Mixed Pial-Dural Arteriovenous Malformation in the Anterior Cranial Fossa Mimicking Dural Arteriovenous Fistula.

Mixed pial-dural arteriovenous malformation (MpdAVM) and dural arteriovenous fistula (dAVF) are rare entities in the anterior cranial fossa (ACF). As dural-pial vascular anastomosis can exist near the cribriform plate, MpdAVM with a small nidus, which cannot be apparently identified, can be logically indistinguishable from dAVF in ACF. A 71-year-old man was referred for evaluation of possible intracranial vascular malformation. Cerebral angiography revealed an arteriovenous shunt in the ACF. The lesion was fed by the bilateral ethmoidal arteries and right orbitofrontal artery, draining through the bilateral cortical veins to the superior sagittal sinus. As a nidus was not detected, dAVF was suspected. Venous interruption was planned with direct surgery. Intraoperatively, an arterial aggregation was observed in the right frontal lobe. The arterial aggregation seemed to be connected to the interrupted drainer in the right ACF. The arterial aggregation was removed and pathologically diagnosed as arteriovenous malformation. Postoperatively, intracerebral hemorrhage was confirmed, and postoperative cerebral angiography confirmed the resolved arteriovenous shunt. The intracranial hemorrhage was possibly due to the timing gap between drainer interruption and removal of the nidus. MpdAVM with a small nidus in the ACF can mimic dAVF. Clinicians must be aware that an unremoved nidus of MpdAVM may postoperatively result in fatal intracranial hemorrhage.

Intrathecal baclofen therapy for Lesch-Nyhan disease: illustrative case.

BACKGROUND: Lesch-Nyhan disease (LND) is a very rare metabolic disorder involving the purine salvage pathway. LND manifests hyperuricemia, self-mutilation, cognitive impairment, and movement disorders such as spasticity and dystonia, whose control is difficult pharmaceutically. OBSERVATIONS: Intrathecal baclofen (ITB) therapy was received by a 22-year-old male for generalized dystonia. His paroxysmal abnormal dystonic posturing reduced after surgery, making the task of caregivers easier despite the unchanged assignment on the dystonia scale during a follow-up period of 4 years. LESSONS: ITB may be a safe and feasible option for dystonic symptoms and difficulty with nursing care in patients with LND.

A case of inferior frontal gyrus infarction manifesting Gerstmann syndrome.

A 48-year-old female suffered from cerebral infarction involving the left inferior frontal gyrus. This was due to ischemic complications of endovascular treatment for subarachnoid hemorrhage. She exhibited severe acalculia, agraphia, finger agnosia, and right-left disorientation (the four features of Gerstmann syndrome), but aphasia was scarcely noticeable. Single-photon emission tomography revealed hypoperfusion in the left inferior frontal area and also in the left parietal area. It is possible that Gerstmann syndrome was caused in the present case by disruption of the association fiber connecting the inferior frontal area with the inferior parietal area.

Premorbid instrumental activities of daily living predicts discharge home following stroke.

BACKGROUND AND PURPOSE: Stroke survivors who remain dependent require multiple healthcare resources, including rehabilitation and nursing care. The effect of premorbid instrumental ADL (IADL) on the discharge destination, which has not been studied previously in detail, is analyzed. MATERIALS AND METHODS: Between April and September 2015, 40 stroke patients admitted to hospital were enrolled prospectively in the present study. The ADL (Barthel index) and IADL (Frenchay activities index: FAI) scores in their premorbid status were recorded. Baseline demographic data, stroke severity (NIHSS) and type of stroke, and whether they lived with family were also recorded. Simple univariate regression was performed between the two discharge destination groups (Home or Not Home). Significant factors were then included in multivariate logistic regression in order to determine the adjusted odds ratio for the discharge destination. A P value <.05 was taken as significant. RESULTS: 25 patients (64.1%) returned home. According to univariate analysis, NIHSS on admission and premorbid FAI were significantly associated with the discharge destination. Multivariate analysis found that NIHSS (OR, 0.71; 95% CI0.56-0.92; p = .008) and premorbid FAI (OR, 1.17; 95% CI1.03-1.33; p = .01) were independent predictors of the discharge destination. CONCLUSIONS: Severity of stroke upon admission and premorbid IADL are associated with discharge destination following stroke.

Transcranial Direct Current Stimulation Improves Pusher Phenomenon.

