Carbon disulfide encephalopathy: cerebral microangiopathy

To understand cerebral blood circulation after long-term exposure to carbon disulfide (CS2), four patients with encephalopathy and polyneuropathy, who had worked in a viscose rayon plant, were studied. Clinical and laboratory examinations, including brain magnetic resonance images (MRI), computed tomography (CT), CT perfusion, and CT angiography, were carried out. Brain CT and MRI disclosed mild cortical atrophy in all four patients, and multiple lesions in the subcortical white matter, and basal ganglia in three patients. Brain CT angiography and perfusion revealed a statistically significant decrease of cerebral blood flow (CBF) in the total brain parenchyma and basal ganglia, and a decrease of the cerebral blood volume (CBV) in the basal ganglia and a prolonged mean transit time (MTT) in the total brain parenchyma, and the territories of the internal carotid artery (ICA), basal ganglia and occipital lobe. In conclusion, the decrease of CBV and CBF, and the prolonged MTT in the total brain parenchyma, ICA, basal ganglia and occipital lobes, indicated a microangiopathy in patients with CS2 encephalopathy.     

Case of juvenile stroke caused by methylenetetrahydrofolate reductase deficiency

The patient had suffered from left hemiparesis at the age of thirteen months, and acute ischemic stroke of unknown etiology had been diagnosed at that time. His hemiparesis gradually disappeared and he was discharged two weeks after the onset without disability. At the age of 17 years, MRI following minor head trauma revealed cerebral infarctions located at the right corona radiata and basal ganglia. Laboratory findings showed hyperhomocysteinemia. Genetic study disclosed methylenetetrahydrofolate reductase deficiency (MTHFRD) (valine/valine type). MTHFRD is not detected by the routine infantile mass screening test for congenital amino acid metabolic disease, and should be considered in any patient with ischemic stroke at under two years of age.     

Dopamine transporter binding study in differentiating carbon disulfide induced parkinsonism from idiopathic parkinsonism

Long-term exposure to carbon disulfide (CS(2)) may induce parkinsonian features. There may be confusion in distinguishing between CS(2) parkinsonism and idiopathic parkinsonism, especially for workers who developed parkinsonian features in viscose rayon plants. We performed clinical examinations, and laboratory studies including magnetic resonance imaging (MRI) and dopamine transporter (DAT) studies with (99m)Tc-TRODAT-1 brain single photon emission computed tomography (SPECT) in three workers who had long-term exposure to CS(2). Patient 1 had polyneuropathy, and encephalopathy with tremor; patient 2 had polyneuropathy, and encephalopathy with parkinsonian features; and patient 3 had pure parkinsonian features without polyneuropathy or cerebellar signs. The treatment with l-dopa was effective in patient 3, but non-effective in patient 2. Brain MRI revealed multiple high signal intensities over the subcortical white matter, basal ganglia, and/or even the brainstem in patients 1 and 2, but normal in patient 3. In DAT studies, the bindings were normal in patients 1 and 2 and was decreased in patient 3. We conclude that CS(2) exposure may induce polyneuropathy, and cerebellar dysfunction in addition to parkinsonian features and that brain MRI may show multiple lesions in the cerebral white matter and basal ganglia. In addition, DAT with (99m)Tc-TRODAT-1 brain SPECT may provide a useful information in differential diagnosis between CS(2) parkinsonism and idiopathic parkinsonism.     

Clinical recovery from stroke lesions and related outcomes

Stroke lesions affect neurological status and are a critical determinant of treatment. This study investigated stroke lesions in terms of clinical recovery and related outcomes. Twenty-seven stroke patients were assessed via longitudinal observational study. Brain lesions were evaluated using MRI. The Fugl-Meyer Assessment and clinical evaluations were performed monthly between 1 and 6 months after onset. The anterior limb (ICAL) and genu (ICG) of the internal capsule were associated with recovery of the upper limbs in chronic stroke patients. Involvement of the anterior half of the middle third of the corona radiata, the ICAL, the ICG, and the caudate nucleus were related to recovery of the lower limbs. Involvement of the middle third of the corona radiata, the ICAL, the ICG, and the lentiform nucleus were associated with sensory recovery. Clinical recovery from stroke, in terms of motor and sensory function, was related to injury in several white matter areas, such as the corona radiata and internal capsule, and was also associated with the basal ganglia as a gait pattern generator. Clinicians should be aware of stroke lesions, and should design therapeutic strategies accordingly, also with respect to treatment duration.     

