Norovirus gastroenteritis and seizures: an atypical case with neuroradiological abnormalities.

We describe an 8-year-old boy admitted because of prolonged seizures during norovirus gastroenteritis without any signs of encephalopathy. Blood tests were normal and cerebrospinal fluid examination resulted negative for both bacteria and viruses. A reverse transcriptase polymerase chain reaction revealed norovirus RNA in a stool sample. A cerebral computed tomography turned out to be normal whereas subsequent cerebral magnetic resonance imaging showed transitory signal abnormalities consistent with vasogenic edema. The post-ictal electroencephalogram revealed normal background activity with sporadic left posterior delta waves. The child was discharged after 10 days with an unremarkable physical examination. A cerebral magnetic resonance imaging and an electroencephalogram after 1 month were both negative. We report a new case of benign infantile convulsions due to norovirus gastroenteritis with neuroradiological abnormalities to the pertinent literature in order to improve knowledge about this disorder and increase the possibility of clarifying its pathogenesis.

Unusual neuroimaging in a young boy with cerebral X-linked adrenoleukodystrophy.

We describe the case of a 7-year-old boy with cerebral X-linked adrenoleukodystrophy presenting with neurobehavioral symptoms. Attention deficit with hyperactive behavior was followed by progressive memory retrieval deficits and difficulty in writing. A detailed review of the clinical history, clinical examination and contrast-enhanced brain nuclear magnetic resonance imaging disclosed indeed an atypical cerebral form of X-linked adrenoleukodystrophy with diffuse anterior and posterior white matter involvement with a typical rim of contrast enhancement. A severe clinical deterioration occurred despite dietary treatment and hormonal replacement therapy. These rare but severe forms of atypical cerebral X-linked adrenoleukodystrophy can more often mimic attention deficit/hyperactivity disorder and should, therefore, be considered in young males with progressive neurobehavioral symptoms, particularly when possibly related to frontal white matter involvement. An accurate clinical history and examination should determine whenever a contrast-enhanced head MRI should be indicated.

Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre.

Juvenile localized scleroderma (JLS) includes several subtypes including plaque morphea, linear scleroderma and the en coup de sabre type which affects face and head. The latter variety may involve the eye and the brain with various appearance and clinical complications.We describe the case of a 6-year-old boy who presented partial complex seizures, with status epilepticus, four months before the appearance of sclerodermatous skin lesions on the face. This case report raises important questions on the pathogenesis of JLS and, particularly, on the issue whether it is a mere autoimmune condition or a neuro-cutaneous disease.

Germinoma with synchronous involvement of midline and off-midline structures associated with progressive hemiparesis and hemiatrophy in a young adult.

INTRODUCTION: Cerebral germinomas, the most common and least malignant intracranial germ cell tumors, usually arise in the pineal or suprasellar region and have characteristic clinical and radiological features. Germinomas more rarely occur in the thalamus, basal ganglia, and internal capsule, causing sometimes cerebral hemiatrophy and hemiparesis. More rarely, other clinical features can be fever of unknown origin, visual disturbance, and neuropsychiatric symptoms. Cerebral hemiatrophy can precede the imaging depiction of the off-midline mass. CASE: The authors present the first case of cerebral germinoma with synchronous involvement of the midline and off-midline structures, with unusual clinical and radiological presentation. DISCUSSION: The literature is reviewed, and the pathogenesis, the clinical findings, the imaging, and the therapy are discussed.

Hemiconvulsion-hemiplegia-epilepsy syndrome: early magnetic resonance imaging findings and neuroradiological follow-up.

We describe a case of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome documented by longitudinal magnetic resonance imaging (MRI). A two-year and nine-month-old boy had a prolonged hemiconvulsion during fever followed by right hemiparesis. Seven days later the imaging abnormality on T2 and diffusion-weighted images (DWI) was limited to the white matter of the left hemisphere. One month later severe gliosis and unilateral brain atrophy were already evident. MRI is useful in the early stages of prolonged seizures and T2 and DWI abnormalities appear to be well correlated with parenchymal damage that results from sustained ictal activity. The neuroradiological findings in our case and in the few HHE patients reported in the literature seem to be very characteristic and, if confirmed in larger series, could permit an early diagnosis.

Acute and late morbidity after limited resection and focal radiation therapy in craniopharyngiomas.

Aggressive surgery of craniopharyngioma can cause severe, life-long hypothalamic and pituitary dysfunctions and possibly further impair visual function; conventional radiation therapy (RT) can affect intellectual functioning and cause secondary tumours. Because of the severe morbidity associated with aggressive surgery, many authors nowadays recommend a less radical approach followed by RT. This combined approach allows achieving 70-83% 10-year local control rates which are comparable to that achieved with aggressive surgery. The main morbidity of this conservative combined approach is represented by pituitary dysfunction secondary to RT, however, sparing severe hypothalamic disturbances. The interval between treatment and onset of the disorder is much longer than in the case of aggressive surgery and this can have a beneficial impact on quality of life, especially in children. This alternative therapeutic approach has become more appealing now that modern RT techniques allow safer delivery of the RT, particularly in childhood.

Diffusion-weighted imaging in the assessment of neurological damage in patients with methylmalonic aciduria.

Diffusion-weighted magnetic resonance imaging (DW-MRI) of the brain was performed in two patients with methylmalonic aciduria who presented signs and symptoms of neurological involvement without metabolic decompensation. Patient 1 presented acute metabolic stroke and patient 2 presented subacute encephalopathy. Brain DW-MRI confirmed very recent damage in patient 1, while the absence of brain lesions on brain DW-MRI indicates the development of more chronic damage in patient 2. Brain DW-MRI represents an additional and complementary tool in the assessment of brain damage in methylmalonic aciduria patients who develop neurological syndrome.

