Decreased Cerebral Blood Flow in Neuro-Behcet''s Syndrome Patients With Neuropsychiatric Manifestations and Normal Magnetic Resonance Imaging—A Preliminary Report

Involvement of the brain is one of the most important complications of Behcet's disease, but its diagnosis is difficult because of the lack of effective imaging tools. Therefore, technetium‐99m ethyl cysteinate dimer (Tc‐99m ECD) single‐photon emission computed tomography (SPECT) of the brain was used to detect abnormal regional cerebral blood flow in patients with neuro‐Behcet's syndrome (NBS). Tc‐99m ECD brain SPECT was per‐formed to detect hypoperfusion areas of the brain in 10 NBS patients with definite neuropsychiatric symptoms or signs and normal brain magnetic resonance imaging (MRI) findings. Tc‐99m ECD brain SPECT demonstrated hypoperfusion areas of the brain in all of the 10 NBS patients. The parietal lobes were the most common areas with hypoperfusion lesions. Tc‐99m ECD brain SPECT is a more sensitive and useful tool than brain MRI for detecting hypoperfusion areas of the brain in NBS patients.     

Interictal brain SPECT in patients with medically refractory temporal lobe epilepsy

The objective of this study was to evaluate the utility of interictal brain SPECT in localizing the epileptogenic focus in a population of patients of Epilepsy Clinic of Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), with medically refractory temporal lobe epilepsy (TLE) and normal computed tomography (CT) scans, studying the correlation between SPECT, electroencephalogram (EEG) and, in 11 cases, brain magnetic resonance imaging (MRI), and to compare the results to the other six literature series. Twelve (52.2%) patients presented abnormal SPECT. Among these, five (41.6% of abnormal SPECTs) presented unilateral SPECT changes at the same side of EEG (hypoperfusion in four and hyperperfusion in one), three (25% of abnormal SPECTs) presented bilateral hypoperfusion and bilateral EEG changes too, and four (33.3%) presented unilateral hypoperfusion and bilateral EEG changes. The statistical analysis was based on fuzzy logic. The correlation index among SPECT X EEG, SPECT X MRI and SPECT X EEG X MRI were highly significant, with significance levels at 0.01, p < 0.0005 and trust interval at 99% in all correlations. The correlation studies between the series presented similar results.     

Late-life psychosis and modern neuroimaging

A historical review of the literature on late-life psychosis (LLP) is presented with special emphasis on the relationship of structural and metabolic brain disease to this condition. The study of this patient population with modern neuroimaging is described. With the use of magnetic resonance imaging (MRI), single photon computerized emission tomography (SPECT), and computerized EEG, the authors estimate that approximately 50 to 60 per cent of cases will have structural or metabolic brain disease as a significant contributing factor to their psychosis. In their experience, silent vascular disease is seen in approximately one quarter of all cases. A logical clinical approach to these patients is presented.     

Correlative study of the cognitive impairment, regional cerebral blood flow, and electroencephalogram abnormalities in children with Down's syndrome

The aim of this study was to investigate possible correlations of the cognitive impairment with abnormalities of regional cerebral blood flow and electroencephalogram in children with (Down's Syndrome) DS. Nine patients with DS were evaluated by single photon emission computed tomography (SPECT) in combination with clinical findings, electroencephalography (EEG), and magnetic resonance imaging (MRI). In cases with IQs below 40, there were one or more findings of abnormal EEG/MRI and brain perfusion SPECT. In 6 cases (66.7%) EEG findings were normal, but 3 (33.3%) had abnormal EEG findings. Perfusion abnormalities were most pronounced in the fronto-parieto-temporal region in the form of hypoperfusion (n = 5) and in the right hemisphere (n = 5) than the left hemisphere (n = 1). These findings suggest that the children with DS had varying levels of structural, perfusion, and electrophysiological abnormalities in the brain and these abnormalities were reflected by measurable alterations of the cognitive functions.     

