Suspicious neuroimaging pattern of thrombotic microangiopathy.

The TMAs are a group of microvascular occlusive disorders characterized by thrombocytopenia and intravascular hemolysis. Literature review reveals a spectrum of neuroimaging findings, including a single case report of multifocal hemorrhagic infarctions. We present a series of 12 patients with TMA demonstrating a similar pattern of multifocal cortical and subcortical hemorrhagic infarctions.

Resting brain perfusion in alcohol-induced psychotic disorder: a comparison in patients with alcohol dependence, schizophrenia and healthy controls.

INTRODUCTION: Alcohol-induced psychotic disorder (AIPD), also known as alcohol hallucinosis, is a rare complication of alcohol abuse. The underlying pathophysiology is poorly understood, and the disorder needs to be differentiated from alcohol withdrawal delirium and schizophrenia. No brain-imaging studies in AIPD have been reported to date. Case reports of brain imaging in AIPD suggest possible dysfunction in the thalamus, basal ganglia, frontal lobes and cerebellum. Our aim was to prospectively compare resting brain perfusion (rCBF) in patients with AIPD, uncomplicated alcohol dependence, schizophrenia and healthy volunteers. METHODS: Single photon emission computed tomography (SPECT) was utilized to compare rCBF in patients with AIPD (n=19), schizophrenia (n=16), uncomplicated alcohol dependence (n=20) and healthy volunteers (n=19). RESULTS: Increased rCBF was demonstrated in the right calcarine area in patients with AIPD compared to healthy volunteers, with a trend towards increased rCBF to the frontal and temporal lobes and the right pallidum. Decreased left sided rCBF to the putamen, parietal, mid-frontal and mid-temporal lobes and heterogenous flow to the cerebellum were demonstrated in patients with AIPD when compared to patients with uncomplicated alcohol dependence. The left posterior cingulate and right cerebellum showed higher and lower rCBF respectively in patients with AIPD compared to patients with schizophrenia. CONCLUSION: Our findings implicate the right occipital lobe and possibly the cerebellum in the pathogenesis of AIPD and have similarities with those previously reported in alcohol withdrawal. Reduced rCBF to the frontal lobes, thalamus and basal ganglia in AIPD as suggested in previous case reports could not be confirmed.

Brachial amyotrophic diplegia (segmental proximal spinal muscular atrophy) associated with HIV infection.

Several forms of motor neuron disease occurring in association with HIV infection have been described. Segmental proximal spinal muscular atrophy or brachial amyotrophic diplegia, a rare segmental variant of motor neuron disease with isolated bilateral upper extremity weakness, has previously been described in a single case report. We describe a patient who is HIV-seropositive presenting with this phenotype and illustrate novel findings on MRI of the cervical cord, consisting of focal atrophy and T2 hyperintense signal change involving the anterior grey matter. Additionally, a number of differences compared with patients without HIV presenting with this motor neuron disease variant are highlighted.

Pilomyxoid astrocytoma: expanding the imaging spectrum.

BACKGROUND AND PURPOSE: Pilomyxoid astrocytoma (PMA) is a recently described variant of pilocytic astrocytoma (PA) with unique clinical and histopathologic characteristics. Because the histopathology of PMA is distinct from that of PA, we hypothesized that PMAs would display distinctive imaging characteristics. We retrospectively reviewed the imaging findings in a large number of patients with PMA to identify these characteristics. MATERIALS AND METHODS: CT and MR images, pathology reports, and clinical information from 21 patients with pathology-confirmed PMA from 7 institutions were retrospectively reviewed. CT and MR imaging findings, including location, size, signal intensity, hemorrhage, and enhancement pattern, were tabulated. RESULTS: Patients ranged in age from 9 months to 46 years at initial diagnosis. Sex ratio was 12:9 (M/F). Twelve of 21 (57%) tumors were located in the hypothalamic/chiasmatic/third ventricular region. Nine (43%) occurred in other locations, including the parietal lobe (2/21), temporal lobe (2/21), cerebellum (2/21), basal ganglia (2/21), and fourth ventricle (1/21). Ten (48%) tumors showed heterogeneous rim enhancement, 9 (43%) showed uniform enhancement, and 2 (9%) showed no enhancement. Five (24%) masses demonstrated intratumoral hemorrhage. CONCLUSION: This series expands the clinical and imaging spectrum of PMA and identifies characteristics that should suggest consideration of this uncommon diagnosis. One third of patients were older children and adults. Almost half of all tumors were located outside the typical hypothalamic/chiasmatic region. Intratumoral hemorrhage occurred in one quarter of patients. PMA remains a histologic diagnosis without definitive imaging findings that distinguish it from PA.