Magnetic resonance imaging of ataxic hemiparesis localized to the corona radiata

A 61-year-old woman developed right hemiparesis with homolateral cerebellar-type ataxia. Computed tomography and magnetic resonance imaging demonstrated left corona radiata lesions, not present on magnetic resonance imaging 1 year earlier. No brainstem lesions were identified, suggesting that ataxic hemiparesis can result from lesions in the corona radiata.     

Early ischemic lesion demonstrated by ictal diffusion-weighted MRI during prior TIA in a patient with cerebral infarction]

We reported a patient with transient ischemic attack (TIA), subsequently evolving to a cerebral infarction, in whom ictal diffusion-weighted magnetic resonance imaging (MRI) detected early ischemic lesion in the left hemisphere. The patient was a 30-year-old right-handed male medical doctor, who had an in-hospital episode of TIA with obtundation and right hemiparesis, which lasted for 150 minutes. Ictal diffusion-weighted MRI obtained 110 minutes after symptom onset demonstrated an area of high signal intensity in the left striatum and corona radiata, whereas T 2-weighted and FLAIR images were entirely normal. Ictal magnetic resonance angiography (MRA) showed occlusive lesions in the M 2 branches of the left middle cerebral artery. The second MRA obtained 90 minutes after resolution of the symptoms showed nearly complete recanalization of the left middle cerebral artery, suggesting that the TIA was embolic mechanism. However the patient rapidly developed similar neurological symptoms again 58.5 hours after the TIA episode, evolving finally to a completed stroke. A brain CT obtained 1 hour after the second episode demonstrated diffuse hypodense lesions in the left basal ganglia and corona radiata, and in the left temporal lobe. MRIs 3 and 7 days later displayed completed infarcts, of which distribution was consistent with that of the hypodense lesions on the earlier CT. The left middle cerebral artery remained patent on the follow-up MRAs. The patient fairly recovered and returned to his premorbid position as medical doctor with a mild residual right hand clumsiness. In this patient, ictal and post-ictal MRAs documented an occlusion and a reopening of the middle cerebral artery. The embolic mechanism remains unknown despite detailed cardiac, vascular, and hematological examinations. In addition to recurrent embolism, we would like to point out that the reperfusion injury, secondary delayed neuronal death, and other factors may be involved in the second exacerbation evolving to the completed stroke.     

Clinical lacunar syndromes as predictors of lacunar infarcts. A comparison of acute clinical lacunar syndromes and findings on diffusion-weighted MRI

OBJECTIVES: To evaluate if patients with acute lacunar syndromes have acute lacunar infarcts or other types of cerebral lesions on diffusion-weighted MRI. METHODS: Patients with acute lacunar syndromes underwent echo-planar diffusion MRI of the brain within 3 days after stroke onset. Localization and size of lesions with hyperintense signal were determined, compared with clinical characteristics and with findings on follow-up T2-weighted MRI. RESULTS: Twenty-three patients participated in the study. Thirteen patients had pure motor stroke, 1 pure sensory stroke, 8 sensorimotor stroke, and 1 ataxic hemiparesis. Twenty-two patients had at least one lesion with increased signal on diffusion-weighted MR images. These acute lesions were in the internal capsule/ basal ganglia/thalamus in 13 patients, subcortical white matter in 5 patients, brainstem in 2 patients, cortex (multiple small lesions) in 1 patient, and cortex + basal ganglia in 1 patient. The median volume of the lesions was 0.6 ml on the initial examination and on follow-up, of 17 patients after 1 to 5 months, 0.5 ml. CONCLUSIONS: Almost all patients with acute ischemic lacunar syndromes have acute lesions on echo-planar diffusion-weighted MRI within 3 days after stroke onset. These lesions are mostly small and subcortical, compatible with lacunar infarcts caused by single penetrating artery occlusion, but in a minor proportion of patients (2 of 23 in our study) a cortical involvement is found.     

