Clinical Study of Cerebral Ischemia in Moyamoya Disease from the View of Development of the Anterior Choroidal Artery

ObjectivesThe anterior choroidal artery (AchA) is one of the collateral vessels in moyamoya disease (MMD). The incidence of cerebral ischemia in MMD was analyzed through the association between development of the AchA and advancement of MMD stage.Materials and MethodsTwelve patients of MMD with cerebral ischemia (infarction; 9 patients, transient ischemic attack; 3 patients) were enrolled. Advancement of MMD was evaluated using Suzuki's stage. The grades in Suzuki's stage were subclassified into a non-progressive stage for grades 1 and 2, and a progressive stage for grades 4 and 5. Dilatation of the AchA was judged as the presence of development of this artery. Development of the AchA was grouped into proximal type and proximal and distal type.ResultsMost frequent locations of infarcts were the anterior and parietal lobes in 6 patients each. Development of the AchA was confirmed on the ischemic side in all patients and on the non-ischemic side in 9 patients. Development of the AchA in the progressive stage was limited in the proximal and distal type on both sides. Development of the AchA in the non-progressive stage was the proximal type on the ischemic side.ConclusionsThe cause of cerebral ischemia was possibly associated with inadequate blood supply of the AchA in the non-progressive stage, and the lower blood flow from the internal carotid artery (ICA) in the progressive stage. Disparity between collateral blood flow from the AchA and the blood flow from the ICA was considered to relate to incidence of cerebral ischemia in MMD.     

Anterior Choroidal Artery Infarction Evaluated with 123I-Imp Single-Photon Emission Computed Tomography and 7 Tesla Magnetic Resonance Imaging

Anterior choroidal artery (AchA) infarction remains a challenging diagnosis although it was first described almost 100 years prior. N-isopropyl-p-[¹²³I]-iodoamphetamine single-photon emission computed tomography (¹²³I-IMP SPECT) and 7 Tesla magnetic resonance angiography (7T-MRA) are not routinely performed in cases of AchA infarction. Therefore, the application of ¹²³I-IMP SPECT and 7T-MRA for AchA infarction has not been reported previously. A 67-year-old man presented with disturbed consciousness, gaze preference to the left, aphasia, right homonymous hemianopia, and right hemiparesis. Brain magnetic resonance imaging revealed infarction of the left posterior limb of the internal capsule. Left middle cerebral artery was clearly seen on MRA. However, ¹²³I-IMP SPECT on day 13 showed cortical hypoperfusion which indicated thalamus involvement with neural deactivation. Additionally, 7T-MRA on day 15 revealed an intact left AchA suggesting reperfusion. The neurological deficits improved gradually after treatment and rehabilitation. This case demonstrates AchA infarction with cortical hypoperfusion associated with thalamus involvement, which was clarified by performing ¹²³I-IMP SPECT and 7T-MRA. Perfusion analysis and evaluation of detailed vascular anatomy in stroke can be expected to elucidate pathological conditions.     

Ruptured distal anterior choroidal artery aneurysm.

Ruptured aneurysms of the distal anterior choroidal artery (AchA) are extremely rare and management is dictated by clinical presentation. This report describes a rare patient with a distal AchA aneurysm and subarachnoid and intraventricular haemorrhage. A 60-year-old woman presented with a sudden onset of severe headache and vomiting. No aneurysms could be found on initial angiograms. A repeat angiogram performed 10 days after admission demonstrated an aneurysm in a branch of the cisternal segment of the left AchA without a definite neck. Surrounding vessels showed multifocal stricture and dilatation. Microsurgical clipping was not performed because the patient died suddenly due to pulmonary failure. The aetiology of the aneurysm in this case and surgical strategy are discussed.     

Differential diagnosis of infundibular dilation versus a small aneurysm of the internal carotid artery: assessment by three-dimensional rotational angiography with volume rendering

We report our findings associated with the differential diagnosis of infundibular dilation (ID) versus a small intracranial aneurysm using three-dimensional rotational angiography with volume rendering (3DRA + VR). Angiographic findings associated with IDs found via two-dimensional digital subtraction angiography (2D-DSA) or 3DRA + VR were reviewed for 138 consecutive patients with known or suspected aneurysms. Two readers independently evaluated the results of 2D-DSA and 3DRA + VR according to the same diagnostic criteria. We also evaluated the ability of 3D-DSA to show the spatial relation between IDs and anterior choroidal (AchA)/posterior communicating (PcomA) arteries. 2D-DSA and 3DRA + VR found 41 and 48 IDs, respectively. 2D-DSA missed five AchA and two PcomA IDs. 2D-DSA was significantly inferior to 3DRA + VR for displaying the spatial relation between IDs and AchA/PcomA (P = 0). Thus, 3DRA + VR provides more useful information for distinguishing IDs from aneurysms. The superiority of 3DRA + VR might be because of its ability to display the spatial relation between IDs and AchA/PcomA.     

