifestations such as seizures, focal deficits, or blindness occurring during pregnancy, mainly in primigravidas. Although it is not seen often in the pediatric population, it occurs with greater frequency among pregnant patients under the age of 15 than in the general population. Serious sequelae can be prevented with prompt and adequate treatment, including termination of pregnancy. (J Child Neurol 1989;4:55-60).
Review Article: The Neuronal Ceroid Lipofuscinoses
Authors: Dyken Paul Richard
Published: June 2008

The neuronal ceroid lipofuscinoses are clinical disorders associated with the accumulation of autofluorescent waxy pigments within cells of several different tissues. Such syndromes always have neurological manifestations. Variations in clinical course, genetics, pathogenesis, and possibly treatment occur in each of the several forms listed under this category. Ten subtypes have now been recognized: (1) chronic, juvenile (Batten type); (2) acute, late infantile (Bielschowsky type); (3) subacute-chronic, adult (Kufs type); (4) acute, infantile (Santavuori-Haltia type); (5) congenital (Norman-Wood type); (6) acute, adult (Zeman-Dyken type); (7) acute-subacute childhood (Bielschowsky variant); (8) chronic, childhood with pervasiveness (Edathodu-Dyken type); (9) chronic, infantile with autism (Dyken type); and (10) chronic, juvenile with ataxia and spasticity (Dyken type). By far the most common of these are the first four disorders listed. It is proposed that this present classification of neuronal ceroid lipofuscinosis is more comprehensive than previous ones and fails to support the hypothesis that this disorder represents a unitary disease process, rather than different diseases with similar characteristics. At present, each of the neuronal ceroid lipofuscinosis types are of unknown etiology. (J Child Neurol 1989;4:165-174).
Clinical Significance of Focal Periodic Discharges in Neonates
Authors: Scher Mark S. , Beggarly Marquita
Published: June 2008

Of 1,114 EEG recordings obtained for 592 neonates, focal periodic discharges were noted on 57 (5%) of recordings for 34 neonates (26 preterm and eight term). Periodic lateralized epileptiform discharges (PLEDs) were noted on four of these recordings. Sixteeen patients (47%) with focal periodic discharges also had electrographic seizures on the same or a subsequent record. Stroke was the most common brain lesion (53%) in this neonatal population. Preterm neonates had discharges that were less than 60 seconds in duration and located in the parasagittal regions, while discharges in term neonates were longer in duration and were located in the temporal regions. Of the 34 neonates, 15 (44%) died, and 58% (11/19) of the survivors were abnormal with respect to neurological development. Focal periodic discharges in neonates, including PLEDs, have the same clinical significance as PLEDs recorded on EEGs in older children and adults. (J Child Neurol 1989;4:175-185).
Fatal Infantile Muscle Phosphorylase Deficiency
Authors: Milstein Jerrold M. , Herron Thomas M. , Haas Joel E.
Published: June 2008

A premature female infant born of a consanguineous union exhibited joint contractures and signs and symptoms of perinatal asphyxia. A muscle biopsy examined by light microscopic, histochemical, and electron microscopic techniques exhibited changes of muscle phosphorylase deficiency and glycogenosis, identical to those of McArdle's disease. Postmortem ultrastructural examination of liver and heart did not reveal lysosomal storage. This case and one previously reported example of fatal infantile phosphorylase deficiency suggest that the clinical spectrum of McArdle's disease may be broader than previously recognized. (J Child Neurol 1989;4:186-188).
Congenital Myopathy With Progressive External Ophthalmoplegia
Authors: Riggs Jack E. , Schochet Sydney S. , Goldfarb Glenn R. , Price Rebecca A.
Published: June 2008

Progressive external ophthalmoplegia is associated with an increasingly heterogeneous group of neuromuscular disorders. We describe a 16-year-old girl with delayed motor milestones and onset of progressive external ophthalmoplegia at age 3 years and proximal muscle weakness at age 10 years. Muscle biopsy specimen demonstrated type I myofiber predominance and type II myofiber atrophy. Although the pathological features of this congenital myopathy are quite nonspecific, the case further illustrates the pathogenetic heterogeneity of the progressive external opthalmoplegia phenotype. (J Child Neurol 1989;4:194-196).
Deficiency of Carnitine Palmitoyltransferase I
Authors: Bonnefont J.P. , Haas Richard , Wolff Jon , Thuy L.P. , Buchta Richard , Carroll J.E. , Saudubray Jean-Marie , Demaugre F. , Nyhan William L.
Published: June 2008