Prognosis and MRI after ischemic stroke of the basal ganglia.

The clinical courses and long-term prognoses in 16 young patients with infarctions of the basal ganglia were evaluated and the recent magnetic resonance imaging findings in 9 of them were examined. Only 5 of 14 patients (35%) had motor sequelae, 4 had hemiparesis, and 1 had gait disturbance. Secondary dystonia occurred in 1 patient. Magnetic resonance imaging disclosed a circumscribed lesion in the basal ganglia, as reflected by T2 high- and T1 low-intensity signals, in all patients. The abnormal region on T2-weighted images usually was more extensive than that observed on T1-weighted ones. The hemiplegic patients each had an area of abnormal intensity in the internal capsule or corona radiata with relatively high signals on the T2- and proton-density-weighted images. Mild to moderate asymmetric atrophy of the midbrain on the side ipsilateral to the stroke lesion was observed in 8 of 9 patients. The mechanism involved may be remote transsynaptic neuronal death of the substantia nigra, as well as Wallerian degeneration of the pyramidal tract.     

Hemidystonia‐hemiatrophy syndrome

To define the clinical and radiological features of patients with the combination of hemidystonia (HD) and hemiatrophy (HA), the HD‐HA syndrome. HD is a very disabling neurological condition that is rarely associated with HA of the affected body part, similar to the hemiparkinsonism‐hemiatrophy syndrome. Method: We reviewed the medical records of 26 patients with the HD‐HA syndrome and the data was entered into a database and analyzed. Video recordings as well as imaging studies were also reviewed. Twenty six patients (14 female) with a mean age at onset of HD at 14.9 years (1–46 years) were followed for a mean of 3.4 years. Fourteen (53%) had HD and HA on the left side and 23 (88%) had hemiparesis preceding the onset of HD. The mean latency from the onset of hemiparesis to the onset of HD was 14.7 years (2 weeks–46 years). All patients with hemiparesis had marked improvement in their weakness prior to the onset of HD. Common causes leading to hemiparesis and subsequent HD were birth or perinatal complications (N = 13) and stroke (N = 10). Seven patients (26%) had associated seizures. Twenty two patients (85%) had abnormal brain MRI: eight had lesions directly involving the basal ganglia and nine had cerebral hemiatrophy or non specific diffuse atrophy. Sixteen patients received botulinum toxin injections and responded well to treatment. HD‐HA is usually associated with static encephalopathy originating at very young age, but the syndrome may also represent delayed sequelae of a stroke or brain injury. © 2008 Movement Disorder Society     

Lacunar syndrome due to intracerebral hemorrhage

It has been recognized that small intracerebral hemorrhage not uncommonly produced lacunar syndromes. In this study, we examined cases of intracerebral hemorrhage presenting as lacunar syndromes. Of 174 cases with recent intracerebral hemorrhage, 19 presented with a lacunar syndrome: 4 presented with pure motor hemiparesis, 5, ataxic hemiparesis, 3, dysarthria-clumsy hand syndrome, 7, sensorimotor stroke, and, none, pure sensory stroke. The sites of hemorrhage were capsular in 11, putamenal in 6, and pontine in 2. In these 19 patients, 17 were hypertensive, and the signs characteristic of parenchymal hemorrhage, e.g., gradual onset, headache, nausea, vomiting and stiff neck, were absent or very rare. Computed tomography revealed that one third of the patients had one or more non-symptomatic lacunae in the basal ganglia, the corona radiata or the anterior limb of the internal capsule. These observations suggests that hypertensive intracerebral hemorrhage causes lacunar syndrome more often than previously considered and is apt to manifest ataxic hemiparesis and sensorimotor stroke. Computed tomography is the only way of differentiating hemorrhagic "lacunar" syndrome from lacunar infarct.     