Quantitative ultrasound at the hand phalanges: comparison with quantitative computed tomography of the lumbar spine in postmenopausal women.

The aim of our study was to evaluate the diagnostic effectiveness of a quantitative ultrasound technique for measuring bone tissue at the proximal phalanges of the non-dominant hand. We correlated the mean value of the amplitude-dependent speed of sound (AD-SoS) and the ultrasound bone profile score (UBPS) measured at the phalanges with bone mineral density (BMD) of the lumbar spine gauged with quantitative computed tomography (QCT). We studied a group of 177 postmenopausal women consecutively presenting for osteoporosis screening. We observed that in the whole study group both AD-SoS and UBPS correlated weakly with BMD, although with statistical significance (r = 0.54 and r = 0.45, respectively; p < 0.0001). The same parameters showed a poor correlation (r = 0.48, p < 0.0001; and r = 0.23, p = 0.017) in the group of patients which QCT has classified as osteoporotic. Finally, no correlation was found in the rest of our population. The correlation coefficients of comparison between our QUS and QCT measurements are not strong enough to allow us a prediction of one measure from the other. Therefore, we do not consider the phalangeal osteosonography a valid substitute of QCT technique for assessment of bone status in postmenopausal women.

Ultrasonographic evaluation of superficial lymph node metastases in melanoma.

The aims of the present work were to assess the diagnostic accuracy of ultrasonographic evaluation of superficial lymph nodes in patients with cutaneous melanoma and to describe the sonographic characteristics which permit early detection of neoplastic nodal involvement. Eighty-seven patients (89 lymph node sites) were studied for approximately a 3-year period, with a minimal surveillance time of 1 year. The ultrasonographic imaging equipment utilized were a 10 MHz scanner with a mechanical and one with 10 MHz electronic linear probe. The characteristics considered indicative of possible metastatic involvement were: round shape (short to long axis ratio > 0.5), no central hilus, nodular areas within the lymph node, sinuosity of the lymph node edges and lymph node with regular morphology and echostructure but with maximum diameter greater than 3 cm. Generally inguinal and axillary lymph nodes are larger than cervical ones. Of the 89 sites explored, 32 were considered 'suspect'. All 32 of these were subjected to cytology using ultrasound-guided, fine needle aspiration. The remaining 56 came in for a periodic control examination during a year. Thirteen of the 32 'suspect' lymph nodes proved positive at the pathologic examination. Two patients whose ultrasound diagnosis was negative developed metastases within 2 to 4 months (ultrasound false negatives). Our study indicates that there are sonographic features indicative of lymph node metastases from melanoma even in the early stages of the disease. Ultrasound scanning, therefore, is a useful diagnostic tool in the follow-up of melanoma patients, identifying which should be subjected to further testing with needle biopsy.

[Imaging of the thymus gland in myasthenia gravis (computerized tomography and magnetic resonance)]. / L'imaging del timo nella miastenia grave (tomografia computerizzata e risonanza magnetica).

Since thymectomy is nearly always indicated as a possible treatment of myasthenia gravis, we examined with Magnetic Resonance (MRI) and Computed Tomography (CT) 22 patients (21-62 years old) to identify the best methodological approach. MR images were obtained with a 1.5 T superconducting unit with slice thickness ranging from 6 to 8 mm and an interslice gap of 0.6 and 0.8 mm. Spin-echo images were acquired with repetition time (TR) < 700 ms and echo time (TE) of 20 ms and T2-weighted images with TR > 1800 ms and TE of 80 ms. The sections were obtained, with cardiac gating, on transverse and sagittal planes. CT was performed with contiguous 5-mm slice thickness, after intravenous bolus injection of contrast medium. All the patients underwent surgery of anterior mediastinum and histologic diagnosis was made. Both CT and MRI correctly identified the patterns of normal thymus or hyperplasia not associated with gland enlargement, the only two cases of hyperplasia with thymic enlargement and clearly demonstrated thymomas. MRI appears to be more accurate in the evaluation of the relationship between thymus and contiguous structures. If pericardial infiltration is suspected, sagittal MR scans yield accurate information on tumor spread. We recommend MRI of anterior mediastinum to rule out the presence of a thymoma and the possible involvement of contiguous structures.

Adolescent sexuality: elements and genesis.

To address the topic of adolescent sexuality and the elements that go into its genesis and evolution, it is necessary to determine when human sexuality, as a body system functioning independently from the human reproductive system, begins to function. In the case of the male, this has been shown by ultrasound technology to be at approximately 17 weeks of gestation, and subsequent developmental stages in both sexes are based on the three primary areas in human development: body, mind, sexuality. Positive aspects of sexual socialization are shown to depend on attitudes of parents, of society, of health and caring professionals, and of the child itself, to whom it is as important a part of its being as are body and mind. Significant research carried out in different parts of the world on childhood sexuality is cited and discussed with its possible long-term meanings, effects, and possible prevention of the current apparent increase in childhood sexual molestation. The need for a massive paradigm shift on the parts of professionals, parents, and society in viewing, nurturing, and protecting the sexuality of infancy and early childhood is emphasized.

On the possible prevention of sexual problems in adolescence.

The author contends that a primary cause of sexual problems is failure to teach children about sex before they reach adolescence. Physical and mental growth are nurtured and supported throughout infancy and childhood, while sexual development is stifled or distorted. As a result children reach adolescence with their natural eroticism maimed. The author pleads for a basic change in the attitudes of adults, so that parents will begin to teach their children about sexuality. By adolescence children should be prepared to understand and accept their capacity for sexual expression and be fully aware of when and how it might or might not be appropriate.