Defective activity of carnitine palmitoyltransferase I was demonstrated in fibroblasts derived from a patient with hypoketotic hypoglycemia. The level of activity observed was approximately 10% of the control mean. Oxidation of palmitate by intact fibroblasts was reduced to 5% of control values. The patient presented at age 14 months with seizures and was found to have marked hypoglycemia and no ketones in the urine. In response to fasting, she developed hypoglycemia, but the curves for acetoacetate and 3-hydroxybutyrate were flat. Administration of medium-chain triglycerides relieved the hypoglycemia and generated a brisk ketogenesis. (J Child Neurol 1989;4:197-202).
Histocompatibility Determinants in Childhood Postinfectious Encephalomyelitis
Authors: Woody Robert C. , Steele Russell W. , Charlton Ronald K. , Smith Valaria
Published: June 2008

Postinfectious encephalomyelitis and multiple sclerosis have clinical, immunologic, and neuroradiographic similarities. We studied HLA determinants in six white children consecutively diagnosed with postinfectious encephalomyelitis. Each of the children had HLA determinants which have been associated with multiple sclerosis. Relative risk (RR) calculations demonstrated that these antigens and genotypes occurred significantly more often in patients with postinfectious encephalomyelitis than in the control population (A3, RR 6.14; B7, RR 6.14; DR2, RR 4.51; A3B7, RR 9.36; A3DR2, RR 5.83; B7DR2, RR 6.13; A3B7DR2, RR 10.90). These data suggest that children with postinfectious encephalomyelitis are genetically predisposed to this demyelinating disease. Although the same HLA determinants were found in these patients as in those with multiple sclerosis, studies of a larger number of postinfectious encephalomyelitis patients will be needed before it can be concluded that the two diseases share a common genetic propensity. (J Child Neurol 1989;4:203-206).
Reversible Treatment-Related Leukoencephalopathy
Authors: Gay Charles T. , Bodensteiner J.B. , Nitschke Ruprecht , Sexauer Charles , Wilson Don
Published: June 2008

We present two children with acute lymphocytic leukemia who developed leukoencephalopathy following administration of a combination of intravenous ara=C and methotrexate during the consolidation phase of chemotherapy. Cranial magnetic resonance imaging showed widespread, abnormally high signal intensity in the deep white matter in both patients, though one patient had normal cranial computed tomographic scan. Treatment was modified, symptoms resolved in 1 to 2 weeks, and the white-matter changes resolved over 6 to 12 months. Intravenous cytarabine and methotrexate appear to act synergistically to enhance the potential for central nervous system toxicity. Awareness of this potentially serious complication of chemotherapy can facilitate timely recognition of leukoencephalopathy with the use of magnetic resonance imaging. (J Child Neurol 1989;4:207-212).
Childhood Onset Inclusion Body Myositis Mimicking Limb-Girdle Muscular Dystrophy
Authors: Riggs Jack E. , Schochet Sydney S. , Gutmann Ludwig , Lerfald Sidney C.
Published: June 2008

Inclusion body myositis was initially recognized in patients with "steroid-resistant polymyositis" and subsequently in patients with other immune-mediated disorders. The finding of inclusion body myositis in a patient diagnosed for 30 years as having limb-girdle muscular dystrophy suggests yet another patient pool that may harbor this entity. ( J Child Neurol 1989;4:283-285).
Neurosonographic Features of Aicardi's Syndrome
Authors: Roland Elke H. , Flodmark Olof , Hill Alan
Published: June 2008

Aicardi's syndrome occurs in females and is associated with profound mental retardation, agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. We report a distinctive combination of abnormalities which was observed on real-time cranial ultrasound scans of two infants with Aicardi's syndrome. These abnormalities include bilateral prominent cysts in the choroid plexus in conjunction with agenesis of the corpus callosum. Recognition of these radiologic findings may suggest the diagnosis of Aicardi's syndrome in early infancy even prior to the onset of seizures or visualization of chorioretinal lacunae. (J Child Neurol 1989;4:307-310).
An Extended Phenotype of an Early-Onset Inherited Nonprogressive Cerebellar Ataxia Syndrome
Authors: Kornberg Andrew J. , Shield Lloyd K.
Published: June 2008

