Longitudinal changes of bone density and bone resorption in hyperthyroid girls during treatment.

Low bone mineral density (BMD) and increased bone turnover are common features of untreated hyperthyroidism in adult patients. The effect of treatment on BMD is still controversial. BMD and bone metabolism in hyperthyroid children have not been thoroughly investigated. In the present study, we measured spinal and whole body BMD by dual-energy X-ray absorptiometry in a group of 13 girls (aged 5.0-14.9 years) at diagnosis of hyperthyroidism. The bone resorption rate was assessed by urine measurement of N-terminal telopeptide of type I collagen (NTX). Hyperthyroid patients have been studied longitudinally during treatment. BMD values and NTX urine concentrations have been also determined in 155 healthy Caucasian girls (aged 2.4-24.2 years). Spinal and whole body bone density measurements were significantly lower compared with healthy controls in untreated hyperthyroid girls, after correction for differences in age and anthropometric measurements (p

Unusual clinical features and early brain MRI lesions in a family with cerebral autosomal dominant arteriopathy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described inherited disorder. The pathologic gene maps on chromosome 19. The clinical spectrum of the disease consists of recurrent strokes, migraine, transient ischemic attacks, mood changes, and dementia. We report a genetically assessed CADASIL family with atypical clinical presentations of epileptic seizures. In two asymptomatic family members there were early brain abnormalities on MRI. Our report expands the clinical spectrum of CADASIL and suggests that it is possibly an undiagnosed disorder.

Cerebrotendinous xanthomatosis (van Bogaert-Scherer-Epstein disease): CT and MR findings.

PURPOSE: To describe the CT and MR findings in the brain and spinal cord of patients with cerebrotendinous xanthomatosis and to seek possible correlations between clinical, biochemical (cholestanol levels), and neuroimaging findings. METHODS: Ten patients with well-defined clinical and biochemical diagnoses of cerebrotendinous xanthomatosis were examined. Brain CT was performed in eight cases. In all patients MR was obtained using spin-echo and gradient-echo sequences. In eight patients spine MR was also performed. RESULTS: Neuroradiologic findings included diffuse cerebral and cerebellar atrophy. In half the cases, atrophy of the brain stem and corpus callosum was also found. In the majority of patients cerebellar bilateral focal lesions and mild white matter signal alterations were present. Spinal cord MR did not show signal abnormalities or atrophy. CONCLUSIONS: We found cranial alterations in patients with severe neurologic impairment, but there was no correlation with cholestanol plasma levels. No spinal cord abnormalities were present.

CT-ventriculography in a cystic lesion of the third ventricle.

One case of cystic lesion of the third ventricle is reported. The authors stress the importance of CT combined with ventriculography for the diagnosis of the intraventricular cystic lesions.

Synthesis, herbicidal activity, and mode of action of IR 5790.

IR 5790, an arylthiadiazolone herbicide structurally related to oxadiargyl and oxadiazon, was synthesized. The herbicidal activity and mode of action of IR 5790 were investigated. This herbicide has broad-spectrum pre-emergence activity against both dicotyledonous and monocotyledonous weeds. The phenotypic responses of susceptible plants, such as interruption of growth and light-dependent development of necrotic areas on the foliage, are consistent with those observed with protoporphyrinogen oxidase-inhibiting herbicides. Tissues exposed to IR 5790 in darkness accumulated protoporphyrin IX, which led to a photodynamic loss of membrane integrity upon exposure to light. Consistent with these physiological symptoms, IR 5790 strongly inhibited protoporphyrinogen oxidase, with an I(50) value of 3 nM. The presence of a sulfur atom did not significantly alter the molecular properties of the thiadiazolone ring, relative to the oxadiazolone ring of oxadiargyl, which explains why IR 5790 has the same mode of action as this herbicide.

Memory impairment in the elderly: a three-year follow-up.

A 3-yr follow-up study on the trend of memory performance in 9 elderly subjects with selective memory defects, which was carried out in an attempt to clarify the evolution of the disorder, revealed marked worsening of sensory visual memory, retention capacity and recent memory. No other mental function was compromised. The results of this study suggest that the onset of a selective memory defect in the elderly is not necessarily the expression of a demential amnestic syndrome, but rather the manifestation of a senile brain deterioration limited to the anatomo-functional memory system.

Thrombolytic therapy for thromboembolism of vertebrobasilar artery.

Thrombolytic therapy was carried out on four patients with brain stem stroke due to thromboembolism of the vertebrobasilar system. Diagnosis was confirmed by angiography. Clinical and instrumental findings indicated a very poor prognosis. Two of these patients were treated with urokinase and the other two with streptokinase for periods ranging from 16 to 44 hours. The interval between onset of symptoms and start of therapy was less than 10 hours for three of these patients. All made a gradual and almost complete recovery after only a few hours of treatment, and only one presented minimal residual neurological disability. The condition of the fourth patient, who started therapy four days after the first episode, remained unchanged until he died 16 hours later. Our observations suggest that thrombolytic therapy may be useful in treating recent acute life-threatening brain stem stroke.

Third ventricle gliomas. Report of 7 cases with benign clinical behaviour.

Neoplasms of the third ventricle are lesions arising within the ventricular cavity, often free but more often pedunculated. True third ventricle tumours are surgically removable. In a consecutive surgical series of 580 intracranial tumours the authors operated on 27 lesions of the third ventricle, 7 of which were benign gliomas. The histological diagnosis was polar spongioblastoma in 5 cases and fibrillar subependymal astrocytoma in 2 cases. The dominant clinical features were a slowly progressive or intermittently increased intracranial pressure syndrome, and mental and visual disturbances. CT and CSF contrastographic studies provided the essential diagnostic data for the therapeutic plan. CT scans at follow-up studies confirmed the successful removal of the lesions. A right trans-ventricular approach was employed in all cases. Depending on the size and position of the lesion, removal was performed through the foramen of Monro or through a sub-choroidal approach. In two patients a ventriculo-atrial shunt was necessary in spite of tumour removal. No surgical mortality occurred and patients are well at a follow-up time of from 2 to 6 years.