A young adult female case of Wilson's disease presenting with mental disorder and frontal lobe signs]

We report a young adult female case of Wilson's disease presenting with mental disorder and frontal lobe signs. The patient was admitted to our neurological unit on October 4, 1999 because of schizophrenia-like symptom, dysphagia, dysarthria and gait disturbance. She showed slowly progressive rigidity and dystonia. Her parents were the second cousins. Neurological examination revealed bilateral pyramidal and extrapyramidal signs, frontal lobe signs (include the imitation behavior). Tendon reflexes were slightly exaggerated in all extremities. Bilateral Babinski, Chaddock and Hoffmann signs were positive. Her verbal IQ on the Wechsler Adult Intelligence Scale-revised was 49. Biochemical examination revealed low plasma copper and ceruloplasmin concentration. Cerebrospinal fluid was normal. Cranial MRI demonstrated diffuse brain atrophy and enlargement of the lateral ventricles. T2-weighted images of the MRI demonstrated hyperintense signal in both thalamus and basal ganglia. SPECT showed hypoperfusion in the left frontal lobe, both thalamus and basal ganglia. EEG revealed diffuse theta wave. The diagnosis of Wilson's disease was made and the treatment of D-penicillamine 900 mg per day was started. This hypoperfusion of SPECT and EEG findings improved after 2 months under D-penicillamine therapy. Neurological findings showed slight improvement. A few Wilson's disease patients presenting with mental disorder have been reported. Wilson's disease should always be considered in differential diagnosis of mental disorders. We emphasize the importance of early diagnosis and treatment of Wilson's disease.     

Sensory (VEP, BAEP, SEP) and motor-evoked potentials, liquoral and magnetic resonance findings in multiple sclerosis

In order to define the most suitable instrumental protocol for the diagnosis of multiple sclerosis (MS), 41 patients with definite (D = 14), probable (P = 14) and suspected (S = 13) MS were examined with CSF immunology, brain MRI and multimodal evoked potentials. The central motor tracts were also tested. The following alteration rates were found: MRI = 78%, CSF = 63.6%, VEP = 70.0%, median nerve SEP = 50%, peroneal nerve SEP = 68.0%, BAEPs = 35.7%, motor-evoked potentials (MEPs) = 74.0%. Altogether, EPs were abnormal in 90% of cases. Normal MRI with altered EPs were found in 22% of cases, whilst a normal EP battery with defective CSF or MRI findings were found in 7%. Twenty-six out of 27 patients with P or S forms were reclassified into a D one when considering EPs and MRI features.     

Single-photon emission computed tomography of brain perfusion: analysis of 60 paediatric cases

Sixty-nine brain perfusion single-photon emission computed tomography (SPECT) scans were performed on 60 paediatric patients with various neurological diagnoses. SPECT was abnormal more frequently in degenerative brain diseases (82 per cent), than in epilepsy (63 per cent), encephalitis (62 per cent), cerebrovascular disease (43 per cent), or other brain disorders (43 per cent). SPECT was more sensitive than EEG, CT and MRI results. SPECT was of considerable value for diagnosis in many cases. It was a decisive aid in two cases, but misleading in another two, so SPECT must be related to findings obtained by clinical and other laboratory methods.     

Superiority of HMPAO ictal SPECT to ECD ictal SPECT in localizing the epileptogenic zone