A father and son with presumed dominantly inherited, nonprogressive, early-onset cerebellar ataxia are reported. The clinical features are similar to those in other reports of this rare disorder, but magnetic resonance imaging revealed generalized atrophy of the cerebellum and not localized vermal atrophy as previously noted. This family illustrates either an extended phenotype of the previously reported disorder or possibly an unique type of autosomal dominant cerebellar ataxia. ( J Child Neurol 1991;6:20-23).
Modified Encephaloduroarteriosynangiosis as a Surgical Treatment of Childhood Moyamoya Disease: Report of Five Cases
Authors: Rooney Cynthia M. , Kaye Edward M. , Scott R. Michael , Klucznik Richard P. , Rosman N. Paul
Published: June 2008

Moyamoya disease is an idiopathic disorder characterized by progressive occlusion of the internal carotid and anterior and middle cerebral arteries, with formation of an extensive abnormal collateral circulation at the base of the brain. Many neurosurgical procedures have been designed to bypass these occluded vessels. The results of one of these procedures, modified encephaloduroarteriosynangiosis was reviewed in five children followed for 3½ to 19½ years. Modified encephaloduroarteriosynangiosis performed unilaterally in one and bilaterally in four of the children, appeared to halt neurologic deterioration, despite angiographic progression, in four of the five children. ( J Child Neurol 1991;6:24-31).
Neuropathologic Findings in Reye Syndrome
Authors: Blisard Karen S. , Davis Larry E.
Published: June 2008

Brain tissue from three patients with a clinical diagnosis of Reye syndrome was compared with tissue from three control patients. All Reye syndrome patients demonstrated cytotoxic cerebral edema, with swelling of astrocyte foot processes, which was not seen in controls. Myelin sheath splitting was seen both in controls and patients, but myelin blebs were uncommon in both. Mitochondrial changes also were not seen. Although cerebral edema is a nonspecific finding, it appears to be characteristic of Reye syndrome. (J Child Neurol 1991;6:41-44).
Academic Underachievement in Children with Epilepsy
Authors: Mitchell Wendy G. , Chavez John M. , Lee Hang , Guzman Bianca L.
Published: June 2008

Academic achievement was studied in 78 children with epilepsy, ages 5 to 13 years, to determine how seizures, treatment of seizures, and sociocultural factors influence academic achievement. Cognitive abilities were assessed with either the McCarthy Scales of Children's Abilities or the Wechsler Intelligence Scale for Children-Revised. Achievement was measured with the Peabody Individual Achievement Tests. Achievement scores were corrected for cognitive ability (IQ), and underachievement was defined as achievement score 1/2 standard deviation or more below IQ. Information regarding seizure history (severity, duration) and treatment with anticonvulsant drugs was obtained. In addition, the family was extensively interviewed regarding the child's environment, behavior, and demographic background. The Home Observation for Measurement of the Environment (HOME) scale was completed on a home visit.

Underachievement was frequent, ranging from 16% (Reading Recognition) to 50% (General Knowledge), but there was no relationship between severity or duration of seizure disorder or total exposure to anticonvulsant medications and achievement. Major determinants of achievement included subscales of the HOME scale, age (older children more likely to be underachieving), and parental education. An equal proportion of newly diagnosed and/or untreated subjects were underachieving compared to those with longstanding epilepsy and anticonvulsant drug treatment. (J Child Neurol 1991;6:65-72).
Sleep-Disordered Breathing in Möbius Syndrome
Authors: Gilmore Robin L. , Falace Peggy , Kanga Jamshed , Baumann Robert
Published: June 2008

Möbius syndrome is a complex neurologic disorder characterized by congenital bilateral facial paralysis associated with lateral gaze paralysis. The syndrome has variable manifestations and several variants, some with somatic stigmata. In 1990, Möbius syndrome is conceptualized as a spectrum of clinical caudal brain-stem signs. Some deficits are manifested by laryngeal paralysis and aspiration. Sleep-disordered breathing syndromes have not been previously reported in association with Möbius syndrome. We report two children with Möbius syndrome and sleep-disordered breathing. Based on known pathologic findings and clinical manifestations, we believe that sleep-disordered breathing may be a common complication of Möbius syndrome and should be sought, since potential outcomes of such complications include serious morbidity. ( J Child Neurol 1991;6:73-77).
Clinical Experience on Headache in Children: Analysis of 92 Cases
Authors: Aysun Sabiha , Yetük Mürüvet
Published: June 2008

