Headache in children's drawings.

Headache is a common health problem in childhood. Children's drawings are helpful in the diagnosis of headache type. Children, especially younger ones, communicate better through pictures than verbally. The aim of the present study is to evaluate the usefulness of drawings of the child's headache in the diagnostic process carried out by a pediatrician and a pediatric neurologist. At the beginning of a visit in a neurological clinic, or on the first day of hospitalization, the child was asked, "Please draw your headache," or "How do you feel your headache?" without any additional explanations or suggestions. Clinical diagnosis of headache type was made on the basis of the standard diagnostic evaluation. For the purpose of this study, children's headaches were categorized as migraine, tension-type headache, or "the others." One hundred twenty-four drawings of children with headaches were analyzed by 8 pediatricians and 8 pediatric neurologists. The analysts were unaware of the clinical history, age, sex, and diagnosis of the patients. The clinical diagnosis was considered the "gold standard" to which the headache drawing diagnosis was compared. There were 68 girls 5-18 years of age and 56 boys 7-18 years of age. Of the 124 children, 40 were clinically diagnosed with migraine (32.2%), 47 with tension-type headache (37.9%), and 37 (29.8%) as the others. Children with migraine most frequently draw sharp elements. Children with tension-type headache mainly drew compression elements and pressing elements. In the group of "the other" headaches, 21 children were diagnosed with somatoform disorders. The most frequent element in this group's drawings was a whirl in the head. Colors used most frequently were black and red, which signify severe pain. There was no difference in sensitivity of diagnoses between neurologists and pediatricians. Because the evaluation of drawings by children with headaches done both by pediatricians and pediatric neurologists was correct for approximately half of the children, the authors decided to prepare a set of test pictures, including characteristic presentations of pain. Preparing a ready set of test drawings may facilitate differentiation for the inexperienced doctors and encourage those children who refuse to draw.

A novel PANK2 gene mutation: clinical and molecular characteristics of patients short communication.

Pantothenate kinase-associated neurodegeneration (PKAN) is a progressive neurodegenerative disorder with autosomal recessive inheritance. The major symptoms of PKAN include the onset before the age of 20 years, progressive pyramidal and extrapyramidal signs, retinitis pigmentosa, optic atrophy, dementia, and iron depositions in the globus pallidus. The authors present 3 patients with proven molecular diagnosis of PKAN, in whom 2 novel mutations of PANK2 gene have been identified.

Lipid raft disease? A new severe congenital myopathy.

We report a 5-year-old girl with a unique neuromuscular disorder manifested by early onset of the disease, delayed motor development, joint contractures, dysmorphy, cobbler's chest, generalized muscle hypoplasia and weakness. Morphological examination revealed muscle cell immaturity and the appearance of multilamellar myelin-like structures within and outside the sarcolemma. Overexpression of aberrant lipids on the surface of affected muscle cells may suggest some failure in lipid raft formation.

Osteoprotegerin--does it play a protective role in the pathogenesis of bone loss in obese perimenopausal women?

INTRODUCTION: Assessment of serum osteoprotegerin (OPG) concentrations in obese patients in comparison to healthy controls and evaluation of a possible correlation between OPG and other markers of bone turnover or calcitropic hormones. MATERIAL AND METHODS: 50 obese perimenopausal women without concomitant diseases (BMI 36.7 +/- 4.1 kg/m(2), mean age 50.4 +/- 4.9 yrs). The control group consisted of 19 healthy women (BMI 24.2 +/- 2.1 kg/m(2); mean age 53.8 +/- 5.1 yrs). In all patients serum concentration of OPG, C telopeptide of type I collagen containing the crosslinking site (CTX), osteocalcin, parathormone (PTH) and vitamin D (25-OH-D(3)) was assessed. Dual energy x-ray absorptiometry (the DXA method) of the lumbar spine and femoral neck was performed using a Lunar DPXL to measure bone marrow density (BMD). RESULTS: In obese perimenopausal women serum OPG, osteocalcin and 25-OH-D(3) levels were significantly lower, and the serum PTH level was significantly higher in comparison to healthy controls. A significantly positive correlation was found between serum OPG level and age in both obese and control subjects. CONCLUSION: The serum OPG level in obese perimenopausal women is significantly lower in comparison to healthy controls and does not correlate significantly with biochemical markers of bone turnover, calcitropic hormones and BMD. It probably cannot play a protective role in the pathogenesis of bone loss in obese perimenopausal women.

Huntington disease in a 9-year-old boy: clinical course and neuropathologic examination.