Duplicated Middle Cerebral Artery Aneurysms Treated by Coil Embolization; A Report of Two Cases and Literature Review

BackgroundDuplication of the middle cerebral artery (DMCA) is an anomalous vessel arising from the internal carotid artery (ICA). Aneurysms at the origin of a DMCA have been reported; however, most have been treated with clipping surgery. Here, we describe two cases of aneurysms at the origin of a DMCA treated with coil embolization.Case presentationCase 1: A seventy-three year-old man presented with severe headache and was diagnosed with subarachnoid hemorrhage (SAH). Digital subtraction angiography (DSA) and 3-dimensional (3-D) DSA showed an aneurysm arising from a DMCA. Coil embolization was performed with DMCA patency. The patient had an uneventful postoperative course.Case 1A 44-year-old woman presented with a history of clipping for an IC-anterior choroidal artery (AchA) aneurysm 8 years prior. Magnetic resonance imaging (MRI) showed regrowth of the aneurysm. 3-D DSA showed an IC-DMCA aneurysm located laterally and distal to the AchA. The DMCA arose from the bottom of the aneurysm. Coil embolization was performed without DMCA occlusion and showed no postoperative ischemic changes.ConclusionAn IC-DMCA aneurysm is rare and may be misdiagnosed as an AchA aneurysm. Clinicians should perform a 3D-DSA evaluation if the aneurysm arises from the lateral wall of the IC to obtain a precise diagnosis and to preserve the DMCA during coil embolization.     

Pure spastic paralysis of corticospinal origin

This presentation includes six cases of chronic bilateral pure motor hemiplegia, one of these with pathological findings; one clinical case of chronic pure motor quadriplegia and one pathologically-studied case of chronic pure motor paraplegia. These cases may illustrate a spectrum of pure corticospinal disorders that heretofore has not been fully recognized.     

Pediatric brain stem tumor presenting as a pure motor hemiparesis

The clinical syndrome of pure motor hemiparesis occurs with lacunar lesions in the internal capsule or in the basis pontis in hypertensive adults. It has been described in three patients with brain stem tumors of which two were children. A 3 1/2 years old boy with a brain stem tumor which presented as pure motor hemiparesis is described.     

Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism

Journal of Neurology - Although pure cerebellar ataxia is usually emphasized as the characteristic clinical feature of spinocerebellar ataxia type 6 (SCA6), parkinsonism has been repeatedly...     

Pure motor hemiparesis due to non-hypertensive putaminal haemorrhage

Summary The syndrome of pure motor hemiplegia (PMH) has been traditionally regarded to be due to lacunar infarcts at various places in the brain. Recently, attention has been drawn towards various other aetiological factors which have been responsible for this clinical presentation. PMH as a result of hypertensive putaminal haemorrhage is extremely uncommon. A case of PMH as a result of non-hypertensive putaminal haemorrhage is described.     

Pure word deafness and pure anarthria in a patient with frontotemporal dementia

A 66-year-old right-handed man developed pure anarthria following pure word deafness. In addition to language disorders, his behavior gradually changed and finally included violence against his wife. Brain magnetic resonance imagings revealed atrophy of the left perisylvian area, which included the inferior half of the precentral gyrus and the upper portion of the superior temporal gyrus, consistent with frontotemporal dementia (FTD). It has been documented as either a disorder of expressive language or as an impaired understanding of word meaning. Unlike with pure anarthria, pure word deafness is not included in the clinical diagnostic current criteria for FTD. However, a large variety of language symptoms can appear in FTD according to the distribution of pathological changes in the frontotemporal cortices. This case suggests that pure word deafness could be a prodomal symptom of FTD.     