Magnetic resonance imaging findings in Leber's hereditary optic neuropathy.

The authors describe magnetic resonance imaging (MRI) findings in 15 subjects with different clinical stages of Leber's disease. A few and no characteristic abnormalities of the optic nerve signal were found. Nevertheless, MRI can be considered as a useful tool to differentiate optic neuritis together with visual and brainstem auditory evoked potentials.

[Internal maxillary artery and collateral circulation in thrombosis of internal carotid artery (author's transl)]. / L'arteria mascellare interna ed i circoli di compenso nella trombosi della carotide interna

From 53 cases of thrombosis of the internal carotid artery in the neck, collateral circulation through external carotid branches was angiographically demonstrated in 34 subjects. Anastomosis between external and intracranial circulations was seen to take place through three ways formed by internal maxillary, superficial temporal and facial arteries. The role of internal maxillary artery is emphasised as the most important pathway in the connections between the two circles.

[Rare malformation of the anterior-inferior cerebellar artery. Cerebellar hemisphere angioma and aneurysm of the labyrinthine artery]. / Una rara malformazione dell'arteria cerebellare antero-inferiore. Angioma cerebellare emisferico ed aneurisma della arteria uditiva interna.

The AA relate a case of a complex malformation of the AICA characterized by aneurysm of the internal auditory artery developed inside the internal acoustic channel associated with an angioma fed by the cerebellar branch of the same artery. During a long period with dizziness, tinnitus, headache in the occipital region, mild sensoneural retrocochlear hearing loss a subarachnoid hemorrhage occurred. The vascular malformations were visualized by a vertebral angiography and the axial projection resulted to be the most important clue. The AA report the case for its rarity and emphasize the vascular origin of certain unexplained kinds of vestibular syndromes in young patients.

[Report of an atypical case of Goldenhar syndrome]. / A propos d'un cas atypique de syndrome de Goldenhar.

The authors report an atypical case of Goldenhar syndrome characterized by hemifacial and cranial hypoplasia associated with severe microtia and anophthalmia on the right side, antimongoloid palpebral fissures, epibulbar epidermoid, corneal anesthesia and preauricular tags on the left side. The bilateral presence of characteristic features of Goldenhar syndrome is rare and lends support to the possibility that the patient presents an intermediate form of developmental defect of the first branchial arch. The differential diagnosis is discussed.

Myelography with iopamidol, a nonionic water-soluble contrast medium: incidence of complications.

A review of immediate and delayed side effects noted in a consecutive series of 1,138 myelograms made with Iopamidol in the Services of Neuroradiology of various hospitals and university clinics at Pavia, Perugia, Vicenza, and Parma (all in Italy). The contrast was administered by lumbar spinal route at iodine concentrations of 200, 300 and 370 mg/ml, in amounts varying from 5 to 20 ml depending on segments being examined and extant clinical indications. The incidence and characteristics of complications (headache, nausea, vomiting, evidence of radicular irritation, etc.) were assessed in relation to examination technics, segments of spine being explored, iodine concentrations, and amounts of contrast injected.

Value of CT scanning in outpatients: preliminary report on a controlled study.

A series of 100 consecutive new outpatients attending the neuroradiology service for a cranial CT scan between 1 October and 24 December 1984 were analyzed. On the day of the investigation each patient was examined by a neurologist, who gave his opinion on the value of a CT scan. The neurologist's ratings were then compared with the CT reports. More than 2/3 of the requests for CT were useless and could have been avoided by thorough clinical evaluation of the patients and full awareness of what CT scanning can contribute to diagnosis and treatment.

Central nervous system involvement in a child with hemolytic uremic syndrome.

We report a 20-month-old girl with postdiarrheal (Shiga toxin) hemolytic uremic syndrome and severe encephalopathy. Magnetic resonance (MR) images were obtained in the acute phase of the disease and after 10 months. The first MR images showed widespread high signal intensity on T2-weighted and low signal intensity on T1-weighted images, in deep and subcortical white matter; the splenium of the corpus callosum was also involved, as well as cerebellar hemispheres. Neurological symptoms and signs gradually disappeared within 35 days. Follow-up MR imaging showed almost complete resolution of the previous findings, and the patient recovered without central nervous system impairment. The neurological lesions were probably due to hypoxia, although several other mechanisms could be involved, such as metabolic derangements and the action of Shiga toxin. In spite of the dramatic clinical manifestations, we observed a good outcome, indicating that patients with similar lesions do not necessarily have a poor prognosis.

Agenesis of the corpus callosum, infantile spasms, spastic quadriplegia, microcephaly and severe mental retardation in three siblings.

A sibship consisting of three siblings, one male and two females with unrelated parents, showed a clinical syndrome including: infantile spasms with hypsarrhythmia, microcephaly, severe mental retardation and spastic quadriplegia. The pneumoencephalogram performed in two sibs showed agenesis of the corpus callosum and aqueductal stenosis with tri-ventricular dilatation. The disorder did not show a progressive course with deterioration of mental and neurologic functions. No biochemical or cytogenetic defect could be identified. Complement fixation for cytomegalovirus was negative. This syndrome complex is probably inherited as an autosomal recessive trait. The clinical and the genetic aspects of the syndrome are discussed.

Rare multiple orbital localizations of sarcoidosis. A case report.

We report a case of bilateral orbital sarcoidosis without other systemic lesions. Steroid therapy did not improve the clinical status of the patient.