Huntington disease is a dominantly inherited, neurodegenerative disorder, usually with onset in the fourth to fifth decade of life but in a small proportion of patients before the age of 20 years. The early-onset form, juvenile Huntington disease, is clinically different from that of more common adult-onset forms and includes cognitive decline, parkinsonism, myoclonus, and seizures. We report a case of a boy with juvenile Huntington disease with a very early age at disease onset (3 years). The suspected clinical diagnosis was confirmed by DNA analysis, which revealed (CAG)(n) expansion into the range characteristic of juvenile Huntington disease (95 repeats). The clinical course of the disease was typical for the juvenile form of Huntington disease, but the diagnosis was not so obvious because there was no history of any neurodegenerative disorder in the family. The child died at the age of 11 years. The detailed neuropathologic investigations performed postmortem showed the characteristic features of Huntington disease. As the patient's de novo mutation was very unlikely to occur, genetic counseling and the possibility of predictive testing were proposed to the family. Indirect molecular data indicate the familial character of the disease, with strong anticipation of transmission.

[The pharmacological treatment of abulic posttraumatic state in children-preliminary report]. / Leczenie farmakologiczne pourazowego stanu abulicznego u dzieci--doniesienie wstepne.

UNLABELLED: The aim of the study was the evaluation of clinical condition of the children treated by Gliatilin and Dexamin for posttraumatic abulic state. MATERIAL AND METHOD: The study included 12 children (8 boys, 4 girls) at the age range between 7-16 years (mean age 7.8 years). The evaluation of clinical condition was performed on admission to the Department, and then at 3rd, 6th and 12th month after head injury. The authors analyzed the kind of injury (posttraumatic changes in neuroimaging) and evolution of patients' clinical condition in the follow-up. RESULTS: The most commonly observed reasons of trauma were motor vehicle accidents. The kind of pathology found on the base of neuroimaging did not affect the results of treatment. The patients were treated by Gliatilin, Dexamin or both. In spite of this treatment all children were rehabilitated and their hearing, sight and speech organ were stimulated. Six months after injury only one patient still presented abulic state and six of our patients were in good general condition. None of our patients revealed abulic state after 12 months of head trauma. In two children the Dexamin treatment was given up for seizures. We did not observe any side effects of Gliatilin. CONCLUSIONS: The kind of trauma and posttraumatic intracranial pathology do not determine the prognosis. The evaluation of treatment should be performed after 6-12 months. Gliatilin and Dexamin treatment improves the clinical state of patients with posttraumatic abulic state.

[Herpes encephalitis at children]. / Opryszczkowe zapalenie mózgu u dzieci.

Significant mortality, high incidences of complications and permanent neurological sequel are still noted in patients suffering fro herpetic encephalitis. They result mainly from delayed diagnosis and treatment of the specific cause. The aim of our paper was the analysis o a clinical course of patients with Herpes simplex encephalitis. From 1999 to 2001 7 patients aged 2 weeks to 15 years, treated in Children' Neurology Department of Silesian School of Medicine, were diagnosed to have herpetic encephalitis. Fever, headache, vomiting, as well as alteration of consciousness, all typical for neuroinfection were main clinical symptoms present on admission. Three children presented with respiratory distress requiring admission to Intensive Care Unit. On examination "cold sores" were found in 2 patients, in remaining 5 the history of exposition to herpes labialis was obtained. On neurological examination we found either right or left hemiparesis in all patients, motor aphasia in 2 and left sided central facial nerve palsy in 1. Lumbar puncture revealed lymphocytosis in 5 patients. Anti-HSV type IgG an IgM antibodies were found in serum of all 6 patients, while only in 2 of them were detected in cerebrospinal fluid (CSF). These were the 2 most severely ill children. In 2 patients DNA HSV using PCR (polymerase chain reaction) method was found in CSF and in serum. Magnetic resonance imaging (MRI) of the head confirmed diagnosis. Although herpetic encephalitis is an uncommon, sporadic disease, the diagnosis should be considered in any child with neuroinfection and early treatment started before laboratory confirmation.

Growth hormone deficiency associated with moyamoya disease in a 16 year-old boy.

Moyamoya disease is a rare cerebrovascular disorder which, according to a few literature reports, can coexist with hypothalamic-pituitary dysfunction. We report a 16 year-old boy referred to our Department because of short stature and headaches. He additionally, at admission, presented discrete facial dysmorphy, bruxism, luxation of temporomandibular joint and cryptorchidism. The height was 146 cm (-4.3 SDS); the sexual development was P2G2A1 and the bone age 11.5 years. The intellectual development was normal. No focal neurological deficits were observed. Based on baseline and stimulated hormonal values, isolated growth hormone deficiency was diagnosed. Malformation of the cerebral vessel was suspected on magnetic resonance imaging and upon angiocomputed tomography and panangiography, a picture suggesting moyamoya disease was obtained. Growth hormone has been administered with daily injections at the dose of 0.025 mg/kg/24h, and the first year height velocity was 12 cm/yr. No adverse events resulting from the treatment have been noted so far. This case indicates that GH deficiency may be associated with moyamoya disease, possibly resulting from chronic cerebrovascular insufficiency.

