Sneddon syndrome presenting with hemicranic attacks: a case report

The case of a young woman suffering from a rare cerebrovascular disease associated with livedo reticularis (Sneddon syndrome) is reported. Hemicranic attacks were the first symptom detected. The patient had a progressive clinical course of neurologic symptoms. Cerebral CT scan, NMR and cerebral arteriography revealed a progressive cerebral multifarctual feature involving middle-size arteries.     

Cerebral ischemia and livedo reticularis in a patient with impairment of coagulation factor VII and free protein S

The association of ischemic cerebrovascular lesions with livedo reticularis is known as Sneddon syndrome. It affects young subjects, primarily women, and its neurological manifestations are TIAs, ischemic stroke, progressive dementia and epileptic seizures. Its etiopathogenesis has still to be clarified. Some authors have associated it with an antiphospholipid antibody syndrome. Recently it has been assumed that a defect in blood coagulation may be involved in its pathogenesis. Here we report a case in which both an increase in coagulation factor VII activity and a deficiency in free protein S were documented.

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Sommario L'associazione di lesioni cerebrovascolari ischemiche e livedo reticularis è conosciuta come sindrome di Sneddon. La sua eziopatogenesi non è ancora stata chiarita. Noi descriviamo un caso in cui sono stati documentati un aumento dell'attività del fattore VII della coagulazione ed una carenza della forma libera della proteina S.

     

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Livedo is a net‐like violaceous skin pattern. It can be classified as physiological or pathological. The physiological livedo reticularis usually appears in cold conditions, whereas the pathological and irregular livedo, which persists in warm temperatures, is often labeled as ‘livedo racemosa’. Some neurological pathologies are associated with livedo, most commonly those with an inflammatory component or those derived from systemic disorders. The present review summarizes the most important central and peripheral neurological diseases in pediatric and adult age groups associated with livedo, providing physicians with an overview of the clinical presentation, etiology, diagnosis and management of these conditions.     

Impact of blood pressure changes in cerebral blood perfusion of patients with ischemic Moyamoya disease evaluated by SPECT

Our aim was to determine the impact of targeted blood pressure modifications on cerebral blood flow in ischemic moyamoya disease patients assessed by single-photon emission computed tomography (SPECT). From March to September 2018, we prospectively collected data of 154 moyamoya disease patients and selected 40 patients with ischemic moyamoya disease. All patients underwent in-hospital blood pressure monitoring to determine the mean arterial pressure baseline values. The study cohort was subdivided into two subgroups: (1) Group A or relative high blood pressure (RHBP) with an induced mean arterial pressure 10–20% higher than baseline and (2) Group B or relative low blood pressure (RLBP) including patients with mean arterial pressure 10–20% lower than baseline. All patients underwent initial SPECT study on admission-day, and on the following day, every subgroup underwent a second SPECT study under their respective targeted blood pressure values. In general, RHBP patients showed an increment in perfusion of 10.13% (SD 2.94%), whereas RLBP patients showed a reduction of perfusion of 12.19% (SD 2.68%). Cerebral blood flow of moyamoya disease patients is susceptible to small blood pressure changes, and cerebral autoregulation might be affected due to short dynamic blood pressure modifications.     

Acute effect of electroconvulsive therapy on brain perfusion assessed by Tc99m-hexamethylpropyleneamineoxim and single photon emission computed tomography

Regional cerebral blood flow (rCBF) was measured in relative terms with Tc99m-hexamethylpropyleneamineoxim and single photon emission computed tomography in 11 patients undergoing electroconvulsive therapy (ECT). The patients suffered from major depressive disorder (n = 8) or schizoaffective disorders (n = 3). rCBF was measured under general anesthesia 3 days prior to the ECT treatment and coinciding with the ECT stimulation. ECT caused a redistribution of the tracers uptake. The uptake became more pronounced in frontal parts of the brain and in the basal ganglia than in posterior parts of the cortex, and the thalamus. This selective effect of ECT on rCBF may be related to catecholaminergic projections to anterior parts of the brain.     

