Absence of susceptibility vessel sign with cancer- associated hypercoagulability-related stroke.

BACKGROUND AND PURPOSE: Susceptibility vessel sign (SVS), a hypointense signal on MR T2-weighted gradient-recalled echo images, is associated with erythrocyte-predominant thrombi, which are often present in cardioembolism (CE). In contrast, cancer-associated hypercoagulability (CAH)-related stroke, which is presumably caused by fibrin-predominant thrombi, is associated with the absence of SVS. We hypothesized that the prevalence of SVS may be of help in distinguishing CAH-related stroke from CE. This study attempted to validate this hypothesis and investigated the usefulness of SVS in differentiating CAH-related stroke from CE. MATERIALS AND METHODS: We retrospectively studied both CAH-related stroke patients (CAH group) and CE patients (CE group), who had major cerebral artery occlusion on MR angiography that was performed within 6 hours of stroke onset. All patients visited our department from 2015 to 2021. CAH-related stroke was defined as 1) complication of active cancer, 2) pre-treatment D-dimer value >3 µg/mL, 3) multiple vascular territories infarctions, and 4) lack of any other specifically identified causes of stroke. We compared SVS positivity rates within each group. Multivariable logistic regression analysis was used to assess the association between the absence of SVS and CAH-related stroke. RESULTS: Of 691 patients with CAH-related stroke or CE, major cerebral artery occlusion was observed in 10 patients in the CAH group and 198 patients in the CE group. The absence of SVS was identified in 55 of 208 patients and was significantly more frequent in the CAH versus the CE group (90% versus 24%, p < 0.05). For predicting CAH-related stroke, absence of SVS demonstrated a sensitivity of 90% (95% confidence interval [95%CI] 59-99), specificity of 78% (95%CI 71-83), positive predictive value of 18 (95%CI 10- 31), negative predictive value of 99% (95%CI 96-99), and a likelihood ratio of 4.06. Multivariable logistic regression analysis revealed that the absent of SVS was independently associated with CAH-related stroke (odds ratio 43, 95% [CI] 6.8-863; p < 0.01). CONCLUSIONS: The absence of SVS was more frequent in CAH-related stroke versus that found for CE. These findings could potentially be helpful for clinical management and differentiating between CE and CAH-related stroke. ABBREVIATIONS: CAH, cancer-associated hypercoagulability; CE, cardioembolism; SVS, susceptibility vessel sign; GRE, gradient recalled echo.

Clinical characteristics of stroke in SARS-CoV-2 infected patients in Japan: A prospective nationwide study.

INTRODUCTION: We investigated the clinical characteristics and outcomes of stroke in SARS-CoV-2 infected patients in Japan. METHODS: This prospective, multicenter observational study of stroke in patients with SARS-CoV-2 infection involving 563 primary stroke centers across Japan was conducted between July 2020, and May 2022. We included 159 stroke cases (131 ischemic stroke, 2 transient ischemic attack (TIA), 21 intracranial hemorrhage, and 5 subarachnoid hemorrhage) and collected their clinical characteristics. Ischemic stroke and TIA (n = 133) were analyzed separately. RESULTS: The mean age of the 159 patients was 70.6 years, with 66% being men. Poor outcomes (modified Rankin Scale score 5-6) occurred in 40% (63/159) at discharge. Among patients with ischemic stroke and TIA, 30%, 18%, 10%, and 42% had cardioembolism, large-artery atherosclerosis, small-vessel occlusion, and cryptogenic stroke or embolic stroke of undetermined source, respectively. One-third (34%) presented with large vessel occlusion (LVO) of the internal carotid, middle cerebral M1, or basilar arteries. Poor outcomes included age (adjusted odds ratio (aOR): 1.06, 95%CI: 1.01-1.12), ischemic heart disease (IHD) history (aOR: 13.00, 95%CI: 1.51-111.70), moderate to severe pneumonia (aOR: 7.78, 95%CI: 1.18-51.42), an National Institutes of Health Stroke Scale score at baseline (aOR: 1.10, 95%CI: 1.03-1.17), LVO (aOR: 14.88, 95%CI: 2.33-94.97), and log10 D-dimer (aOR: 3.38, 95%CI: 1.01-11.26). CONCLUSION: Upon discharge, 40% of SARS-CoV-2 infected patients with ischemic stroke and TIA had poor outcomes. Poor outcomes were associated with older age, IHD history, moderate to severe pneumonia, higher NIHSS scores, LVO, and higher log10 D-dimer. REGISTRATION: UMIN Clinical Trials Registry: https://www.umin.ac.jp/ctr/. Unique identifier: UMIN000041226.

Repeated Acute Exacerbations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Accompanied by Pain and Swelling in Distal Extremities.

An 81-year-old man experienced acute progression of weakness in the extremities accompanied by a fever, tenderness, and swelling in distal parts of the extremities. He had flaccid tetraparesis with fasciculations and general hyporeflexia. Nerve conduction studies indicated demyelinating sensorimotor neuropathy. A cerebrospinal fluid examination revealed elevated proteins without pleocytosis. Immunological treatments were effective, but his symptoms exhibited repeated relapse and remission phases. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with an acute onset. The highlight of this case is pain with inflammatory reaction recognized as red flags of CIDP, with the clinical course and electrophysiological findings compatible with CIDP.

A Case of Cardiogenic Stroke With a Novel LMNA Variant (c. 1135C>A; p.Leu379Ile).