[Thermal comfort in preterm babies. Infra-red colour thermography findings. Preliminary report]. / Ocena komfortu cieplnego u noworodków urodzonych przedwczesnie przy uzyciu metody termografii w podczerwieni - doniesienie wstepne.

OBJECTIVE: To assess the thermal comfort of clinically stable neonates with normal body temperature by using infra-red colour thermography (THY). MATERIAL AND METHODS: 32 babies were enrolled in the study. The axillary temperature was maintained in the range 36.6-37.0 degrees C and the air temperature in the incubator remained within the neutral range according to Hey and Katz. Temperature distribution was measured by THY The abdomen and foot skin temperature, as well as the difference between both parameters were recorded. Thermal comfort for THY was defined as a difference between core and peripheral temperatures (Deltat) in the range of 1 to 2 degrees C. RESULTS: Thermal comfort defined according to THY criterion was fulfilled only in 12 patients (37.5%). We found Deltat< 1 degree C in 14 patients (43.8%), and Deltat>2 degrees C in 6 patients (6.3%). Thermal comfort defined by standard care varied from founded by THY p<0.0001. High foot temperature >or=35.0 degrees C in THY was found as a determinant for Deltat< 10 degrees C. Multivariate logistic regression analysis found gestational age <30 weeks to be connected with the risk of hyperthermia [odds ratio 8.4 (95% Cl 1.2-61.2). CONCLUSIONS: We concluded that there is a risk for hyperthermia in prenaturely, immature babies when nursed in standard neutral temperature. Infra-red colour thermography gives additional information which can be used for further studies on determination of optimal thermal comfort in newborn.

The influence of weight loss on serum osteoprotegerin concentration in obese perimenopausal women.

OBJECTIVE: To assess the influence of weight reduction therapy on serum osteoprotegerin (OPG) concentration in obese patients and compare these results with normal-weight controls. RESEARCH METHODS AND PROCEDURES: Forty-three obese women (BMI, 36.7 +/- 4.1 kg/m2; mean age, 50.1 +/- 4.5 years) were studied. The control group consisted of 19 normal-weight women (BMI, 24.2 +/- 2.1 kg/m2; mean age, 53.8 +/- 5.2 years). In all patients, serum concentrations of OPG, C telopeptide of type I collagen containing the cross-linking site (CTX), osteocalcin, parathormone, 25-(OH)-D3 (vitamin D), and total calcium and phosphorus were assessed before and after a 3-month weight reduction therapy. RESULTS: In obese subjects, serum concentrations of OPG, 25-(OH)-D3, osteocalcin, total calcium, and phosphorus were significantly lower, and serum concentration of parathormone was significantly higher, before weight reduction therapy in comparison with normal-weight controls. After weight reduction, a significantly higher serum concentration of 25-(OH)-D3 and CTX and significantly lower concentration of OPG were found. DISCUSSION: Serum concentration of OPG was significantly lower in obese patients in comparison with normal-weight controls. Weight reduction therapy resulted in further decrease in OPG serum concentrations. Therefore, OPG cannot be treated as a protective factor from bone loss in obese patients.

Determination of lactic acid level in systemic liquids in children with progressive encephalopathies.

BACKGROUND: This article reports the results of research into the activities of lactic acid concentrations in the body fluids of children with progressive encephalopathies (PE) in comparison to patients with non-progressive encephalopathies (NPE) and those with non-progressive encephalopathies with concomitant epilepsy (NPEE). The study was designed to determine whether there is difference between the serum and CSF lactic acid concentrations in children with progressive encephalopathies (PE), static (non-progressive) encephalopathies (NPE) and non progressive encephalopathies with concomitant epilepsy (NPEE), and whether the clinical status correlates with the concentration of these biochemical markers in children with PE. MATERIAL/METHODS: The assessment involved 138 children of both sexes, whose age ranged between 8 months and 15 years, diagnosed and treated in the Neurology Department at the Pediatric Clinic of the Silesian Medical Academy in Katowice between 1995 and 1997. Lactate concentrations were determined in serum and cerebro-spinal fluid and analyzed statistically. RESULTS: The findings showed higher serum and CSF concentrations in children with PE than in patients who manifested non-progressive forms of encephalopathy. The degree of clinical symptom aggravation in PE children was likewise analyzed and compared to the values of lactate concentrations in body fluids; however, no correlation was found between these parameters. CONCLUSIONS: Children with progressive encephalopathies present higher lactate concentrations in serum and cerebrospinal fluid than patients with static (non-progressive) encephalopathy.