Cortical circumferential profile of SPECT cerebral perfusion in Alzheimer''s disease

We developed a semiautomatic method termed “cortical circumferential profiling” for objective analysis of cerebral cortex function in emission tomographic neuroimaging studies. This method treats cortex as a continuous ring near the outer brain edge. A computer algorithm samples the cortex at 60 contiguous, equiangular locations, using 1-cm² samples. These values are plotted as a function of cortical angle to produce the cortical circumferential profile. This method was used in a study of regional cerebral perfusion in 15 patients with Alzheimer's disease and 8 elderly control subjects using N-isopropyl [I-123]-iodoamphetamine. Cortical circumferential profiling decreases variability, examines the entire cortex within slices at preselected levels above the orbital-meatal line, and facilitates intrasubject and intersubject comparisons.     

Mapping of SPECT regional cerebral perfusion abnormalities in obsessive-compulsive disorder

Locations of cerebral perfusion abnormalities in obsessive-compulsive disorder (OCD) were mapped with single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI). This report is a new, more thorough analysis of a previous study of these subjects that used region-of-interest methods. Ten obsessive-compulsive patients and seven age- and sex-matched control subjects were studied. Image sets were converted into stereotaxic space, normalized to each subject's mean cerebral value, then group averaged. Difference images were calculated and searched for regions with significant between-group cerebral perfusion differences. Obsessive-compulsive patients had significantly higher relative cerebral perfusion in medial-frontal and right frontal cortex and in cerebellum, and significantly reduced perfusion in right visual association cortex. Increased frontal Perfusion agrees with several prior reports. The caudate nucleus, which has been controversial in neuroimaging studies of OCD, did not display a difference between groups. The results of this study provide information about the locations and extents of cerebral perfusion abnormalities in OCD. Regional abnormalities were compared with those reported in prior functional neuroimaging studies. Issues related to OCD hyperfrontality and frontal lateralization of psychopathology are discussed. Normal caudate nucleus findings are considered in relation to prior functional imaging studies and hypotheses of OCD pathology. © 1994 Wiley-Liss, Inc.     

Cerebral perfusion inhomogeneities in schizophrenia demonstrated with single photon emission computed tomography and Tc99m-hexamethylpropyleneamineoxim

Cerebral blood flow was measured in relative terms with Tc99m-hexamethylpropyleneamineoxim (HMPAO) and single photon emission computed tomography (SPECT) in 28 female schizophrenic patients (20 acute and 8 chronic) classified according to DSM-III. Eleven normals served as controls. The acute patients were classified according to positive and negative symptoms. Patients with predominantly positive symptoms showed by and large normal and homogeneous cerebral isotope uptake. Those with negative symptoms, and the chronic patients, showed inhomogeneous tracer uptake with multiple regions of hypoperfusion in slices 4-6 cm above the orbitomeatal line. The findings support in principle the notion that schizophrenia with negative or chronic symptoms does not affect the whole brain homogeneously. Brain imaging with Tc99m-HMPAO and SPECT might be used to distinguish various types of schizophrenia.     

强迫症患者^99m锝-双半胱乙酯脑显像特点及临床相关研究

目的探讨强迫症患者的脑灌注特点,分析其与认知损害及临床特点间的关系。方法对39例首发且未经治疗的强迫症患者行单光子发射计算机断层扫描（SPECT）脑显像、威斯康星（WCST）认知功能测查及耶鲁-布朗强迫量表（Y—BOCS）评定临床特点。结果（1）强迫症组与正常对照组比较,强迫症组的双侧前额叶、前颞叶的RAR值分别为（94．4±6．1,99．8±6．4,91．6±6．1,92．8±6．6）,高于正常对照组（88．3±7．1,86．9±7．9,84．0±4-7．2,84．4±7．3）,差异有统计学意义（t=6．78,11．93,7．15,9．40;P值均〈0．05）。（2）脑灌注与威斯康星结果的相关分析：右前额叶血流灌注与正确数成负相关（r=0．783,P=0．014）,右前颞叶血流灌注与错误数成正相关（r=0．384,P=0．047）,右前额叶、左丘脑血流灌注与持续错误数成正相关（r=0．511,0．610;P=0．041,0．049）。（3）强迫症组双侧前额叶、右侧前颞叶的血流灌注与强迫思维成正相关（r=0．617,0．309,-0．358;P值均〈0．05）。结论强迫症患者双侧前额叶、前颞叶的灌注存在明显增高现象。强迫症患者的右前额叶、左丘脑血流灌注异常与认知功能损害相关。强迫症患者的双侧前额叶、右侧前颞叶灌注与强迫思维程度成正相关,提示前额叶、右前颞叶的功能异常可能是强迫思维产生的脑功能基础。     