PURPOSE: We examined diagnostic performances of Tc-99m hexamethylpropylene amine oxime (HMPAO) and Tc-99m electron capture detection (ECD) ictal single-photon emission computed tomography (SPECT) to localize the epileptogenic zones in mesial temporal lobe epilepsy (TLE) and neocortical epilepsy (NE). METHODS: Epileptogenic zones were identified by invasive EEG or surgical outcome. Ictal SPECT was performed with stabilized Tc-99m HMPAO (TLE, 17; NE, 23) and with Tc-99m ECD (TLE, 7; NE, 7). Single-blind visual interpretation was used to localize the epileptogenic zones. Asymmetric index was calculated. Subtraction ictal SPECT was coregistered to a magnetic resonance imaging (MRI) template. RESULTS: In TLE, the sensitivity of Tc-99m HMPAO SPECT was 82% (14 of 17) and that of Tc-99m ECD SPECT was 71% (five of seven). The asymmetric index (AI; 25 +/- 10) of Tc-99m HMPAO SPECT was larger (p = 0.05) than the AI (13 +/- 13) of Tc-99m ECD SPECT in patients with TLE. In NE, the sensitivity of Tc-99m HMPAO SPECT was 70% (16 of 23), but that of Tc-99m ECD SPECT was 29% (two of seven). The AI (15 +/- 10) of Tc-99m HMPAO SPECT was significantly larger (p = 0.02) than the AI (4.8 +/- 6) of Tc-99m ECD SPECT in patients with NE. Subtraction ictal SPECT coregistered to MRI supported the visual assessment. CONCLUSIONS: We concluded that the sensitivity of Tc-99m ECD ictal SPECT is similar to that of Tc-99m HMPAO ictal SPECT in TLE; however, ictal hyperperfusion was higher with the Tc-99m HMPAO SPECT. In patients with NE, Tc-99m HMPAO ictal SPECT also was superior to Tc-99m ECD ictal SPECT in sensitivity and degree of hyperperfusion.     

Acute myelopathy of unknown aetiology: a clinical,neurophysiological and MRI study of short-and long-term prognostic factors

Brain and spinal cord magnetic resonance imaging (MRI), multimodal evoked potentials (EPs) and cerebrospinal fluid (CSF) analysis were performed in 27 patients with acute myelopathy of unknown aetiology (AMUA), to detect the diagnostic and prognostic values of paraclinical tests at presentation. Spinal cord MRI was abnormal in 56% and brain MRI in 33% of the patients. Visual EPs were abnormal in 7%, median somatosensory EPs in 17%, tibial somatosensory EPs in 56% and motor EPs in 35% of the cases examined. Brain-stem acoustic EPs were normal in all the patients. CSF oligoclonal bands (OBs) were detected in 30% of cases. The patients were divided into subgroups according to the short-term clinical outcome (complete, partial or absent recovery). There were no significant differences among the three groups as regards MRI findings. Patients with complete recovery showed a significantly lower frequency of tibial somatosensory EP and motor EP abnormalities. According to the paraclinical findings at onset and on the basis of a long-term clinical follow-up (mean duration 24 months), 6 patients were diagnosed as having clinically definite multiple sclerosis, while 21 did not develop further neurological disturbances. Only the presence of CSF OBs was significantly more frequent in patients with definite multiple sclerosis. Our study indicates that EPs exploring spinal cord function are more powerful than spinal MRI for predicting the short-term outcome of AMUA, while the combined use of brain MRI and CSF OBs has the highest negative predictive value for the subsequent development of clinically definite multiple sclerosis.     

Brain Single Photon Emission Computed Tomography Imaging in Landau-Kleffner Syndrome

Summary: Five right–handed children with Landau-Kleffner syndrome (LKS) who had disease onset between the ages of 3 and 9 years were studied with EEG and single-photon emission computed tomography (SPECT) before and, in four cases, after 6 months of corticosteroid treatment. EEG findings included both focal and generalized spikes as well as spike-wave discharges with bilateral temporal predominance. These increased markedly during sleep in 1 child, and continuous spike-and-wave complexes appeared during slow-wave sleep in another patient. Neuropsychological testing demonstrated verbal auditory agnosia. Magnetic resonance imaging (MRI) was performed in 4 children and was normal. Brain SPECT imaging demonstrated abnormal perfusion in the left temporal lobe in all patients. The response to corticosteroid therapy was mixed. Our findings reinforce the concept that LKS is a functional disease affecting the language-dominant brain areas. We conclude that SPECT imaging may be of diagnostic assistance in the evaluation of this syndrome of unknown etiology.     

