Sequential MR studies of a patient with white matter disease presenting psychotic symptoms: ADEM versus single-episode MS

Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) are both demyelinating white matter disorders. It is difficult to differentiate ADEM from single episode MS because of the similar clinical presentation, cerebral spinal fluid (CSF) analysis, histological finding and magnetic resonance image (MRI) appearance. We report an ADEM case with unusual clinical presentations of predominant psychiatric symptoms, and relatively long disease course. Initially, we were not able to distinguish it from single episode MS. By means of sequential MR images followed up 2 years and spectroscopy studies, and the dramatic clinical improvement after corticosteroid therapy, ADEM was diagnosed as the disease entity of this patient. In this case report we will present the MR findings of this patient and discuss the differentiation between the ADEM and MS.     

Verifying complete obliteration of carotid artery-cavernous sinus fistula: role of color Doppler ultrasonography.

The purpose of this study was to evaluate the role of color Doppler ultrasonography in verifying obliteration of carotid artery-cavernous sinus fistula before and after therapeutic embolization or gamma knife radiosurgery. Before treatment, carotid artery-cavernous sinus fistula showed the following data on color Doppler ultrasonography: (1) increased blood flow in the common carotid artery (220 to 1264 ml/min with mean+/-SD of 728+/-378 ml/min); internal carotid artery (435 to 1097 ml/min with mean+/-SD of 834+/-216 ml/min) in fistulas of the direct type; and external carotid artery (85 to 257 ml/min with mean+/-SD of 170+/-75 ml/min) in fistulas of the indirect type in comparison to the contralateral side; (2) reverse pulsatile flow or spiculated wave form with turbulent flow in the engorged superior ophthalmic vein on the lesion side in all patients. All of the above abnormal hemodynamic changes became normal in six patients immediately after first embolization, in two patients with balloon embolization combined with subsequent direct embolization by direct puncture through the superior orbital fissure or internal carotid artery embolization, and in five patients after gamma knife radiosurgery at 4, 4, 8, 9, and 9 months, respectively. Color Doppler ultrasonography might be a good modality in long-term follow-up of carotid artery-cavernous sinus fistula after gamma knife radiosurgery and embolization.     

Intracranial subdural hematoma coexisting with improvement in spontaneous intracranial hypotension after an epidural blood patch

A 36-year-old male had spontaneous intracranial hypotension (SIH) presenting with refractory headache for 4 months. Multiple epidural blood patches (EBPs) yielded relief of symptoms, but the course was complicated, with asymptomatic intracranial subdural hematoma (SDH). Except for SDH, other radiological diagnostic signs of SIH were resolved and the patient’s headaches improved after EBP. Owing to a mass effect and persistent cerebrospinal fluid (CSF) leakage, surgical repair of the spinal leakage was performed, but no cranial procedures were carried out. Postoperatively, the SDH completely resolved, but there was still CSF leakage at the level where surgery was performed. The patient has remained free of headache or other events for 3 years. It was reduction rather than elimination of the spinal CSF leak that yielded remission of SIH. In summary, intracranial SDH can be a complication of inadequately treated SIH (i.e. persistent minor CSF leakage). Management of SDH should focus on correction of the underlying SIH rather than craniotomy for hematoma evacuation.     

The Val66Met polymorphism of the brain-derived neurotrophic-factor gene is associated with geriatric depression

Brain-derived neurotrophic-factor (BDNF), the most abundant of the neurotrophins in the brain, has been implicated in both major depression and cognitive function. This study examines the association between the BDNF-gene Val66Met polymorphism and depression susceptibility and severity, age-of-onset, cognitive function and suicidal attempt history in an elderly Chinese sample population. We genotyped the BDNF-gene Val66Met polymorphism in 110 elderly inpatients diagnosed with major depression and 171 age- and sex-similar control subjects. All patients were assessed with the Hamilton Rating Scale for Depression (HAM-D) for depression severity and the Mini-Mental Status Examination (MMSE) for cognitive function after admission. Suicide attempt history and age-of-onset of depression were evaluated by interview and medical record. The BDNF Val66Met genotype distribution was significantly different between depressed patients and control subjects (P = 0.003) and there was a significant excess of Met allele in the depressed patients compared to the control group (P = 0.001). The BDNF polymorphism did not affect age-of-onset, depression severity, cognitive function or suicidal attempt history. The results suggest that the BDNF Val66Met polymorphism is a relevant risk factor for geriatric depression.     

Prediction of Alzheimer's disease in mild cognitive impairment: a prospective study in Taiwan

The relationship between apolipoprotein E (ApoE) and clinical manifestations of mild cognitive impairment (MCI) has not been investigated in non-Caucasian populations. This prospective study was conducted in an ethnic Chinese population to evaluate the correlations of ApoE genotype, cognitive performance, medial temporal structure volumes, and clinical outcome in amnestic MCI. Twenty normal elders, 58 MCI, and 20 mild Alzheimer's disease (AD) patients received neuropsychological, MRI, and ApoE genotype assessments at baseline. Patients with MCI had intermediate cognitive performance and hippocampal volumes between those in normal and AD groups. In each diagnostic group, 4 carriers (E4+) consistently had smaller hippocampal volume than non-carriers (E4−) did. Nineteen MCI subjects (32.7%) converted to AD during the 3-year study period. Compared with MCI non-converters and E4− MCI converters, E4+ MCI converters had the smallest hippocampal volume. However, 4 was not a predictor for AD. Both cognitive performance and hippocampal volume were predictive for progression to AD. However, stepwise Cox regression model integrating both neuropsychological and radiological variables showed that global cognitive performance was the only significant predictor for AD. A poor global cognitive score may be more crucial than a small hippocampal volume in the prediction of AD.     

Computed tomography and magnetic resonance imaging characteristics of giant pituitary adenomas.