A case of Foix-Chavany-Marie syndrome and crossed aphasia after right corona radiata infarction with history of left hemispheric infarction]

Foix-Chavany-Marie syndrome (FCMS) is a syndrome that presents facio-pharyngo-glosso-masticatory diplegia with automatic voluntary dissociation. Its most common etiology is stroke in the regions of bilateral opercula. We described a 75-year-old woman with FCMS and crossed aphasia. She had cerebral infarction of left middle cerebral artery territory 23 years before. At that time she had transient right hemiparesis, but no aphasia. This time, she suddenly became mute and was brought to our hospital. Neurological examination revealed severe weakness in her bilateral lower face, pharynx, tongue, and sternocleidomastoideus. She had no weakness of limbs. Her listening comprehension was moderately disturbed and handwriting was paragraphic. Her emotional facial movement was maintained despite of disturbed volitional facial movement. CT scan disclosed fresh infarction at the right corona radiata and old infarction at the left middle cerebral artery territory. In this patient, lesions at the left operculum and right corona radiata with the preserved right operculum gave rise to FCMS. This implies following possibilities: 1) the corticobulbar tract and corticospinal tract run separately at the corona radiata, 2) volitional and emotional tracts of facial movement run separately at the corona radiata. It was demonstrated that FCMS is not always caused by bilateral operculum lesions. Our patient did not show aphasia after the first stroke including left language area, but became severely aphasic after the right corona radiata infarction. Simultaneous occurrence of FCMS and aphasia after corona radiata lesion suggested that the corticobulbar tract and a tract that conducts linguistic information are running adjacently in the corona radiata. Our case suggested that restricted corona radiata lesion may cause severe subcortical aphasia and in case of additional contralateral corticobulbar tract lesion, severe dysarthria may occur.     

大脑中动脉梗死患者运动功能的恢复与脉络丛前动脉供血脑区有关？

no chinese abstract     

低场MRI和MRA在成人烟雾病诊断中的价值探讨

目的探讨低场MRI和MRA对成人烟雾病的诊断价值。方法回顾性分析24例成人烟雾病的临床与低场MR资料。结果 MRI表现：双侧放射冠区、基底节区多发脑梗死、软化14例,顶、枕叶大面积脑梗死2例,其中梗死合并出血6例;单纯脑出血8例,其中放射冠区、基底节区出血6例,左顶枕叶出血1例,脑室内出血1例。MRA表现：单侧大脑前动脉、大脑中动脉近段狭窄或闭塞16例,双侧大脑前动脉、大脑中动脉近段闭塞8例,丘脑-基底节区有异常血管网12例。结论 低场MRI可显示成人烟雾病的脑实质及血管病变,可作为烟雾病的筛选方法。     

Vascular ataxic hemiparesis: a re-evaluation.

Ataxic hemiparesis is commonly considered as one of the "typical" lacunar syndromes. Using the prospective stroke registries from Lausanne and Besançon, 100 patients were selected consecutively (73% men, 27% women; age 64.7 (SD 13.6) years) with a first stroke and ataxic hemiparesis (hemiparesis or pyramidal signs and ipsilateral incoordination without sensory loss). Brain CT or MRI was performed on all patients. A primary haemorrhage was present in 5%, an infarct in 72%, isolated leukoaraiosis in 9%, and no apparent abnormality in 14%. The locations of lesions were the internal capsule (39%), pons (19%), thalamus (13%), corona radiata (13%), lentiform nucleus (8%), cerebellum (superior cerebellar artery territory) (4%), and frontal cortex (anterior cerebral artery territory) (4%). The clinical features of ataxic hemiparesis with different locations were almost identical. Only minor associated signs allowed the localisation of the lesions (paraesthesiae with a lesion in the thalamus; nystagmus or dysarthria with a cerebellar or pontine location). Crural paresis with homolateral ataxia was seen only with cortical paramedian frontal lesions. Presumed hypertensive small artery disease was not always found, but was still the leading cause of stroke, being present in 59% of the patients and in 62% of those with small deep infarcts. A potential source of embolism (arterial or cardiac) was found in one fourth of the patients. Therefore no definite association can be made between ataxic hemiparesis and lacunar infarction. In particular, so called uncommon lesion locations may not be rare. After extensive investigations a diagnosis of lacunar infarct can be retained in only slightly more than half of the cases.     