Cerebral activity modulation by extradural motor cortex stimulation in Parkinson's disease: a perfusion SPECT study

Extradural motor cortex stimulation (EMCS) has been proposed as alternative to deep brain stimulation (DBS) in the treatment of Parkinson's disease (PD). Its mechanisms of action are still unclear. Neuroimaging evidenced motor cortical dysfunction in PD that can be reversed by therapy. We performed left hemisphere EMCS surgery in six advanced PD patients fulfilling CAPSIT criteria for DBS with the exception of age >70 years. After 6 months, we measured regional cerebral blood flow (rCBF) at rest with SPECT and Tc-99m cysteinate dimer bicisate off-medication with stimulator off and on. Clinical assessment included Unified Parkinson's Disease Rating Scale part II and III, Abnormal Involuntary Movement Scale and mean dopaminergic medication dosage. We used statistical parametric mapping for imaging data analysis. Clinically we observed no mean changes in motor scales, although blinded evaluation revealed some benefit in individual patients. We found significant rCBF decrements in the pre-central gyrus, pre-motor cortex and caudate nucleus bilaterally, left prefrontal areas and right thalamus. Perfusion increments were found in cerebellum bilaterally. EMCS determined significant modulation of neuronal activity within the cortico-basal ganglia-thalamo-cortical motor loop in our cohort of advanced PD patients. However, these effects were paralleled by mild and variable clinical efficacy.     

Sneddon's syndrome (livedo reticularis and cerebrovascular lesions) with antiphospholipid antibodies and severe dementia in a young man: a case report

We present the case of 37-year-old man with Sneddon's syndrome and antiphospholipid antibodies. His chief neurological manifestation was rapidly progressive dementia, which developed 6 years after the appearance of livedo reticularis.     

Tc-99 HMPAO SPECT study of regional cerebral blood flow in olanzapine-treated schizophrenic patients

Dopamine D₂ blocking typical antipsychotic drugs are known to change the cerebral perfusion patterns of schizophrenic patients, especially in the frontal cortex and basal ganglia. In recent years atypical antipsychotics such as olanzapine, which have high serotonin 5-HT_2A/dopamine D₂ occupation ratios, have been shown to be more effective in the treatment of schizophrenia symptoms. The aim of this study was to evaluate the regional cerebral blood flow (rCBF) of the schizophrenic patients treated with olanzapine in a within-subject design. Twenty-four patients with schizophrenia participated as subjects in the study. Each subject was scanned in a medication-free state and after 6 weeks of 10 mg/day fixed dose olanzapine treatment. Despite the clinical improvement seen in the patients, repeated-measures analysis of variance showed that olanzapine produced no significant changes in cortical rCBF after the six-week treatment. This finding indicates that unlike typical antipsychotics olanzapine has no negative effect on cortical cerebral perfusion patterns of schizophrenic patients. Received: 12 December 2001 / Accepted: 5 December 2002 RID="*" ID="*"The preliminary report of this paper had been presented at 12^th European College of Neuropsychopharmacology Congress in London, 1999 and awarded by poster award. This work had been carried out at Erciyes University School of Medicine, departments of psychiatry and nuclear medicine. Correspondence to Ali Saffet Gonul, MD     