Multi‐modal brain imaging showing brain damage to the orbitofrontal cortex and left hemisphere,in a case of prolonged hypoglycemia‐induced transient hemiplegia followed by persistent encephalopathy

A 21‐year‐old left‐handed male patient was admitted with a 19‐h history of coma after substantial insulin injection for suicide attempt. Although the patient recovered from coma 3 days after injury, he experienced transient hemiplegia followed by permanent brain damage. Electroencephalogram (EEG), brain magnetic resonance imaging (MRI), and brain single‐photon emission computed tomography (SPECT) identified the localization of this dysfunction, but consistency between clinical symptoms and brain images changed depending on the course of treatment. Transient hemiplegia corresponded to abnormal waveforms on EEG and decreased cerebral blood flow on SPECT, whereas persistent dysfunctions corresponded to abnormal brain regions on MRI and SPECT.     

CORRELATIVE STUDY OF THE COGNITIVE IMPAIRMENT,REGIONAL CEREBRAL BLOOD FLOW,AND ELECTROENCEPHALOGRAM ABNORMALITIES IN CHILDREN WITH DOWN’S SYNDROME

The aim of this study was to investigate possible correlations of the cognitive impairment with abnormalities of regional cerebral blood flow and electroencephalogram in children with (Down's Syndrome) DS. Nine patients with DS were evaluated by single photon emission computed tomography (SPECT) in combination with clinical findings, electroencephalography (EEG), and magnetic resonance imaging (MRI). In cases with IQs below 40, there were one or more findings of abnormal EEG/MRI and brain perfusion SPECT. In 6 cases (66.7%) EEG findings were normal, but 3 (33.3%) had abnormal EEG findings. Perfusion abnormalities were most pronounced in the fronto-parieto-temporal region in the form of hypoperfusion (n = 5) and in the right hemisphere (n = 5) than the left hemisphere (n = 1). These findings suggest that the children with DS had varying levels of structural, perfusion, and electrophysiological abnormalities in the brain and these abnormalities were reflected by measurable alterations of the cognitive functions.     

长期应用抗癫癎药控制的颞叶癫癎患者发作间期SPECT异常灌注灶修复与长程视频脑电图变化的临床研究

目的：探讨抗癫癎药（AEDs）长期控制的颞叶癫癎患者发作期间应用单光子发射电子计算机断层扫描（SPECT）检查异常灌注灶的修复。方法：颞叶癫癎患者经AEDs、步长脑心通胶囊与尼莫地平治疗,并在两次发作间期行SPECT、长程视频脑电图（V-EEG）和CT/MRI检查。结果：44例癫癎患者平均年龄为25岁;平均病程为3.5年;44例患者中病因明确者为19例（家族史2例,围产期损伤1例,热性惊厥3例,颅脑损伤出血1例,颅脑外伤5例,脑膜脑炎3例,脑部手术2例,多病因2例）。癫癎发作控制平均为25个月。治疗前发作间期SPECT异常（颞叶和非颞叶）感兴趣区（ROI）为77.3%,长程V-EEG异常77.3%（其中癎样放电88.2%）,治疗前CT/MRI异常47.7%。治疗后发作间期SPECT正常增加4.5%（P〉0.05）,异常灶减少8.3%（P〉0.05）;长程V-EEG正常增加27.3%（P〈0.05）,癎样放电减少53.3%（P〈0.05）;结论：长期控制的颞叶癫癎患者的异常灌注灶修复明显低于其他类型癫癎,防治重点应放在颞叶癫癎症状出现之前的继发性全身强直阵挛发作。     

Focal cortical dysfunction and blood-brain barrier disruption in patients with Postconcussion syndrome.