Massive posterior fossa tuberculous abscess developing in a young child treated for miliary tuberculosis. Possible role of very rapid acetylation of isoniazid.

A 21-month-old infant presented with acute obstructive hydrocephalus due to a large tuberculous abscess in the posterior fossa 3 months after starting treatment for miliary tuberculosis. Insertion of a ventriculo-peritoneal shunt resulted in some clinical improvement but subsequent neurological deterioration occurred due to massive enlargement of the tuberculous abscess despite apparently adequate antituberculosis therapy. Repeated drainage procedures of the abscess eventually resulted in resolution and clinical improvement. As part of the workup for poor weight gain and the unusual clinical course, the patient's acetylation status for isoniazid was determined and found to be very rapid. Doubling the daily dose of isoniazid was followed by a dramatic weight increase and further clinical improvement. Decreasing the load of tuberculous antigen by draining the abscesses and increasing the pulse exposure of isoniazid is the best possible explanation for the clinical improvement finally seen in this patient.

Acute stroke in a child with miliary tuberculosis.

Neurological symptoms in childhood miliary tuberculosis are generally caused by underlying tuberculous meningitis (TBM), since the 2 conditions commonly occur concurrently. Cerebral infarction, a well-recognized complication of TBM, usually results from tuberculous periarteritis and secondary thrombosis. Neuropathological studies have demonstrated that the anterior cerebral circulation is more commonly affected than the arteries of the vertebro-basilar system, and basilar artery occlusion as a presenting manifestation of childhood miliary tuberculosis or TBM has not been described before. We report a 13-month-old infant who presented with fever and convulsions, terminating in acute decerebration after a second prolonged seizure 1 week after the onset of symptoms. Magnetic resonance (MR) imaging demonstrated density changes compatible with acute vertebro-basilar ischemia as well as multiple cerebral granulomas. A chest radiograph showed diffuse miliary tuberculosis. Postmortem examination confirmed this diagnosis and revealed acute occlusion of the basilar artery by an infected (septic) thromboembolus showing granulomatous inflammation, which most likely arose from an endocardial vegetation with identical histology.

DSM-III-R classification of psychiatric symptoms in systemic lupus erythematosus.

DSM-III-R criteria applied in the evaluation of 88 systemic lupus erythematosus patients revealed a point prevalence rate of 18.2% for psychiatric disorders, the most common diagnosis being adjustment disorder (11.4%). No patients had disorders compatible with a functional psychosis. Psychiatric morbidity was not associated with increased disease activity, corticosteroid use, brain magnetic resonance imaging abnormalities, or electroencephalogram abnormalities. High scores on a life event scale were associated with psychiatric disorders, suggesting that psychosocial stress is etiologically important. Cognitive testing showed that poor performance on the Stroop Colour-Word Inference Test was associated with psychiatric disorders.

Semiquantitative postembedding characterization of intermediate filaments in central nervous system lesions using immunoelectron microscopy.

Standardized postembedding immunoelectron microscopy was performed to demonstrate glial fibrillary acidic protein (GFAP) and vimentin in individual intermediate filaments to determine the diagnostic value of demonstrating ultrastructural and immunophenotypic characteristics of intermediate filaments in routine brain biopsy specimens. Dual expression of GFAP and vimentin was observed in the astroblastoma and astrocytes of Alexander's disease. The antigen availability for vimentin, however, was too low to allow reliable assessment of the GFAP:vimentin ratio in individual intermediate filaments and/or filament bundles. In meningioma, only vimentin positive intermediate filaments were found. GFAP positive intermediate filaments were present in all other specimens except the oligodendroglial components of the mixed glioma, which were devoid of intermediate filaments. GFAP positivity in the filamentous periphery and electron-dense core of Rosenthal fibers was demonstrated. Technical and tissue processing factors had a significant effect on particle density values obtained for individual specimens. Although the number, distribution, and density of glial intermediate filaments varies in different astroglial entities, correlation of particle density values determined by immunoelectron microscopy with relative GFAP concentrations in different lesions requires utmost caution. Nevertheless, application of the postembedding approach to routinely fixed biopsy specimens indicated an association of different entities with the exclusive presence of GFAP and/or vimentin in individual intermediate filaments, thus emphasizing the diagnostic value of intermediate filament typing for pathological characterization.

Mycotoxic peripheral myelinopathy, myopathy, and hepatitis caused by Diplodia maydis in vervet monkeys.

During recent historical times many Africans changed their diet to one based on maize. The grain is regularly contaminated by fungi which are toxigenic to domestic animals and birds. After one of the fungi, Diplodia maydis, in pure culture on maize, was added to the food of omnivorous primates there was demyelination of nerves, atrophy, degeneration and necrosis of muscle, and hepatitis. These preliminary results are applicable to veterinary and laboratory animal sciences. They may also be medically significant since neuromuscular syndromes of unknown cause are prevalent among Africans. Nerve conduction velocities and organ weights are defined for vervet monkeys.