Early Diagnosis by Diffusion-Weighted MRI of Pure Motor Stroke Limited to Finger Weakness

Pure motor neurologic deficits occur among 3%–14% of all patients with ischemic stroke. Pure motor monoparesis occurs among 2%–22% of patients with pure motor stroke. The authors report the case of a 73-year-old woman with isolated finger weakness as the sole manifestation of a small cortical-subcortical pure motor stroke diagnosed within 4 hours of onset by means of diffusion-weighted echoplanar magnetic resonance imaging. To the authors' knowledge, the case of a patient with pure motor stroke with finger weakness as the only manifestation has not been previously reported. Increased awareness of this rare clinical presentation of pure motor stroke coupled with the promise of prompt diagnosis by means of diffusion-weighted MRI should lead to earlier stroke intervention.     

Vascular cognitive impairment

Cognitive impairment commonly accompanies clinical syndromes associated with vascular disease of the brain. Because of evolving definitional criteria, however, the frequency of cognitive impairment attributable to cerebrovascular disease is difficult to determine. Dementia occurs in up to one-third of elderly patients with stroke, a subset of whom have Alzheimer's disease (AD) rather than a pure vascular dementia syndrome. In fact, pure vascular dementia has been shown to be uncommon in most large autopsy series. A mixed etiology of AD and cerebrovascular disease is thought to become more common with increasing age, although no clinical criteria for the diagnosis of AD with cerebrovascular disease are currently available. Epidemiological studies have implicated subcortical small-vessel disease as a risk factor for cognitive impairment and dementia, but the cognitive expression and clinical significance of MRI white matter changes in individual patients is difficult to establish. The frequency of specific neuropathologic features of vascular cognitive impairment depends largely on study inclusion criteria. Cerebral meningocortical microangiopathies with distinctive clinicopathological profiles are associated with dementia in both sporadic cases and familial syndromes. In patients with AD, the contribution of amyloid-beta protein to the degree of cognitive impairment has not been clearly defined.     

Diagnostic and classification criteria for the Guillain-Barré syndrome

BACKGROUND: Diagnostic criteria for the Guillain-Barré syndrome (GBS) have been available since 1978. Since then, several variants have been described. More recently, a distinction has been made between pure motor forms, severe sensory forms, primary axonal and primary demyelinating varieties. Associations of clinical characteristics, and specific infections and the presence of antiganglioside antibodies have been found. For further studies on GBS, it is therefore necessary to reconsider the available diagnostic criteria and add additional criteria for subclassification. METHODS: A panel of (20) experts was formed. The literature representing the recent developments in GBS subclassification was reviewed. Following a consensus protocol, diagnostic and classification criteria were formulated. RESULTS: The diagnosis of GBS can usually be made on clinical characteristics. A schedule for subclassification has been made to cover also the clinical variants in a systematic manner.     

Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy.

The clinical syndrome of pure akinesia has most often been associated with progressive supranuclear palsy (PSP) and is characterized by difficulty initiating gait and "freezing" during walking, writing and speaking. Similar syndromes have been described under the rubrics of primary progressive freezing gait and primary gait ignition failure. We investigated the specificity of the clinical syndrome of pure akinesia with gait freezing (PAGF) for PSP-tau pathology. Among 749 patients archived at the QSBB, only 7 fulfilled proposed diagnostic criteria of: gradual onset of freezing of gait or speech; absent limb rigidity and tremor; no sustained response to levodopa; and no dementia or ophthalmoplegia in the first 5 years of disease. In these cases detailed pathological examination was performed. PSP was the pathological diagnosis in six patients, and Parkinson's disease (PD) in the seventh. As defined, this syndrome had a positive predictive value of 86% for PSP-tau pathology. In the cases with PSP there were no additional features of coexistent vascular or PD and the median PSP-tau score was 3, reflecting relative mild tau load. The clinical syndrome of PAGF appears to have a high specificity for PSP-tau pathology. This relatively uncommon presentation of PSP-tau pathology has less severe tau accumulation than in the more common, "classic" PSP clinical phenotype: Richardson's disease.     

Late motor involvement in cases presenting as “chronic sensory demyelinating polyneuropathy”

Chronic inflammatory demyelinating polyneuropathy (CIDP) is usually characterized by prominent motor deficits. A pure sensory presentation, labeled chronic sensory demyelinating neuropathy (CSDN), has been reported, but it is unclear if this neuropathy is a distinct clinical and immunologic entity or merely the sensory presentation of the more usual sensorimotor CIDP. We describe 5 patients with what initially appeared to be CSDN; 3 subsequently developed substantial weakness coincident with the electrophysiologic appearance of multifocal motor conduction block. These cases indicate that, in some cases, CSDN may be a transitional clinical stage of CIDP in which the more usual sensorimotor deficits develop later. Immune-based therapy, including intravenous immunoglobulin, was found to be effective in both the pure sensory and sensorimotor types. © 1995 John Wiley & Sons, Inc.     