An 83-year-old man suffered from cerebral infarction of the right middle cerebral artery territory. In association with severe left hemiparesis and hemispatial neglect on the left side, he showed severe pusher phenomenon (PP), which made rehabilitation difficult. Transcranial direct current stimulation (tDCS) was applied to the parietal area (2 mA × 20 min/day; anode on the right and cathode on the left) for 8 days, which resulted in remarkable improvement of PP and caused prolongation of static sitting time. tDCS of the parietal area could be a novel treatment option of PP following stroke.

Carotid Artery Stenting for Symptomatic Internal Carotid Artery Stenosis Associated with Moyamoya Disease.

BACKGROUND: Surgical intervention in a case of internal carotid artery stenosis with moyamoya vessels has not been well described. We present a case with detailed description of the surgical procedure and perioperative management. CASE DESCRIPTION: A 58-year-old man with symptomatic internal carotid artery stenosis had concurrent moyamoya vessels intracranially. He had motor weakness and numbness in the left upper extremity. Magnetic resonance imaging showed an ischemic lesion in the right frontal lobe and right chronic subdural hematoma. On day 18 after surgery for subdural hematoma, carotid artery stenting was performed under proximal balloon protection and distal filter protection. After carotid artery stenting, systolic blood pressure was maintained at <120 mm Hg. Hyperperfusion syndrome or hemorrhagic complication did not occur. The patient was discharged without any neurologic deficits. CONCLUSIONS: In our patient with internal carotid artery stenosis with moyamoya vessels, strict control of blood pressure likely contributed to prevention of hyperperfusion syndrome or hemorrhagic complications.

A local excitation magnetic resonance imaging method for intracranial unruptured aneurysm at the distal internal carotid artery.

It is often difficult to diagnose an intracranial aneurysm at the distal internal carotid artery by conventional magnetic resonance imaging. PURPOSE: We assessed the effectiveness of the local excitation technique, a new application for magnetic resonance imaging, to clarify the geometric structure of aneurysm and adjacent branches at the distal internal carotid artery. STUDY DESIGN: Two independent evaluators diagnosed 10 cases of suspected aneurysms at the distal internal carotid artery by T2-weighted magnetic resonance imaging with application of local excitation, adding it to conventional time-of-flight-magnetic resonance angiography. FINDINGS: We successfully distinguished the aneurysm from infundibular dilatation in five of 10 cases. CONCLUSION: Our results suggested that addition of local excitation to conventional magnetic resonance angiography was effective to diagnose unruptured aneurysm at the distal internal carotid artery, to clarify the configuration of the prominent lesion or whether the location of the adjacent branch orifice on the parent vessel was symmetric or asymmetric.

Alteration of Venous Drainage Route in Idiopathic Normal Pressure Hydrocephalus and Normal Aging.

Idiopathic normal pressure hydrocephalus (iNPH) is a highly prevalent condition in the elderly population; however, the underlying pathophysiology in relation to the aging process remains unclear. To investigate the effect of removal of cerebrospinal fluid by lumbar "tap test" on the cerebral circulation in patients with iNPH, 14 patients with "probable" iNPH were studied using a novel blood tracking technique based on blood oxygenation level-dependent (BOLD) magnetic resonance signal intensity. By tracking the propagation of the low-frequency component of the BOLD signal, extended venous drainage times were observed in the periventricular region of the patients, which was reversed by tap test. Interestingly, the venous drainage time in the periventricular region exhibited an age-related prolongation in the healthy control group. Additional regression analyses involving 81 control subjects revealed a dissociation of deep and superficial venous systems with increasing age, presumably reflecting focal inefficiency in the deep system. Our results not only provide insights into the etiology of iNPH, but also point to a potential non-invasive biomarker for screening iNPH.

Spontaneous Resolution of Cerebral Pial Arteriovenous Fistula After Angiography: Report of Two Cases.

