In order to assess the early presenting symptoms and signs of myotonic dystrophy in childhood, 12 subjects (four females and eight males), offsprings from eight different families, were examined. The patients' ages ranged from six to 15 years and their course has been followed for four years. The family history, the presenting symptoms, the clinical features and course, the mental evaluation, the electromyographic, ophthalmological and histopathological findings, the serum enzymes, the cardiovascular and endocrinological systems of all the subjects were discussed. The authors observed that, under the same conditions of age and duration of illness, the disease is worse in those patients whose mother is the affected relative and that same investigations (EMG, ERG, echocardiography and muscle biopsy) were altered also in the pre-clinical stage of the disease.

Adolescent↗

PubMedDate not supplied

[Neuropsychological development of children born to HIV-positive mothers].

138 children born to HIV infected mothers (58 HIV infected and 80 seroreverted uninfected) were followed up at the Institute of Infectious Diseases of the University of Pavia. All patients had an evaluation of neurological assessment every three months done by a pediatric neurologist (Amiel-Tison and Towen) and an evaluation of psychological assessment by observation of child-mother interaction. The development quotient was examined using the Griffiths Scales and mental development using the Wisc-R scale. The incidence of Central Nervous System involvement was 36% in children with symptomatic HIV infection, the hump of incidence of severe encephalopathy was in the first 4 or 5 year of life, another hump (smaller) was in preadolescence. Development deficit and psychological problems were discovered both in asymptomatic infected and in non infected children, without difference of percentage between the two groups.

Acquired Immunodeficiency Syndrome↗

PubMedDate not supplied

Clinical and neurophysiological study in diabetic children and adolescents.

We investigated 82 unselected insulin dependent diabetes mellitus children and adolescents by clinical and electrophysiological evaluation to assess the frequency of diabetic neuropathy and to relate the results to age and height of patients, duration of illness and degree of metabolic control. Clinical abnormalities were found in about 1/3 of patients: these signs of diabetic neuropathy were related to the age and the duration of diabetes but not to the degree of metabolic control. Neurophysiological study showed alterations, especially of sensory nerve conduction. These results were seen to be related to height and age of patients and duration of illness but not to the degree of metabolic control, probably due to the young age of the patients. Our study confirms that diabetic neuropathy is a heterogeneous disorder that may be caused by the interaction of host susceptibility and vascular, metabolic and perhaps environmental components also in diabetic children.

Adolescent↗

PubMedDate not supplied

Personality characteristics in juvenile tension headache and migraine.

The authors report the personality characteristics of 30 subjects, 16 males and 14 females, average age 13.7 years: 13 suffered from migraine without aura, 8 from migraine with aura and 9 from chronic tension-type headache. The study was based on a detailed clinical assessment (psychodynamically-oriented interviews with the child and its parents) and evaluation by tests. A blind test of the clinical personality characteristics was then carried out. The results of these investigations indicated that 13/30 of the subjects had a neurotic personality organization, 12/30 were borderline and 5/30 had a "white relation". Given the subjects' youth, these conditions are probably to be considered transient. From the data collected it seems that migrainous subjects are distributed along a continuum that ranges from one end characterized by more evolved and adaptive mental organization and defence mechanisms and the opposite end, where mental organization is less evolved and adaptive and at a greater risk of somatization.

Achievement↗

PubMedDate not supplied

Ataxia-telangiectasia: MR and CT findings.

OBJECTIVE: The aim of our study was to describe the neuroradiologic features of 12 patients with ataxia-telangiectasia (A-T), a degenerative multisystemic autosomal recessive hereditary disorder with onset in childhood. Clinical features include cerebellar ataxia, oculocutaneous telangiectasias, and recurrent bronchopulmonary infections. Patients present varying states of immunodeficiency and a high incidence of neoplasms. Chromosomal instability with a rearrangement of chromosomes 7 and 14 is always present. MATERIALS AND METHODS: We describe the neuroradiological findings (10 MR and 2 CT) in 12 subjects: 11 with A-T and 1 heterozygote parent. RESULTS: The images revealed a diffuse cerebellar atrophy, with marked involvement of the vermis and unusual decreased thickness of the superior cortex of the cerebellar hemispheres. Hypoplasia of the inferior vermis and a large cisterna magna were also frequent signs. CONCLUSION: Magnetic resonance is the technique of choice in this type of disorder since it permits better visualization of the posterior fossa structures.

Adolescent↗

PubMedDate not supplied

Hepatic echinococcus disease: our experience over 22 years.

BACKGROUND/AIMS: It is important to single out the indications for surgery of hydatid disease of the liver at an early stage. For this, a fully comprehensive diagnostic work up of the patient is required. Surgery represents the most effective therapy and involves resection of the cysts and their outer capsule. This must be as complete as possible. MATERIAL AND METHODS: Between 1970 and June 1992, 95 patients received this combined treatment for hydatid disease although only those presenting from 1985 were followed up. RESULTS: No serious complications were noted apart from pyrexia due to secondary cholangitis or reactive pleuritis. This responded quickly to antibiotics and there was no mortality. No cases of relapse occurred although one case did show evidence of reappearance of the disease on serological testing alone, all other tests being negative. CONCLUSION: The most effective treatment for echinococcus cystic disease of the liver is radical surgery. Results are best when surgery is combined with medical therapy of benzoimidazole drugs given pre- and post-operatively. From our experience, we are now confident that surgical treatment must always be preceded and then followed by treatment with benzoimidazole antihelminthics.

Adult↗

PubMedDate not supplied

Guidelines and recommendations for the treatment of migraine in paediatric and adolescent patients. Italian Society for the Study of Headache.

Explore the source record for details and available documents.

Adolescent↗