PURPOSE: The purpose of this study was to characterize the computed tomography (CT) and magnetic resonance (MR) imaging features of giant pituitary adenoma (GPA) and to demonstrate the pathways of the spread of GPA. METHODS: CT and/or MR imaging scans of 356 patients evaluated preoperatively for single pituitary tumor were reviewed. Fourteen tumors (4%) fulfilled the radiologic criteria for GPA. There were 10 male and four female patients, ranging in age from 22 to 71 years (mean, 52 yr). We retrospectively reviewed the CT and MR imaging scans of these patients to characterize tumor appearances and identify the pathways of tumor extension. RESULTS: Thirteen tumors (93%) extended upward to the suprasellar cistern, and/or hypothalamus and third ventricle. Infrasellar extension through the sellar floor and sphenoid sinus to the skull base, or to the ethmoid sinus or the nasopharynx, was identified in seven patients (50%). Eight patients (57%) had lateral invasion to the cavernous sinus. Temporal and frontal extension was apparent in seven patients (50%) and six patients (43%), respectively. Five patients (36%) had posterior subtentorial extension to the posterior fossa. Histologically, only two GPAs showed invasive features. There was no correlation among histologic features, pituitary hormone concentrations, and evidence of tumor aggressiveness on CT and MRI scans. CONCLUSIONS: GPA has the potential for widespread, multi-directional extension. Our results indicate that any type of pituitary adenoma, regardless of its endocrinologic activity, may invade surrounding structures. Suprasellar invasion is the most common pathway of tumor spread, followed by infrasellar, lateral, anterior, and posterior routes.     

Probable variant Creutzfeldt–Jakob disease in Asia: a case report from Taiwan and review of two prior cases

New variant Creutzfeldt–Jakob disease (vCJD) was first identified in the UK in 1996, and was causally linked to bovine spongiform encephalopathy. Herein we report the first case of vCJD in Taiwan: a 34-year-old man who had lived in the UK between 1989 and 1997. The patient presented with depression, irritability, personality change, painful feet and allodynia, followed by gait ataxia and cognitive impairment. Electroencephalograms did not show the typical appearance of sporadic CJD. The cerebrospinal fluid 14-3-3 protein immunoassay was negative. Brain magnetic resonance imaging revealed high signal lesions involving bilateral caudate nuclei, left lentiform nucleus, bilateral dorsomedial thalami and pulvinar on fluid-attenuation inversion recovery, T2- and diffusion-weighted imaging. Prion protein gene analysis showed homozygous for methionine at codon 129. The patient developed akinetic mutism at 16 months and died at 28 months after onset. The clinical presentation and neuroimaging findings were compatible with the vCJD cases reported since 1996, and met the World Health Organization Case Definition for probable vCJD. In this communication, we also review two other cases of vCJD in Asia. All three cases were assumed as imported cases from the UK because of the residential or travel history of the patients.     

Association of APOE genetic polymorphism with cognitive function and suicide history in geriatric depression

Apolipoprotein E (APOE) has been associated with a variety of late-life neuropsychiatric disorders, including geriatric depression. This study determined whether APOE genotypes affect vulnerability to geriatric depression. We also tested the effect of the presence of the APOE epsilon4 (APOE4) allele on age of onset, suicide attempt history and cognitive function in geriatric depressed patients. We genotyped APOE in 111 elderly inpatients diagnosed as having major depression and 144 normal controls. The depressed patients were evaluated at baseline using the Hamilton Rating Scale for Depression and the Mini-Mental State Examination (MMSE) after admission. Age of onset of depression and suicide attempt history in the depressed group were evaluated by interview and medical record. We found no association between APOE genotypes and geriatric depression (p = 0.342) or APOE4 status and age of onset of depression (p = 0.281). However, compared with depressed subjects lacking the APOE epsilon4 allele, depressed subjects who were also APOE4 carriers showed significantly lower MMSE scores (p = 0.021) and an increased suicide attempt history (p = 0.012). The APOE genotype may contribute to cognitive performance and suicidality in geriatric depression, rather than being a specific risk factor for the disorder.     

Cervico-cerebrovascular anomalies in children with PHACE syndrome.

BACKGROUND: The acronym PHACE describes a rare neurocutaneous syndrome encompassing the following features: posterior fossa brain malformation, hemangioma, arterial anomalies, coarctation of aorta and cardiac defects, and eye abnormalities. This report describes the cervico-cerebrovascular anomalies (CCVA) in 7 patients with PHACE syndrome. METHODS: The medical records of 7 children (4 girls and 3 boys) with the diagnosis of PHACE syndrome who underwent cranial computed tomography and magnetic resonance imaging as well as complete cerebral angiography were retrospectively studied. RESULTS: All 7 patients had underlying CCVA. Neurologic symptoms were present in 5 of the 7 patients. CCVA included vascular stenosis and/or occlusion (n = 5), aberrant course or origin of arteries (n = 7), persistent embryonic carotid-vertebrobasilar anastomoses (n = 2) and fusiform or berry aneurysm formation (n = 5). Arterial agenesis or absence was found in 2 patients and hypoplastic arteries were present in 3 children. CONCLUSIONS: PHACE syndrome is associated with a high incidence of wide-spectrum CCVA. The most common CCVA is aberrant course or origin of arteries, followed by arterial stenosis and/or occlusion, aneurysm formation, arterial agenesis or hypoplasia and persistent carotid-vertebrobasilar anastomosis. Part of the CCVA may be associated with secondary neurologic sequelae. PHACE syndrome should be considered in any children with extensive facial hemangioma. Neuroimaging such as brain computed tomography, magnetic resonance imaging, and magnetic resonance angiography are recommended in order to search for structural cerebral defects and intracranial vascular alterations.