Utility of Tc 99m HMPAO SPECT in the early diagnosis of Rasmussen’s syndrome

Neuroradiological examinations are important in the diagnosis of Rasmussen’s syndrome (RS), but they frequently result normal in the early phase of the disease. We performed Tc 99m HMPAO SPECT (single photon emission computed tomography) on an 11-year, 5-month-old, right-handed girl, two months since the first seizure. SPECT showed a reduced uptake of HMPAO in the left temporal region despite normal results with magnetic resonance imaging (MRI). After six months, she developed epilepsia partialis continua, neurological signs appeared, and MRI showed cortical atrophy on the left side. SPECT seems to be a useful, inexpensive and diffuse technology able to give information in the early phases of RS useful in the prevention of serious consequences of the disease within a medical therapeutic program (ganciclovir or intravenous immunoglobulins).

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Sommario è noto che le indagini neuroradiologiche sono importanti nella diagnosi della syndrome di Rasmussen (RS), ma è altrettanto veto che esse risultano spesso normali nelle fasi precoci della malattia. Descriviamo il caso di una paziente di undici anni e cinque mesi, affetta da RS, che è stata sottoposta a Tc 99m HMPAO SPECT due mesi dopo la prima crisi. La SPECT ha mostrato una riduzione della perfusione che interessava il lobo temporale sn, mentre la risonanza magnetica (RMN) risultana normale. Sei mesi più tardi la paziente sviluppava un’epilessia parziale continua e la RMN mostrava una atrofia corticale emisferica sn. Pensiamo che la SPECT sia un esame utile, economico e di largo impiego in grado di fornire ulteriori informazioni, rispetto a quelle già descritte, sulle fasi iniziali della RS. L’individuazione precoce di una RS permette infatti di instaurare una terapia medica (ganciclovir o immunoglobuline) prima della comparsa delle gravi conseguenze della malattia.

     

Regional cerebral blood flow measured by99mTc-HMPAO SPECT differs in subgroups of Alzheimer's disease

Summary Alzheimer's disease (AD) is a heterogeneous entity. Identifying AD subtypes might have impact in patients' response to different treatment strategies. We designed a study to examine regional cerebral blood flow (rCBF) in AD subtypes. To identify AD subtypes, we performed a cluster analysis including performance on memory, language, visuospatial, praxis, and executive functions. The rCBF measured by^99mTc-HMPAO SPECT was referred to the cerebellum. We examined 35 patients fulfilling the NINCDS-ADRDA criteria of probable AD and 13 age and sex-matched healthy cognitively intact controls. AD patients were at the early stage of the disease, their mean Mini-Mental Status (MMS) score (S.D.) was 22.5 (3.6). The cluster analysis revealed two AD subgroups: AD1 (N=12) and AD2 (N=23). The subgroups did not differ in age, sex, or global clinical severity as assessed by MMS and Brief Cognitive Rating Scale (BCRS). Both subgroups had equally impaired memory. The AD2 group was inferior to the AD1 group on verbal, visuospatial, praxic, and executive functions. The AD1 group showed reduced rCBF ratios in the temporal and parietal cortices and the amygdala compared to controls. The AD2 group differed from controls in the rCBF ratios of frontal, temporal, parietal, occipital, basal ganglia, and amygdaloid regions bilateral and from AD1 in the rCBF ratios of frontal and temporal cortices. In AD patients, the rCBF ratios did not correlate with MMS or BCRS scores. In contrast, several significant correlations were found between decreases rCBF ratios and impairment of memory and other cognitive functions. In conclusion, a cluster analysis on neuropsychological test performance identified two AD subgroups that differed on the neuropsychological profile and on the rCBF in spite of similar global clinical severity.     

Regional cerebral distribution of [Tc-99m] hexylmethylpropylene amineoxine in patients with progressive aphasia.