Postconcussion syndrome (PCS) refers to symptoms and signs commonly occurring after mild head injury. The pathogenesis of PCS is unknown. The authors quantitatively analyzed EEG recordings, localized brain sources for abnormal activity, and correlated it with imaging studies. Data from 17 patients with neurologic symptomatology consistent with ICD-10 criteria for PCS was analyzed. Normalized quantitative EEG (QEEG) revealed significantly higher power in the delta band and lower power in the alpha band compared with matched controls. The generators for the abnormal rhythms were focally localized in neocortical regions. Brain computerized tomography and/or MRI did not reveal focal abnormality at the time of diagnosis. Single photon emission computed tomography (SPECT) after 99mTc-ethylcysteinate dimer administration showed a focal reduction in perfusion in 85% (n = 11) of the patients, and abnormal blood-brain barrier (BBB) after 99mTc-diethylenetriaminepentaacetic acid administration in 73% (n = 8). In 75% of these patients, low-resolution brain electromagnetic tomography analysis showed that the generators for abnormal rhythms were closely related to the anatomic location of the BBB lesion. These data point to focal cortical dysfunction in conjunction with BBB disruption and hypoperfusion as a possible mechanism of pathogenesis in at least some PCS patients, and offer QEEG and SPECT as important tools in evaluating these patients.     

Short interval change of 99mTc-ethyl cysteinate dimer single photon emission computed tomography in Wilson's disease]

We studied short interval change of cranial computed tomography (CT), magnetic resonance imaging (MRI) and 99mTc-ethyl cysteinate dimer single photon emission computed tomography (99mTc-ECD SPECT) in a case of Wilson's disease. Before treatment, CT scan showed low density changes in the bilateral thalamus and basal ganglia, and MRI demonstrated high intensity in same lesions. 99mTc-ECD SPECT study revealed a hypoperfusion in bilateral thalamus. After 2 months under D-penicillamine therapy, neurological findings had improvement. Hypoperfusion in the thalamus with 99mTc-ECD SPECT significantly improved, whereas abnormal findings of CT scan and MRI persisted. 99mTc-ECD SPECT study may be useful for the planning of the treatment of Wilson's disease.     

Chronological changes in brain MRI, SPECT, and EEG in neurosarcoidosis with stroke-like episodes

Abstract  Neurosarcoidosis with recurrent stroke-like episodes has been reported only rarely in the literature. We repeatedly measured brain magnetic resonance images (MRI), SPECT, and electroencephalograms (EEG) in a 32-year-old male patient with this condition. In the acute stage of the episodes, he showed semi-coma followed by severe dysphasia; left-frontal EEG delta wave activities and left-sided hyperperfusion SPECT were noted. Gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA) MRI findings were aggravated diffusely in the convalescent stage of the disease rather than in the acute stage. The EEG and SPECT findings were clearly reflective of an early stage of acute encephalomeningitis caused by the neurosarcoidosis, and thus are more useful than MRI for evaluation of the acute inflammatory process in this condition.     

肝型和无症状型肝豆状核变性颅脑磁共振分析

目的观察肝型和无症状型的肝豆状核变性颅脑MRI表现。方法对我院2014年5月-2016年5月收治的97例肝型和28例无症状型WD患者,均行头部MRI检查。结果 125例患者中颅脑磁共振检查发现有108例患者(87.8%)有脑部异常信号样改变,其中肝型83例,无症状型25例;83例有脑萎缩改变,肝型63例,无症状型20例。结论肝型和无症状型肝豆状核变性患者颅脑MRI异常信号比例较高,主要集中在壳核;信号改变主要为对称性长T_1和长T_2,未发现有混杂样信号改变;病程与脑干和壳核相关,发病年龄与壳核、苍白球、尾状核密切相关;Child-Pugh分级与颅脑磁共振改变没有明显相关性。     

Brain perfusion SPECT and EEG findings in children with autism spectrum disorders and medically intractable epilepsy