A case of young adult presenting with cerebral infarction caused by homocystinuria]

Homocystinuria is a congenital metabolic disorder, and has been known as life-threatening risk factor of vascular disease including ischemic stroke. We report a case of cerebral infarction due to homocystinuria. The patient was a 21-year-old woman exhibiting left hemiparesis and a previous history of ectopia lentis. Magnetic resonance imaging showed multiple fresh infarctions in the right frontal and temporal lobes, basal ganglia, corona radiata, and internal capsule. The right common carotid angiogram demonstrated complete occlusion at the origin of the right internal carotid artery. Further investigation clarified increased level of serum methionine and homocysteine and urinary homocystin due to cystathionine beta-synthase deficiency. Homocystinuria was diagnosed as the cause of cerebral infarction. The patient was treated by low methionine diet and administration of folic acid, cobalamin, and aspirin. It should be recognized that some patients with homocystinuria are missed in the neonatal screening for congenital metabolic disorders. Recent studies indicated that the homocysteinemia is one of risk factors of ischemic stroke in the general population as well as in the patients of homocystinuria. We recommend metabolic screening for homocystinuria, when treating a juvenile patient with ischemic stroke of unknown etiology.     

Ipsilateral weakness caused by ipsilateral stroke: A case series

IntroductionThere are few reported cases of ipsilateral weakness following ischemic or hemorrhagic stroke. In these rare cases, ipsilateral weakness is typically the result of damage to uncrossed components of the corticospinal tract (CST) which were recruited in response to previous CST injury.Patients and methodsWe report a series of six cases of acute ipsilateral weakness or numbness following a hemorrhagic or ischemic stroke from three medical institutions in Saudi Arabia.ResultsThree of these patients presented with right-sided weakness caused by an ipsilateral right hemispheric stroke, while two exhibited left-sided symptoms and one had only left-sided numbness. In all six cases, the ipsilateral corona radiata, internal capsule, basal ganglia, insula, and thalamus were involved. No concomitant opposite hemisphere or brainstem lesion in none of the patients was evident. Two patients had previous strokes affecting the brainstem and left corona radiata, respectively. Complete stroke workup to reveal the cause of stroke was carried out, however no functional MRI was performed.ConclusionIschemic or hemorrhagic stroke may indeed result in ipsilateral weakness or numbness, though in very rare cases. We assume that the most likely mechanism of their ipsilateral weakness subsequent to the ipsilateral stroke was a functional reorganization favoring CST pathways within the ipsilateral hemisphere.     

A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy

BACKGROUND: Clinical features suggesting a diagnosis of progressive supranuclear palsy (PSP) include early falls, axial rigidity, vertical supranuclear ophthalmoplegia, and levodopa unresponsiveness. When these clinical features are present, the diagnosis is almost always PSP, yet vascular disease sometimes has a similar presentation, referred to as vascular PSP. OBJECTIVE: To evaluate clinical and pathologic features of cases of vascular PSP submitted to a PSP brain bank. DESIGN: Review of gross and microscopic neuropathological features, determination of tau haplotype, and medical record review of 4 patients with an antemortem diagnosis of PSP who did not meet the pathologic criteria for PSP and instead had vascular pathologic abnormalities. RESULTS: All patients had vertical supranuclear ophthalmoplegia, a history of falls, and a gradually progressive disease course. Falls began 1 year after symptom onset, and all patients had asymmetric findings on a neurological examination. A magnetic resonance imaging scan revealed lacunar basal ganglia infarcts in one patient and an increased T2-weighted signal in the corona radiata and centrum semiovale in another. Gross and microscopic neuropathological studies demonstrated infarcts in the cerebral cortex (n = 4), thalamus (n = 4), basal ganglia (n = 3), and cerebellum (n = 4). The brainstem was affected in one patient, but no infarcts were detected in the subthalamic nucleus or substantia nigra. Of the 4 patients, 3 carried an H2 tau haplotype, a rare occurrence in the general population. CONCLUSIONS: Asymmetric signs, falls after 1 year of symptom onset, vascular lesions on a magnetic resonance imaging scan, and an H2 tau haplotype may help differentiate vascular PSP from PSP. Thalamic and basal ganglia infarcts are common in patients with vascular PSP and, when present, may contribute to misdiagnosis.     