Progressive aphasia is a prominent clinical feature of several neurodegenerative disorders. This study used hexylmethylpropylene amineoxine (HMPAO) single photon emission computed tomography (SPECT) to estimate blood flow in areas of the brain that mediate language in patients with progressive aphasia and matched control subjects. The patient population consisted of four men and 12 women with a mean +/- SD age of 69.1 +/- 7.6. Of these, eight were classified as having a nonfluent form of aphasia, whereas the other eight had a fluent form. The patients were compared to 16 healthy volunteers who were studied with an identical protocol. The SPECT images of the brain were acquired with 740 MBq (20 mCi) of Tc-99m-labeled HMPAO on a triple-headed gamma camera equipped with fan beam collimators. The images were analyzed with a set of standardized templates. Mean counts per pixel in 33 regions of interest were compared to the mean counts in the whole supratentorial brain. A laterality index was determined for homotopic regions using the equation 100 x (R - L)/(1/2 x (R - L)). Patients with progressive aphasia had several regions of significantly decreased HMPAO uptake in the left cortex when compared to the homotopic regions on the right. The most prominent deficit in the nonfluent group, as determined by the laterality index, were found in the left dorsolateral prefrontal region (p < 0.05), whereas the most prominent deficits in the group with fluent aphasia were found in the left temporal and parietal language centers (p < 0.05). The left subcortical nuclei were differentially affected, particularly in patients with nonfluent aphasia. The HMPAO SPECT indicates that multiple regions of the left hemisphere are dysfunctional in patients with progressive aphasia. The pattern of perfusion deficits in patients with fluent aphasia appears to be distinct from the pattern in patients with nonfluent aphasia.     

Sneddon Syndrome: A Comprehensive Overview

Sneddon syndrome (SS) is an episodic or chronic, slowly progressive disorder and characterized by generalized livedo racemosa (patchy, violaceous, skin discoloration) and recurrent cerebrovascular events. The histopathology of skin and brain is remarkable for a noninflammatory thrombotic vasculopathy involving medium- and small-sized dermal and cerebral arteries, respectively. Approximately 80% of the SS patients are women with a median age of diagnosis at 40 years. However, the onset of the disease during childhood have been reported. Etiopathogenesis of SS is unknown with 2 primary mechanisms proposed – autoimmune/inflammatory versus thrombophilia. SS is primarily classified as antiphospholipid positive or negative type. Neurological manifestations usually occur in 3 phases: (1) prodromal symptoms such as headaches, dizziness, and vertigo, (2) recurrent strokes, and (3) early onset dementia. Livedo racemosa precedes the onset of recurrent strokes by more than 10 years, but in many instances, the significance of the skin lesion is recognized only after the appearance of the stroke. The involvement of the heart valves, systolic labile hypertension, and retinal changes are also commonly associated with this syndrome. Treatment of SS is primarily based on anecdotal reports. Antiplatelet and antithrombotic agents are used for secondary stroke prophylaxis, and a recent study showed a relatively lower stroke recurrence rate with the universal use of antiplatelet/antithrombotic agents. Routine use of anti-inflammatory or immunosuppressive therapies is controversial. Neuropsychiatric prognosis of SS is relatively poor with predominant deficits in the concentration, attention, visual perception, and visuospatial skills.     

缺血性脑血管疾病的乙酰唑胺脑负荷试验研究

目的　建立乙酰唑胺 (ACZ)脑负荷试验的方法及脑血流量变化的正常参考值 ,提出评价脑血管储备功能的指标。方法　正常对照组 6例 ,脑血管疾病患者 43例。用 99m Tc-乙撑双半胱氨酸二乙酯 (ECD)先做基态脑血流 (CBF)及 SPECT显像 ,3 6～ 48h后做脑负荷显像 (静脉注射 ACZ 1g,2 0 m in后按基态同样条件进行 )。患者均行 CT检查 ,部分行 MRI、TCD、DSA检查。 7例患者治疗后进行了随访并复查 SPECT显像。图像分析 :(1)视觉分析 :比较 ACZ试验前后的变化 ,分 3型 :A型 ,反应差 ,ACZ试验后出现病变 (A1 )或病变扩大 (A2 ) ;B型 ,反应好 ,病变缩小或消失 ;C型 ,无反应。 (2 )半定量分析 :计算颈动脉及大脑半球峰时、半球血流占全脑血流百分数 ;计算 ACZ试验前后病变区及病侧半球血流量增加率 (△ % )及放射性摄取比值 (UR)。结果　 (1)正常对照组两侧比较各值无差别。 ACZ试验后全脑△ %为 2 5 .0 %± 8.0 9% ,ACZ试验前后各区 UR均 >0 .90 1。 (2 ) 3 2例短暂性脑缺血发作(TIA)患者脑 CBF显像 42 %有闭塞性脑血管病变 ,与 TCD、DSA结果相符。 (3 ) ACZ试验提高了 TIA和小梗死灶的阳性率 ,前者由 5 9.3 7%升至 87.15 % ,后者由 73 %升至 90 %。 (4) A型患者疗效好 ,B型疗效差。 (5 )小梗死伴有对侧小脑或同侧丘     