Acute motor axonal neuropathy--an atypical presentation

Guillain-Barré syndrome, an autoimmune disorder of the peripheral nervous system, is divided into several subtypes according to clinico-pathologic findings and epidemiologic characteristics. A pure motor variant without involvement of the sensory nerves has been reported in rare cases. This report details the clinical, immunologic, and serial electrophysiologic findings of two patients with an acute, exclusively motor, axonal neuropathy.     

Etiology of motor or sensory stroke: a prospective study of the predictive value of clinical and radiological features.

The diagnostic utility of clinical and radiological features for distinguishing penetrating artery disease from other causes of stroke has been questioned. To address this issue, we prospectively evaluated more than 40 features in 85 patients with pure motor, sensorimotor, or pure sensory syndromes. Nonischemic causes were present in 4 patients. The causes of ischemic stroke in the other 81 patients were penetrating artery disease (32 patients), large artery occlusive disease (17), cardioembolism (12), other causes (8), and undetermined (12). Of the features evaluated, frequent transient ischemic attacks (greater than or equal to 1/day), transient ischemic attacks occurring only within 1 week of stroke, pure motor hemiparesis (similar involvement of face, arm, and leg), pure sensory stroke, and round or oval infarction were associated (p less than 0.05) with penetrating artery disease. However, only pure sensory stroke involving two or more regions of the body and pure motor hemiparesis associated with subcortical infarction of less than 1.5 cm had positive predictive values exceeding 90% for penetrating artery disease. Carotid bruit ipsilateral to an anterior circulation infarction, monoparesis, and striatocapsular infarction had positive predictive values exceeding 90% for causes other than penetrating artery disease; however, half of the striatocapsular infarctions were of undetermined cause. These data suggest that certain clinical and radiological features are useful for distinguishing penetrating artery disease from other causes of stroke.     

Chronic remitting-relapsing experimental allergic encephalomyelitis induced in monkeys with homologous myelin basic protein

A chronic remitting-relapsing form of experimental allergic encephalomyelitis (EAE) has been produced in monkeys sensitized to homologous myelin basic protein in Freund's complete adjuvants by the technique of suboptimal treatment after the onset of disease. Not only does the clinical course resemble that of human multiple sclerosis more closely than does the clinical course of acute EAE, but so also does the histological reaction, with more-nearly pure demyelination, rather than the hyperacute hemorrhagic-necrotic lesions that occur so commonly in untreated monkeys with ordinary acute EAE.     

Effect of capsular infarct size on clinical presentation of stroke

We reviewed the medical records and cranial computed tomograms of 74 patients with acute capsular infarcts to investigate the correlation between infarct size and clinical symptoms. Average infarct size varied significantly by clinical syndrome; patients with sensorimotor stroke had the largest infarcts, patients with pure motor hemiparesis had middle-sized infarcts, and patients with ataxic hemiparesis or the dysarthria-clumsy hand syndrome had the smallest infarcts. Although it has been proposed that the type of lacunar syndrome is determined entirely by the infarct location, our results suggest that infarct size is another important factor influencing the clinical presentation of lacunar syndromes.     

Dimensions of mania: differences between mixed and pure episodes.

OBJECTIVE: The presence of at least five dimensions in mania has recently been established. This study extends previous findings by comparing the dimensions of pure vs. mixed mania. MATERIALS AND METHOD: One hundred and three inpatients with bipolar I disorder, manic or mixed (DSM IV), were assessed with SCID-I, YMRS and HDRS-21. The five-factor solution found after applying factorial analysis with Varimax rotation was compared between manic and mixed patients. RESULTS: There were differences between pure mania and mixed states on factor 1 (depression) and factor 3 (hedonism). There was a tendency to present higher values on factor 5 (activation) in the pure manic group. No differences were found in factor 2 (dysphoria) and factor 4 (psychosis). DISCUSSION: Hedonism and activation dimensions are present to a lesser degree in mixed states. Although the principal difference between mixed and pure bipolar disorder is the existence of depressive symptoms, the depressive dimension is strongly present in patients with pure mania. CONCLUSIONS: There is need to search for core depressive symptoms in all patients suffering from mania and to evaluate their outcome in clinical trials.