We analyzed, retrospectively, 92 patients with headache to determine the changes in the order of frequency of causes with the development of neuroimaging studies and its efficacy in the investigation of patients with headache. The type of headache was redefined according to the International Headache Society (IHS) diagnostic criteria. Migraine was the most frequent cause of headache and the rest in decreasing order were: tension-type headache, sinusitis, and epilepsy. The percentage of the findings relevant to headache in computed tomographic (CT) scans, magnetic resonance images (MRIs), Waters' projection (radiographs), and electroencephalograms (EEGs) were respectively 4.2%, 33.3%, 16%, and 25%. Neuroimaging studies are not necessary in the routine evaluation of patients with headache unless there is an abnormality in the findings. When it is needed, MRI, which has higher yield, can take the place of CT scanning. The most important point is taking a proper history of headache and making a thorough physical and neurologic examination of the patient. (J Child Neurol 1998;13:202-210).
Somatosensory Evoked Potentials With a Unilateral Migration Disorder of the Cerebrum
Authors: Ozawa Hiroshi , Inagaki Masumi , Aikoh Hiroshi , Hanaoka Shigeru , Sugai Kenji , Hashimoto Toshiaki , Kaga Makiko
Published: June 2008

The somatosensory evoked potentials in two children with a unilateral migration disorder (pachygyria) of the cerebrum, which was detected by MRI, were examined in order to evaluate the function of the malformed sensory cortex. A 5-year-old girl had slight left hemiparesis, seizures, and mental retardation, and a 4-month-old boy had left hemiparesis. Neither patient showed distinct sensory disturbance. Short latency somatosensory evoked potentials and somatosensory evoked potentials recordings demonstrated that the early cortical component, N20, was absent and a positive wave appeared on paretic left-hand stimulation. On nonparetic right-hand stimulation, the primary evoked response (N20-P30) of the left hemisphere, which originates in Broadmann area 3b, was almost normal. Multichannel recordings on the scalp of one patient revealed that a positive wave without polarity inversion appeared posterior to the right central sulcus on median nerve stimulation on the paretic side. The radial dipole in the sensory cortex (area 1 or area 3a) or motor cortex (area 4) could have formed the positive/negative biphasic wave in the relatively wide centroparietal area in the present patients. In the case of unilateral cortical dysplasia, the malformed cortex with subnormal function of sensation might induce the change in the early component of somatosensory evoked potentials. (J Child Neurol 1998;13:211-215).
"Extensor Toe Sign" by Various Methods in Spastic Children With Cerebral Palsy
Authors: Ghosh Debabrata , Pradhan Sunil
Published: June 2008

Upper motor neuron lesion in adults is usually associated with spasticity and "extensor toe sign" on plantar stimulation (extensor plantar response). There are various methods of eliciting this sign including the classic method by Babinski. Other methods produce this response when the area of reflexogenic zone is increased due to upper motor neuron lesion. There are varying reports of Babinski positivity in spastic cerebral palsy. This study was undertaken to assess the sensitivity of different methods of eliciting "extensor toe sign." An attempt has also been made to correlate the severity of spasticity with the combined "extensor toe sign" positivity by various methods and with the increase in reflexogenic zone. Eighty-one children with spastic cerebral palsy were examined. Twelve had hemiplegia; therefore, a total of 150 limbs were tested. "Extensor toe sign" was elicited by 12 different methods in each patient. The sensitivity of each method was calculated and compared with each other one. The assessment of spasticity was done using the Ashworth Tone Scale. The severity of spasticity was correlated with "extensor toe sign" positivity using various methods. Classic Babinski reflex was positive in 75% of cases, whereas Gonda-Allen sign was positive in 90% of cases followed by Allen-Cleckley (82%), Chaddock (74%), and Cornell (54%). All other signs had sensitivity of less than 30%. There was no increase in sensitivity after combining them. There was significant negative correlation between the spasticity and the combined "extensor toe sign" positivity (by all the methods). This study, therefore, suggests that the majority of patients with spastic cerebral palsy have positive "extensor toe sign." The Gonda-Allen method is more sensitive than the classic Babinski method. A positive "extensor toe sign" is negatively correlated to the degree of spasticity. (J Child Neurol 1998;13:216-220).
Generalized Tonic and Tonic-Clonic Seizures of Childhood
Authors: Kramer Uri , Nevo Yoram , Yael Leitner , Harel Shaul
Published: June 2008

