T1-weighted fluid-attenuated inversion recovery and T1-weighted fast spin-echo contrast-enhanced imaging: a comparison in 20 patients with brain lesions.

T1-weighted fluid-attenuated inversion recovery (FLAIR) sequence is a relatively new pulse sequence for intracranial MR imaging. This study was performed to compare the image quality of T1-weighted FLAIR with the T1-weighted FSE sequence. Twenty patients with brain lesions underwent T1-weighted fast spin-echo (FSE) and T1-weighted FLAIR during the same imaging session. Four quantitative and three qualitative criteria were used to compare the two sequences after contrast. Two of four quantitative criteria pertained to lesion characteristics: lesion to white matter (WM) contrast-to-noise ratio (CNR) and lesion to cerebrospinal fluid (CSF) CNR, and two related to signals from normal tissue: grey matter to WM CNR and WM to CSF CNR. The three qualitative criteria were conspicuousness of the lesion, the presence of image artefacts and the overall image contrast. Both T1-weighted FSE and FLAIR images were effective in demonstrating lesions. Image contrast was superior in T1-weighted FLAIR images with significantly improved grey matter-WM CNRs and CSF-WM CNRs. The overall image contrast was judged to be superior on T1-weighted FLAIR images compared with T1-weighted FSE images by all neuroradiologists. Two of three reviewers considered that the FLAIR images had slightly increased imaging artefacts that, however, did not interfere with image interpretation. T1-weighted FLAIR imaging provides improved lesion-to-background and grey to WM contrast-to-noise ratios. Superior conspicuity of lesions and overall image contrast is obtained in comparable acquisition times. These indicate an important role for T1-weighted FLAIR in intracranial imaging and highlight its advantage over the more widely practiced T1-weighted FSE sequence.

Fucosidosis: immunological studies and chronological neuroradiological changes.

A 3.5-y-old boy of Arabic origin had the clinical features of both type 1 and type 2 fucosidosis, consistent with an intermediate form of the disease. The activity of his leucocyte alpha L-fucosidase was absent. He presented with recurrent sinopulmonary infection and otitis media in addition to paronychia and a periapical dental abscess. Investigation of his systemic immune function did not reveal a significant underlying defect, but subtle abnormalities, particularly of antibody production and secretory IgA, cannot be excluded. The cranial magnetic resonance images showed periventricular and subcortical white matter abnormalities and mild cortical atrophy in addition to globus pallidus changes.

Dandy-Walker malformation associated with syringomyelia: an unusual presentation.

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Lissencephaly associated with cerebellar hypoplasia and myoclonic epilepsy in a Bedouin kindred: a new syndrome?

Clinico-radiological assessment of three mentally retarded members of a large Bedouin kindred showed lissencephaly, spastic paraparesis, myoclonic epilepsy and cerebellar hypoplasia. It seems that the familial association of lissencephaly/myoclonic epilepsy/cerebellar hypoplasia represents a new entity.

Case report: calcified pancreas and bile ducts from schistosomiasis.

Schistosomiasis is one of the commonest parasitic diseases affecting mankind. Egyptian schistosomiasis (mansoni and haematobium) is capable of producing fibrosis and/or calcification, resulting in striking radiological features in target organs. Our two cases with biliary and pancreatic calcification provide a combination of features not documented previously.

Radiologically visible gallbladder calcification due to schistosomiasis.

In four out of six gallbladders with schistosomal infestation, calcified granulomas were dense enough to be seen on abdominal radiographs. In one patient, faint calcification seen on a specimen of the excised gallbladder was not visible on the abdominal radiograph. Calcification on abdominal radiographs, especially serpiginous, seen in the region of the neck of gallbladder, appears to be the clue to the diagnosis of gallbladder schistosomiasis in people from endemic areas.

CT of genitourinary calcification due to schistosomiasis.

Calcification of the ureters, bladder and seminal vesicles was detected in six patients studied on computed tomography (CT) for evaluation of genitourinary schistosomiasis and in four others examined for intestinal schistosomiasis. Other findings included pyelocaliectasis in ten kidneys, vesical carcinoma in one, seminal vesicle granuloma in one and non-visceral calcification in pelvic tissue in one patient.

Autosomal recessive ataxia, slow eye movements, dementia and extrapyramidal disturbances.

An Arab family with an autosomal recessive form of spinocerebellar degeneration with slow eye movements is reported. Hitherto all the reported cases were either sporadic or of autosomal dominant inheritance. Associated are progressive intellectual impairment and extrapyramidal dysfunction as well as peripheral neuropathy and skeletal abnormalities. Muscle biopsy revealed non-specific mitochondrial abnormalities. The spectrum of eye movement abnormalities is discussed and the literature is reviewed. It is concluded that the hallmark of this syndrome (slow or even absent saccades) is one of a group of oculomotor abnormalities, all being characterized by delayed initiation and slow velocity. The syndrome seems to be related to the olivopontocerebellar degenerations, but differs in that there is in addition selective degeneration of certain tracts and nuclei in the mesencephalon and probably more rostral structures.

Neurological presentation of neuro-Behçet's syndrome: clinical categories.