急性脑血管病并发脑性盐耗综合征36例

脑性盐耗综合征（cerebral salt wasting syndrome，CSWS）是指颅内疾病导致肾脏钠及水排出量增多，临床表现为低钠血症、尿钠尿量增多及低血容量的一组综合征。以往文献报道CSWS多发生于颅脑损伤、颅内肿瘤及其术后。为探讨CSWS在急性脑血管病中的发生、发展规律及影响因素，本文对近年来我院收治的各种急性脑血管病518例进行顺序跟踪观察，其中36例并发CSWS，报道如下。     

Cerebral blood flow velocity and perfusion in purulent meningitis: a comparative TCD and 99MTC-HMPAO-SPECT study

In 15 patients (median age 33 years; range 17–74 years) suffering from acute pneumococcal (10 cases) and meningococcal (five cases) meningitis, cerebral blood flow velocity (CBFV) was measured in the M₁– segment of the middle cerebral artery (MCA) by transcranial Doppler sonography, and cerebral perfusion changes were evaluated by ^99m-Tchexamethylpropylene amine oxime single photon emission computed tomography (HMPAO SPECT). The objective of the study was to test whether increased CBFV during the acute phase of purulent meningitis reflects hyperemia, and to evaluate focal perfusion abnormalities and their correlation to CBFV changes.
In eight patients with marked side-differences in CBFVs during the acute phase of the disease SPECT scans were normal in five. In three patients unilateral perfusion defects correlated with the side of higher CBFV. In seven patients presenting with symmetrically elevated CBFV, SPECT scans were normal in four and revealed focal abnormalities in the remaining three. Follow up SPECT scans were normal in 14/15 patients.
The results of our study suggest that elevated CBFV in acute bacterial meningitis does not reflect cerebral hyperemia. Focal cerebral perfusion defects occur independently from functional alterations in the cerebral macrovasculature. A causative pathophysiologic relationship of high CBFV and focal perfusion defects cannot be drawn from these data.     

阿尔茨海默病的脑血流灌注研究

目的探讨AD患者脑血流灌注的特征性变化。方法选择20例确诊AD患者和10例认知正常者,对所有入组对象行SPECT脑血流灌注显像检查,对所得图像进行视觉分析和半定量分析,比较两组对象各脑区血流灌注情况的差异,并比较AD患者左、右脑叶脑血流灌注的差异。结果 (1)视觉分析结果显示,AD组以颞叶或顶叶脑血流灌注减低为主,各占55%,其中双侧颞顶叶血流灌注减低者占15%,单侧颞顶叶血流灌注减低占20%。正常认知组SPECT影像学表现各异,额叶、颞叶、顶叶、枕叶、丘脑、基底节血流灌注减少情况均存在,各占10%。但无双侧颞顶叶血流灌注减少者,40%表现完全正常。(2)半定量分析结果显示,AD组在右额叶、双侧颞顶叶、右枕叶血流灌注显著低于正常认知组(P<0.05),以右侧颞叶血流灌注降低最明显,左右颞顶叶血流低灌注程度对比无显著性差异。结论 AD患者的SPECT特征性表现为双侧对称性颞顶叶血流低灌注,其中右侧颞叶血流灌注下降最严重。