Objective: We performed brain perfusion single-photon emission computed tomography (SPECT) to detect the abnormal brain region in children with both autism spectrum disorders (ASD) and medically intractable epilepsy. Methods: Fifteen children aged 4–16 years underwent multimodal examinations (MRI, interictal and/or ictal ECD-SPECT, EEG and MEG) to investigate their indications for surgical treatment. All children were diagnosed with ASD according to DSM-IV criteria and intractable epilepsy. Despite medical treatment for more than a year, all experienced at least one seizure per month. All had no underlying basic disorders. Each SPECT result was statistically analyzed by comparing with standard SPECT images obtained from our institute (easy Z-score imaging system; eZIS). The relationship between the eZIS pattern and EEG abnormalities or clinical symptoms was investigated. Results: All children showed focal abnormal patterns on eZIS and focal spikes on EEG. In all children, eZIS revealed a mixed hypoperfusion pattern, especially in the prefrontal cortex, medial frontal cortex, anterior cingulate cortex, medial parietal cortex, and/or anterior temporal cortex. In seven of 12 children who underwent interictal SPECT studies, areas of hypoperfusion were related to the focus observed on EEG; in six children, the focal EEG spikes represented areas of hyperperfusion. The children were divided into two groups according to the main type of hypoperfusion patterns seen on eZIS; medial-cingulate type and temporal type. No significant relationship was observed between the areas of hypoperfusion and clinical symptoms. eZIS showed the epileptic focus clearly on ictal SPECT. Conclusions: SPECT was useful to detect the abnormal brain region not only in searching for the epileptic focus but also in assessing the low or high functioning region of the brain.     

Brain SPECT, CT and EEG in 45 cases of epilepsy during the intermission

The regional cerebral blood flows (rCBF) in 45 cases of epilepsy during the interictal period were determined with brain SPECT. The results were compared those obtained with CT scans and EEG. 48.89% of the SPECT were found to be abnormal while 8.98% were suspected to be abnormal; the 35.71% of the CT scans were found to be abnormal; 16 cases (39.02%) were found to show focal abnormalities in the EEG while 9 cases (21.95%) had epileptic form discharge. SPECT seemed to show more significance in discovering the abnormalities in the epileptics during the interictal period than CT or EEG. However, the combined use of these three methods of examinations would be of greater help for identifying the focal abnormalities in epilepsy. Two of the 22 cases with abnormal SPECT had increased rCBF, whereas the other 20 cases had decreased rCBF. Among the 22 cases of abnormal SPECT and 4 cases of suspected abnormalities, the locations of the lesions as indicated with SPECT in 3 cases were not consistent with those as with CT. There were 5 cases in which the SPECT findings were not consistent with those in EEG.     

Voxel-Based Mapping of Cortical Ischemic Damage Using Tc 99M L, L-Ethyl Cysteinate Dimer Spect in Acute Stroke

BACKGROUND AND PURPOSE: When performed soon after stroke onset, single-photon emission computed tomography (SPFCT) with hexamethylpropylenamine oxime or Tc 99m L,L-ethyl cysteinate dimer (ECD) has significant added predictive value compared to neurological scores. With ECD SPECT, the degree of tracer uptake reduction predicts neurological recovery, and using a 40% threshold to characterize irreversibly damaged tissue (IDT), significant correlations have been observed. However, correlations between this uptake threshold and tissue outcomes have not been assessed. The purpose of this study was to validate the 40% ECD uptake threshold for the probabilistic mapping of IDT using an automatic, voxel-based approach. METHODS: In 10 acute stroke patients, the authors first compared early ECD SPECT and late coregistered magnetic resonance imaging (MRI) data and assessed for IDT and "tissue at risk" (i.e., tissue with ECD uptake below and above 40%) the percentage of voxels ultimately infarcted and noninfarcted on late brain MRI. They then assessed the correlations between the volumes of brain tissue compartments and subsequent neurological recovery. Finally, to assess whether visual SPECT analysis is reliable compared to the more complex voxel-based approach, the authors compared the predictive value of the 2 methods for neurological recovery. RESULTS: The majority of IDT voxels (average = 84%), defined by ECD uptake < 40%, evolved toward infarction, and 51.8% to 100% of at-risk voxels (average = 89%) escaped infarction. The extent of IDT correlated significantly with neurological recovery (P = .0009). There was good agreement between visual and voxel-based analyses (P = .0004). CONCLUSION: The results support the validity of the ECD uptake thresholds chosen, suggesting that ECD uptake can reflect neuronal viability and that ECD SPECT can be useful for the early detection of potentially salvageable tissue and irreversible damage. These preliminary results encourage the use of this method in a clinical setting for fast decision making in choosing acute therapy.