In a retrospective analysis of all our patients with seizure onset prior to age 16 years, 25 patients with primary generalized tonic (n = 10) or tonic-clonic (n = 15) seizures were identified. These patients constituted 5.7% of the total seizure patient population in our institute between the ages of 1 month and 16 years. The natural history of generalized tonic-clonic seizures is known to be benign; however, that of isolated primary generalized tonic seizures is not clear. Therefore, an attempt was made to characterize the patients suffering from primary generalized tonic seizures and determine their outcome. Analysis of our patient population shows that both seizure types are characterized by early onset of generalized seizures that appear in normally developed children with a normal electroencephalographic background. The children usually respond quickly to antiepileptic drugs. A long-term follow-up (mean period of 7.6 years) was possible in 84% of the patients, and showed that 95% of them were seizure free at the end of the follow-up period. There was no significant difference between the two groups in regard to age of onset, family history, and seizures at follow-up. In conclusion, the natural history of patients with generalized tonic seizures is similar to the benign course of those with generalized tonic-clonic seizures. (J Child Neurol 1998;13:221-223).
A Description of the Comprehensive Test of Nonverbal Intelligence
Authors: Wiederholt J. Lee , Rees Forest J.
Published: June 2008

The Comprehensive Test of Nonverbal Intelligence, or CTONI, has become an essential compliment to the traditional tests of intelligence, such as the WISC-III, DTLA-3, and the Binet. The CTONI provides examiners with a measure of nonverbal reasoning that requires no spoken language or complex motor skills. The CTONI has been proven to be unbiased with regard to gender, minority, or disabling condition. Finally, it is possible to estimate the intelligence of people without the contamination of social, ethnic, or disability bias. (J Child Neurol 1998; 13:224-228).
Neonatal Encephalopathy in Two Boys in Families With Recurrent Rett Syndrome
Authors: Schanen N. Carolyn , Kurczynski Thaddeus W. , Brunelle David , Woodcock Maureen M. , Dure Leon S. , Percy Alan K.
Published: June 2008

Rett syndrome (RTT) has been described in its classic form only in females. Although the majority of cases are sporadic, familial cases give valuable insight into the genetic basis and phenotypic variability of the disorder. The exclusive occurrence of classic Rett syndrome in females led to the hypothesis that the Rett syndrome locus is likely to be X-linked and mutations are lethal in hemizygous males. We identified two boys in families with recurrent Rett syndrome who had encephalopathies with neonatal onset and who may represent the phenotype of males harboring Rett syndrome muta tions. The difference in severity of disease in these males and their female relatives supports the location of Rett syndrome locus on the X-chromosome. (J Child Neurol 1998;13:229-231).
Recombinant Human Bone Morphogenetic Protein-2 as an Osteoinductive Biomaterial and a Biodegradable Carrier in a Rabbit Ulnar Defect Model
Authors: Kokubo Satoshi , Nozaki Kazutoshi , Fukushima Shinji , Takahashi Koichiro , Miyata Keiji , Fujimoto Ryuhei , Yokota Shoji
Published: June 2008

We investigated early local changes induced by recombinant human bone morphogenetic protein (rhBMP)-2 and a novel carrier, poly[L-lactide-co-glycolide] copolymer-coated gelatin sponge (PGS). A 1.5 cm segmental bone defect was created in the diaphysis of the right ulna of male Japanese white rabbit. Defects received PGS with or without rhBMP-2 (0, 0.4, or 1 mg/cm3) and were harvested at 3, 7, 14, 21, or 28 days post implantation for histological examination. Immuno-staining for vascular endothelial growth factor (VEGF) was also performed. Spindle-shaped cells were observed in the rhBMP-2-treated groups 3 and 7 days after implantation. Bone regeneration was detected after 14 days in the rhBMP-2-treated groups and the bone area increased with time and dose. Expression of VEGF was observed in all groups at 3 days and was maintained by 14 days only in the defects treated with rhBMP-2 at a dose of 1 mg. These results indicate that rhBMP-2 exert its osteo-inductive activities via the promotion of osteogenic cell mobilization, and possibly via angiogenesis based on VEGF induction. Foreign-body reactions to the implanted PGS were similar to those observed when either poly[L-lactide-co-glycolide] copolymer or gelatin was individually implanted. These results indicate that the PGS is a useful and safe carrier for rhBMP-2.
Chitosan Beads as Templates for Layer-by-Layer Assembly and their Application in the Sustained Release of Bioactive Agents
Authors: Grech Jessica M.R. , Mano Joao F. , Reis Rui L.
Published: June 2008