BACKGROUND: Cerebral pial arteriovenous fistula (AVF) is a rare disorder, and its natural course is uncertain. The present article reports 2 rare cases of pial AVF that underwent spontaneous cure after diagnostic cerebral angiogram. CASE DESCRIPTIONS: A 73-year-old man presented with generalized seizure and reported severe but intermittent headache in the right temporo-occipital area. Single-photon emission computed tomography (SPECT) showed hyperperfusion in that area. The main finding of a cerebral angiogram was an arteriovenous shunt at the cortical surface of the right temporal area. Soon after this diagnostic angiogram, the symptoms vanished. A further cerebral angiogram showed the disappearance of this pial AVF, and a SPECT study showed resolution of the hyperperfusion of the affected area. A 69-year-old man, with a history of intracerebral hemorrhage on the left parietal area 3 years earlier, presented with speech disturbance and headache on his left side. SPECT showed hyperperfusion in the left temporoparietal area. A cerebral angiogram showed an arteriovenous shunt at the surface of the left parietal area. During the same cerebral angiogram session, the pial AVF disappeared after the third injection of a contrast medium with magnification. Two days after the cerebral angiogram, the patient's headache disappeared and speech disturbance gradually improved. SPECT also showed disappearance of the hyperperfusion. CONCLUSIONS: It is possible that pial AVF is a cause of headache and neurologic symptoms in association with focal hyperperfusion. Diagnostic cerebral angiography should be performed to make a definite diagnosis; after this, pial AVF sometimes disappears.

Combination of Transcranial Direct Current Stimulation and Neuromuscular Electrical Stimulation Improves Gait Ability in a Patient in Chronic Stage of Stroke.

BACKGROUND: Walking ability is important in stroke patients to maintain daily life. Nevertheless, its improvement is limited with conventional physical therapy in chronic stage. We report the case of a chronic stroke patient showing a remarkable improvement in gait function after a new neurorehabilitation protocol using transcranial direct current stimulation (tDCS) and neuromuscular electrical stimulation (NMES). CASE PRESENTATION: A 62-year-old male with left putaminal hemorrhage suffered from severe right hemiparesis. He could move by himself with a wheelchair 1 year after the ictus. Anodal tDCS at the vertex (2 mA, 20 min) with NMES at the anterior tibialis muscle had been applied for 3 weeks. The Timed Up and Go test and 10-meter walk test improved after the intervention, which had been maintained for at least 1 month. CONCLUSION: This single case suggests the possibility that tDCS with NMES could be a new rehabilitation approach to improve the gait ability in chronic stroke patients.

Evaluation of the Arterial Wall in Vertebrobasilar Artery Dissection Using High-Resolution Magnetic Resonance Vessel Wall Imaging.

BACKGROUND: High-resolution magnetic resonance vessel wall imaging (HRMR-VWI) has been used to explore vascular diseases such as vasculitis and vulnerable plaque of intracranial arteries. Although vessel wall inflammation is suspected as one of the causes of cerebral arterial dissection, there have been few reports regarding the application of HRMR-VWI to arterial dissection. We have therefore evaluated the efficacy of HRMR-VWI in patients with vertebrobasilar artery dissection. METHODS: HRMR-VWI was performed on 5 patients who had been diagnosed with nonhemorrhagic vertebrobasilar artery dissection. RESULT: Four patients exhibited vessel wall enhancement on HRMR-VWI, the range of which corresponded with the dissection sites identified by cerebral angiogram, magnetic resonance imaging, or magnetic resonance angiography. The enhancements observed in all cases were extensive as compared with the findings of conventional angiography. CONCLUSION: HRMR-VWI is thought to elucidate the condition of the affected vessel wall more in detail as compared with conventional methods.

Caudal cingulate infarction manifesting astasia.

INTRODUCTION: Astasia is a rare presenting symptom of stroke, usually known as 'thalamic astasia', induced by a lesion in the ventrolateral thalamus. We report a case of caudal cingulate infarction manifesting astasia. CASE PRESENTATION: A 58-year-old male presented with inability to sit, stand and walk (astasia). No apparent motor weakness was noticed in the extremities. MRI revealed cerebral infarction in the caudal cingulate gyrus, which was located between the vertical commissure anterior (VCA) line and vertical commissure posterior (VPC) line. His symptoms persisted for 1 year to a lesser degree. CONCLUSION: Lesions in the caudal cingulate gyrus can present with astasia. The responsible lesion is located in the cingulate gyrus between the VCA and VPC line, which might correspond to the caudal cingulate zone in humans. We should keep in mind that astasia can be a presenting symptom of stroke.

Pure intracerebral mass lesion of adult T-cell leukemia/lymphoma--case report.