In an attempt to categorize the diverse neurological manifestations of Behçet's syndrome, a group of 10 patients was prospectively studied; the diagnosis of Behçet's syndrome was not known in any of them prior to their neurological presentation. A reasonably clear distinction can be made to divide the manifestations into three general categories with some overlap. Category I includes patients with increased intracranial pressure with or without cerebral venous sinus thrombosis. Category II are those presenting with a stroke. Category III are those patients who present with spinal cord involvement and CSF pleocytosis. Brainstem involvement was noted independently of the group. In general, patients with Neuro-Behçet's syndrome showed little ocular involvement. The course of the illness both in morbidity and mortality tended to be better in category I, and mortality was only seen in category II patients. In populations susceptible to the disease, the clinical categorisation proposed may alert the clinician to the possibility of neuro-Behçet's syndrome whenever patients with pseudotumour cerebri, dural sinus thrombosis, stroke in the young, unexplained myelitis or possible multiple sclerosis are encountered.

Late onset cerebello-pontomesencephalic degeneration.

Two siblings are presented with late onset, rapidly progressive truncal ataxia, paralysis of down-gaze and loss of up-gaze saccades in association with other oculomotor dysfunctions as well as dementia. Electron microscopic muscle studies revealed abnormal distribution and form of the mitochondria, probably being the ultrastructural basis of the pathologic changes. A neurological syndrome as that described here has not been reported before.

A giant spinal aneurysm with cord and root compression.

A patient suffering from a giant spinal aneurysm is reported. The diagnosis was made by angiography and dynamic computed tomography. The patient was treated successfully by clipping of the feeding artery.

CT of cerebral hydatid disease.

Six cases of cerebral hydatid disease (CHD) were seen in Kuwait over a period of 8 years. The typical CT appearance of a large well-defined spherical nonenhanced unilocular cyst was seen in four cases. Two unusual but characteristic types of calcification were seen, one in each of the remaining two cases.

Internal carotid artery occlusion in a young female with mitral valve prolapse.

A healthy 28 year old housewife presented with sudden right hemiplegia and aphasia. No predisposing factors could be ascertained apart from posterior cusp mitral value prolapse on two dimensional echocardiography. Extensive investigations confirmed the presence of cerebral infarction and persistent occlusion of the left internal carotid artery near its origin.

Autosomally inherited recessive spastic ataxia, macular corneal dystrophy, congenital cataracts, myopia and vertically oval temporally tilted discs. Report of a Bedouin family--a new syndrome.

A Bedouin family is described with an unusual form of spinocerebellar degeneration. Spastic ataxia was found to be associated with congenital cataracts, macular corneal dystrophy and non-axial myopia, in the absence of retardation of somatic or mental maturation. Immunological abnormalities were common. Genetic analysis revealed that the pedigree is expressing the transmission and segregation of a single mutant autosomal recessive gene.

Human cystic hydatid disease: treatment with interrupted courses of mebendazole.

Ten patients suffering from intra-abdominal cystic hydatid disease, confirmed by serological tests and CT scan were treated with mebendazole. Each received 1800 mg day-1 either as a continuous therapy for 18 weeks, or as interrupted 6-week courses for a minimum of three courses, with intervals of 19-22 weeks between the courses. One patient dropped out of the study because of the development of jaundice 11 days after initiation of treatment. Nine patients were treated and followed up for the minimum of 20 months; of these, three patients received continuous therapy and six received interrupted therapy. One patient from the continuous therapy group showed progressive regression of his cyst, one showed minimal radiological changes only, while the third patient showed minimal radiological changes plus per-operative evidence of partial destruction of the cyst. Of those treated with interrupted therapy, one patient showed continuous progression in the size of the cyst with the appearance of a new cyst, although there was radiological and operative evidence of partial destruction of the cyst. One patient initially showed regression, but later progression of his cyst, while four patients showed no benefit from the treatment.

Abdominal hydatid disease: follow-up of mebendazole therapy by CT and ultrasonography.

Computed tomography (CT) and Ultrasonography (US) were used for the diagnosis and follow-up of 10 cases of abdominal hydatid disease receiving treatment with Mebendazole because of a relative contraindication to surgery. With the exception of one case, the duration of follow-up varied from 20-36 months. There was an estimated 60% decrease in cyst size in one case, 10% decrease in another, unchanged in six (mostly calcified ones) and 60% increase in one case. CT density measurements of fluid content of the cysts increased in three cases accompanied by increased internal echoes on US in one case. The number and size of daughter cysts were reduced in one case and more calcification was noted in two cases. There was an overall slow and poor response to treatment and four cases eventually had surgery.

CT of hepatic schistosomiasis mansoni.

Schistosomal periportal fibrosis produced a typical pattern on computed tomography in five patients. Low-density periportal tissue, present throughout the liver, enhanced strongly after the administration of contrast medium. While rounded in cross section, the thickened periportal tissue produced linear and branching patterns when imaged in longitudinal section. In all cases, the sonographic features were typical of schistosomal periportal fibrosis. A lack of awareness of the distinctive features of periportal fibrosis may result in a mistaken diagnosis of hepatic metastases.

Pituitary abscess.

Three cases of pituitary abscess are presented. The history of recurrent attacks of aseptic meningitis, together with radiological and clinical features suggestive of pituitary tumor, appear to form a fairly typical picture of the condition. Long follow-up was possible in two of the cases. There are no radiological features which distinguish the lesion from pituitary tumor, hence the importance of recognizing the significance of such a clinical presentation with radiological evidence of sellar enlargement. Pituitary abscesses seem to occur in preexisting pituitary tumors. The possible relationship with pituitary infarction is discussed.