Uncoated chitosan beads and chitosan beads coated with hyaluronic acid/chitosan (HA/Ch) multilayers, were used to investigate the controlled release of gentamicin sulphate (GS). Greater encapsulation efficiency was observed for the layer—by-layer multilayer coated beads. The in vitro drug release was in a slower sustained manner compared with noncoated chitosan beads. The differences in in vitro drug release results may be explained by the barrier effect of the coating that impedes diffusion of GS and supporting complementary water uptake. These findings indicated that a slower sustained release of gentamicin can be obtained using multilayer coatings of HA/Ch on chitosan beads and that this process could be used as a drug delivery system. In addition, agglomerates of these bead could provide a porous support in tissue engineering applications.
On the Date and Contents of a Portuguese Medieval Technical Book on Illumination
Authors: Cruz António João , Afonso Luís Urbano
Published: June 2008

The book this article discusses is a late medieval Portuguese technical text on illumination written with Hebraic characters. It belongs to a miscellaneous manuscript at Parma's Biblioteca Palatina. Discovered in 1803, it was attributed to Abraham ben Judah Ibn Hayyim and dated to 1262. Soon some authors assigned it to the fifteenth century. The paper's water-marks, recently observed, confirmed the fifteenth century date. Yet, the possibility that this text could be a copy of an older original remains. However, the discussion of the historical context and the content also suggests that the original dates more probably from the fifteenth than the thirteenth century. In terms of structure and content this text should not be considered a treatise but a heterogeneous compilation which, besides the Hebraic marks, presents significantly alchemic, Castilian and Arabic influences.
Agricultural Technology in Kashmir (A.D. 1600 to 1900)
Authors: Hangloo R.L.
Published: June 2008

This article seeks to locate and analyse the ecological and socio-political conditions specific to hill agriculture within Kashmir in a medieval context. It draws upon a range of sources—texts, travel accounts and personal observation, as also interaction with farmers—to put the story together.
Market Efficiency in Emerging Stock Market
Authors: Mobarek Asma , Mollah A. Sabur , Bhuyan Rafiqul
Published: June 2008

This study seeks evidence on whether the return series on Bangladesh's Dhaka Stock Exchange (DSE) is independent and follows the random walk model. The study focuses on assessing if the DSE deviates from idealised efficiency. The sample primarily includes all the listed companies on the DSE daily price index over the period 1988 to 2000. The results of both non-parametric (Kolmogrov—Smirnov: normality test and run test) and parametric test (Auto-correlation test, Auto-regressive model, ARIMA model) provide evidence that the security returns do not follow the random walk model and the significant auto-correlation coefficient at different lags reject the null hypothesis of weak-form efficiency. The results are consistent with observations in different sub-samples without outlier and for individual securities. This anomaly with the efficient market hypothesis supports the thought that the market does not respond to new information instantaneously. This may be due to a delay in dissemination to new price sensitive information or biases (under or over reaction) in the response of market participants to such information. It may also be for the momentum effect related to herding in particular ‘positive feed back trading’ or ‘trend following’ the trading strategy by the average investors.
A Survey of Plethysmographic Assessment in North America
Authors: Howes Richard J.
Published: June 2008

A survey of North American plethysmographic assessment centers was undertaken to examine the extent to which variability exists among them. Results clearly revealed a field in which there is abundant inconsistency in both plethysmographic assessment procedures and data interpretation. A critical need for standardization was identified if plethysmographic assessment is to develop further its potential to assist in the clinical assessment and treatment of sex offenders.
Cognitive Distortions and Affective Deficits in Sex Offenders: A Cognitive Deconstructionist Interpretation1
Authors: Ward Tony , Hudson Stephen M. , Marshall William L.
Published: June 2008

Sexually aggressive behavior is often facilitated and justified by distorted thinking and affective deficits. However, there is no clear conceptual model that accounts for the mechanisms which generate these phenomena and informs treatment goals. Sex offenders frequently demonstrate a number of underexplained features such as denial or minimization of offending, victim blaming, passivity, covert planning, dependence on immediate consequences, empathy and other emotional regulation deficits, intimacy and other social competency deficits, and, finally, alcohol and other drug problems. These features are usually seen as offense precursors. We argue that Baumeister's construct of cognitive deconstruction, the process by which people attempt to reduce the negative implications of self-awareness, provides both a middle-level theoretical explanatory framework that integrates these puzzles in a parsimonious way and a mechanism that suggests that these features may be consequences of, as well as precursors to, an offense. Suggestions for research and clinical practice are offered.