Magnetic Resonance Imaging Findings in Japanese Encephalitis

Ten patients with Japanese encephalitis diagnosed by serological criteria underwent magnetic resonance imaging (MRI) in axial and coronal sections. In 6, a second MRI study was done. The MRI findings were compared with the clinical outcome. Four patients died within several months of onset, 2 had sequelae such as hemiparesis and dementia, and the remaining 4 had no sequelae. In 9 of 10 patients, either diffuse or patchy white matter lesions were observed bilaterally, together with abnormalities in areas such as the thalamus, basal ganglia, and brainstem. For 3 patients who died or remained demented, the second MRI revealed extensive, diffuse white matter abnormalities. This study indicates that Japanese encephalitis can produce white matter involvement, although gray matter structures such as the thalamus, basal ganglia, and brainstem are more severely affected. The severity of these MRI lesions correlated with the clinical outcome.     

单侧孤立基底节区腔隙性脑梗死早期神经功能恶化的多因素分析及缺血耐受的相关性研究

目的 探讨单侧孤立的基底节区腔隙性脑梗死早期神经功能恶化（early neurological deterioration, END）的相关因素及其与脑缺血耐受（brain ischemic tolerance,BIT）机制的研究。 方法 回顾性分析经磁共振证实的167例单侧新发孤立的基底节区腔隙性脑梗死患者的临床资 料,并排除大动脉狭窄患者。根据卒中发生后1周内动态的美国国立卫生研究院卒中量表（National Institutes of Health Stroke Scale,NIHSS）评分情况将167例患者分为END阳性及END阴性两组,使用 Logistic回归分析研究与脑梗死早期神经功能恶化有关的因素。 结果 167例患者中42例发生END,125例未发生END。多因素分析结果显示病灶累及内囊后肢侧脑室 旁放射冠后部是预测END的独立危险因素,两组比较差异有显著性（P <0.01）;而发病年龄、脑梗死 病史、糖尿病史、入院收缩压及脑白质病变与END有相关性,且比较差异有显著性（P <0.05）。分别 以有无合并糖尿病、不同病灶位置进行分层并层间的单因素及多因素分析,结果显示,有无合并糖尿 病、病灶部位的不同不影响年龄、卒中史、脑白质与END阳性的关系,END与既往脑梗死病史、脑白质 病变、发病年龄存在相关性,比较差异有显著性（P <0.05）。 结论 单侧孤立基底节区腔隙性脑梗死早期END与病灶是否累及内囊后肢侧脑室体旁后部密切相关, 同时也与是否合并脑白质病变、既往脑梗死病史、糖尿病史、年龄等因素相关。年龄≥65岁、收缩压 高、卒中史、脑白质病变是END阳性的保护因素。缺血不耐受为腔隙性脑梗死发生END的主要机制。     

Neuroradiological findings in classical late infantile neuronal ceroid-lipofuscinosis

We describe a girl aged 5 years, 6 months who began to have seizures at the age of 3 years, 9 months. A cranial CT scan revealed mild, generalized cerebral atrophy. During the next year, she gradually developed ataxia, myoclonic jerks, and bilateral optic nerve atrophy and lost motor skills. A second CT scan performed 12 months after the onset of first symptoms revealed marked progression of cerebral atrophy, especially in the infratentorial area. MRI demonstrated bilateral, periventricular hyperintensities in the T₂-weighted images but no changes in the basal ganglia. Electron microscopic investigations of skin biopsies demonstrated curvilinear bodies, confirming the suspected diagnosis of late infantile neuronal ceroid-lipofuscinosis (LINCL). Predominance of cerebral atrophy in the infratentorial area is typical of LINCL. Periventricular white matter lesions may be evident on MRI scans of patients with classical and LINCL-variant disease. In contrast to neuroradiological findings in patients with LINCL-variant disease, findings in patients with classical LINCL revealed no changes in the basal ganglia.