Neurocysticercosis: correlative pathomorphology and MR imaging.

CT and MR images of 32 patients with neurocysticercosis were correlated with pathomorphology. Gross morphological features of cystic larvae, complex arachnoid cysts, granulomatous abscesses, basal meningitis and mineralised nodules correlated closely with the images obtained, especially on MR, where resolution permitted visualisation of larval protoscolices. Our material indicates three forms of the natural history of neurocysticercosis related chiefly to anatomic location, and provides details of the evolution of large, complex arachnoid cysts.

MR of childhood tuberculous meningitis.

MR imaging was performed on 27 children with stage II-III tuberculous meningitis for the specific purpose of examining the brainstem, as well as comparison with other CT features of the disease. In addition to defining the ischemic disturbances of basal ganglia and diencephalon more clearly, MR also demonstrates the frequent occurrence of parenchymal signal abnormalities in the brainstem and adjacent temporal lobes, which are invisible or uncertain on CT. Although the presence of brainstem abnormalities on MR correlated well with clinical findings of brainstem dysfunction, clinical staging on admission remains the best prognostic indicator in advanced TBM. We also review the MR features of basal exudation, hydrocephalus and tuberculoma.

Diagnostic problems of leptomeningeal lymphoma. A report of 3 cases.

Three cases are presented in which occult lymphoreticular malignant tumour spread to the spinal and cranial subarachnoid spaces inducing a problematic neurological illness characterised by poorly localised neuralgic pain, slowly progressive paresis and, in 2 patients, papilloedema with computed tomographic evidence of ventricular dilatation. Despite intensive investigations, diagnosis was only achieved at autopsy. A progressive disturbance of spinal and cranial nerve function should direct the attention of the clinician to the possibility of diffuse meningeal involvement by a malignant or inflammatory process.

A case of the Neu-Laxova syndrome: prenatal ultrasonographic monitoring in the third trimester and the histopathological findings.

A diagnosis of the Neu-Laxova syndrome was made by ultrasonography in the third trimester of pregnancy. Initial ultrasonographic examination, at approximately 28 wk gestation, showed intrauterine growth retardation (IUGR), hypoechoic skeletal structures, kyphosis, feeble fetal activity, and restricted limb movement. Subsequent sonograms showed microcephaly with a receding forehead and prominent eyes, generalised edema and flexion deformities of limbs. Late third trimester findings included polyhydramnios, swelling and webbing of the knee and elbow joints, and severe edema of the hands and feet, giving the impression of absent digits. The clinical features of this case are consistent with group II of Curry's classification of the Neu-Laxova syndrome [Curry, 1982]. The histopathological findings, a triad of dermatological features, poor cortex formation of the long bones, and central nervous system (CNS) dysgenesis, are discussed. In view of the 25% recurrence rate, at risk pregnancies should be carefully monitored by ultrasonography: at 6-8 wk for accurate dating, at 12-16 weeks for active fetal limb movement, and at 16-24 wk for facial and skeletal anomalies, the detection of IUGR, and polyhydramnios.

Antenatal ultrasonographic findings in tuberous sclerosis. Report of 2 cases.

Tuberous sclerosis is an autosomal dominant disease with variable expression. The initial manifestations have been reported at varying stages postnatally, but little is known about the fetal age at which the disease may be detected. The antenatal ultrasonographic features in 2 affected fetuses, at 40 and 26 weeks respectively, are reported. The second case appears unique because of the early fetal involvement and the abnormally large brain tumour.

Correlative clinical, neuroradiological and pathological findings in subacute sclerosing panencephalitis: a report of 5 cases.

Changes in the brain in subacute sclerosing panencephalitis (SSPE) as detected on computed axial tomography (CT) have so far been insufficiently assessed. An attempt was therefore made to correlate the clinical, neuroradiological and pathological findings in 5 consecutive, unselected cases, 4 of which were somewhat atypical. It is concluded that within the context of a degenerative neurological disorder of childhood, the CT findings in SSPE correlate well with clinical staging, and also illuminate certain aspects of the pathogenesis and dynamics of the disease process.

Nemaline rod disease, with reference to the routine use of histochemical methods in autopsy investigations.

A 50-year-old man had suffered from an undiagnosed form of muscle disease for most of his life. Death was due to cardiac failure. Numerous samples of muscle, obtained at autopsy, were subjected to the usual histochemical techniques used in investigating neuromuscular disease. Frozen sections revealed the abnormal accumulations of Z-band protein that characterize nemaline rod disease. That this disorder would have escaped diagnosis had only routine paraffin-embedded sections been examined is emphasized.