A 48-year-old female presented with a rare case of adult T-cell leukemia/lymphoma (ATL) occurring as only intracerebral mass lesion manifesting as progressively worsening headaches, transient mild weakness of the left lower extremity, bilateral papilledema, and left homonymous hemianopsia. Laboratory examination showed transient leukocytosis (15900/microl) without febrile episode or elevation of C-reactive protein. Neuroimaging revealed a solitary enhanced mass lesion in the right occipital lobe adjacent to the choroid plexus with prominent perifocal edema. The patient underwent gross total removal, and the histological diagnosis was intracerebral ATL. She underwent radiation therapy and chemotherapy after local recurrence and metastasis to an optic nerve. The lesions had disappeared on magnetic resonance imaging with contrast medium 10 months after onset and no recurrence was detected even 5 years later. Intracerebral ATL should be considered in the differential diagnosis of intracerebral mass without leukemia or systemic lymphoma.

Heme oxygenase-1 gene therapy for prevention of vasospasm in rats.

OBJECT: Hemoglobin causes contraction of cerebral arteries and is also believed to cause vasospasm after subarachnoid hemorrhage (SAH). The goal in this study was to determine if overexpression of heme oxygenase-1 (HO-1), the principal enzyme involved in the metabolism of hemoglobin, would reduce contractions of cerebral arteries brought on by hemoglobin and decrease vasospasm after experimental SAH. METHODS: Injection of adenovirus expressing HO-1 (Ad5HO-1) into the cisterna magna of rats produced a significant increase in expression of HO-1 messenger RNA, and protein and HO-1 activity in the basilar artery ([BA]; p < 0.05 for each measure compared with vehicle and/or control virus, according to analysis of variance or unpaired t-test). Injection of adenovirus expressing beta-galactosidase (Ad-betaGal) produced only mild, statistically nonsignificant increases. The HO-I immunoreactivity was localized to the BA adventitia after injection of Ad5HO-1 or Ad-betaGal. Injection of Ad5HO-1 and Ad-betaGal increased the baseline diameter of the BA (measured directly via a transclival window) and brainstem cerebral blood flow (CBF), measured by laser Doppler flowmetry, compared with vehicle. Contraction of the BA after addition of hemoglobin was significantly inhibited, reduction in brainstem CBF was significantly prevented, and carboxyhemoglobin concentration was significantly increased in rats injected with Ad5HO-1 compared with Ad-betaGal and vehicle. Vasospasm was significantly ameliorated in rats in which Ad5HO-1 was injected into the cisterna magna at the time of SAH in a double-hemorrhage model. CONCLUSIONS: These results show that overexpression of HO-1 inhibits arterial contractions induced by hemoglobin and can reduce vasospasm after experimental SAH.

Up-regulation of parathyroid hormone receptor in cerebral arteries after subarachnoid hemorrhage in monkeys.

OBJECTIVE: Complementary deoxyribonucleic acid array analysis was used to determine whether vasospasm after subarachnoid hemorrhage (SAH) is associated with changes in gene expression. METHODS: Right SAHs were created in three monkeys, and the right and left middle cerebral arteries were collected 3, 7, or 14 days after SAH. Vasospasm was assessed by angiography performed on Day 0 and at tissue harvest. A complementary deoxyribonucleic acid array containing 5184 genes was used to screen for changes in gene expression by comparing the right and left middle cerebral arteries. RESULTS: There was significant expression (greater than fivefold expression of messenger ribonucleic acid compared with internal standard control) of 537 genes (10%) in the middle cerebral arteries. One hundred sixty-four genes (31%) did not change significantly, and 373 (69%) were differentially expressed at 3, 7, or 14 days after SAH. These 373 genes changed from 1.2- to 7-fold as compared with control arteries. The most common pattern was a progressive increase with increased time after SAH. The functions of differentially expressed genes included the regulation of gene expression, cell proliferation, inflammation, membrane proteins and receptors, kinases, and phosphatases. There was a marked increase in parathyroid hormone and parathyroid hormone receptor with time after SAH. Immunoblotting demonstrated a significant increase in parathyroid hormone receptor protein. CONCLUSION: The up-regulation of these proteins involved in vascular relaxation suggests that they may play a role in vasospasm. The progressive increase in messenger ribonucleic acids involved in the functions noted suggests that the pathogenesis of cerebral vasospasm involves cell proliferation, inflammation, and possibly smooth muscle phenotype change.