Laminopathy is muscular dystrophy caused by an LMNA gene mutation. It is characterized by cardiac disease such as atrial fibrillation. We report a case of laminopathy in a 49-year-old woman who presented with cardiogenic stroke. She had experienced weakness in her limb-girdle muscles since childhood, atrial fibrillation, cardiomyopathy, and mild contracture of the ankle joints, and had a familial history of heart disease. Gene analysis identified a novel heterozygous variant, c. 1135C>A (p.Leu379Ile), in the LMNA gene. Laminopathy can be an underlying disease in ischemic stroke, especially in young to middle age.

Present State of the Infection Protection System and Effects of a Change in the In-Hospital System in Endovascular Treatment for Large Vessel Occlusion in Acute Stroke Patients at a Single Institution.

Objective: Endovascular treatment (EVT) for large vessel occlusion in acute ischemic stroke patients during the SARS-CoV-2 epidemic requires the implementation of an in-hospital system to guard against infection. Changes to this system may be needed upon aggravation of the epidemic in a particular region. The objective of this study was to clarify the present state of infection protection and the effects of a change in the in-hospital system in EVT at a single institution. Methods: The subjects were consecutive patients treated by EVT under the protocol of infection protection using medical history and chest CT at our hospital between April 2020 and February 2021. For the subjects, background factors, time metrics, including door-to-puncture time (D2P), clinical outcome, and success of infection protection for medical staff were examined. The patients were divided into a group of those with PCR measurement after EVT (Group C; from April 2020 to November 2020) and a group of all with PCR measurement before EVT (Group P; from December 2020 to February 2021). Time metrics and clinical outcome were compared between the groups. Results: There were 69 subjects, including 40 and 29 patients in groups C and P, respectively. The median age was 82, which was higher in group P. The median D2P was 70 min, which did not differ significantly between the two groups, but it was slightly longer in group P than in group C by multivariate analysis. A favorable outcome (modified Rankin Scale 0-2 at 3 months) was observed in 23 patients (38%), which did not differ significantly between the two groups, but the rate of a favorable outcome was slightly lower in group P than in group C by multivariate analysis. Although medical staff wearing full personal protection equipment were needed for 15 patients (22%), 12 of whom were suspected of being positive and three (4%) were confirmed positive for SARS-CoV-2 by PCR, no staff member who participated in EVT was infected. Conclusion: The median D2P was 70 min and 38% had a favorable outcome of EVT under the present state of infection protection. After a change in the in-hospital system for clinical settings during the SARS-CoV-2 epidemic, the D2P increased and the rate of a favorable clinical outcome slightly decreased, but both were not significantly affected and infection protection for medical staff was effective. Therefore, the effects of a change were acceptable considering the circumstances.

Predictors of Reperfusion and 90-day Functional Outcome After Mechanical Thrombectomy for Large Vessel Occlusion Strokes.

OBJECTIVES: Whether elderly patients with adverse comorbidities or strong vascular meandering benefit from mechanical thrombectomy to the same degree as patients who participated in the pivotal randomized controlled trials on this procedure (MR CLEAN, ESCAPE, EXTEND-IA, SWIFT PRIME, REVASCAT, DAWN, and DEFUSE 3) remains unknown. We aimed to investigate the predictors of reperfusion and 90-day functional outcome using real-world clinical data, without excluding elderly patients with adverse comorbidities or patients in whom vascular access could not be achieved. MATERIALS AND METHODS: We retrospectively reviewed consecutive patients with acute ischemic stroke who underwent or in whom mechanical thrombectomy was attempted at Japanese Red Cross Matsue Hospital from April 2015 to June 2020. RESULTS: Altogether, 111 mechanical thrombectomies in 111 patients (average age 77.2 years) were attempted for acute ischemic stroke. Vascular access was not achieved in 8 (7.2%) cases. In the multivariable analysis, age ≥85 years (odd ratio [OR] 0.191, 95% confidence interval [CI] 0.057-0.641, p = 0.007) and presence of adverse comorbidities (OR 0.265, 95% CI 0.090-0.659, p = 0.016) were associated with failed reperfusion. The diffusion-weighted imaging (DWI)-ASPECT score ≥6 (OR 4.650, 95% CI 1.610-13.40, p = 0.005) was associated with good 90-day functional outcomes. Presence of adverse comorbidities was not a predictor, but it had a relatively strong correlation with poor functional outcome. CONCLUSIONS: Mechanical thrombectomy in elderly patients should be considered very carefully if they are aged ≥85 years, have low DWI-ASPECT score and have clear evidence of pre-existing adverse comorbidities.

Cerebrovascular imaging of cerebral ischemia in acute type A aortic dissection.

BACKGROUND AND PURPOSE: Stanford type A aortic dissection (AAD) sometimes causes acute ischemic stroke (AIS) or transient ischemic attack (TIA). There is little understanding of cerebrovascular imaging of AIS or TIA in patients with AAD. METHODS: Consecutive AIS/TIA patients with AAD who were admitted within 4.5 h of onset were reviewed. We compared findings of MRI/MRA between these and consecutive AIS/TIA patients without AAD within 4.5 h of onset. RESULTS: Seventeen AAD and 249 non-AAD patients were identified. Compared to non-AAD patients, AAD patients had infarcts more frequently in the right anterior cerebral artery (ACA) territory (18% vs. 2%, P = 0.007) and the right middle cerebral artery (MCA) territory (71% vs. 29%, P < 0.001). There was no difference between the groups regarding whether it was perforator or cortical infarct, single or multiple infarcts, unilateral or bilateral infarcts, or ischemic change extension. On the MRA imaging, the AAD patients more frequently had poor visualization of the right internal carotid artery (ICA) (47% vs. 6%, P < 0.001). After adjustment for sex, age and confounding factors, the right ACA territory infarct [odds ratio (OR), 12.2; 95% confidence interval (CI), 1.4-119.4], the MCA territory infarct (OR, 4.9; 95% CI, 1.0-25.0) and poor visualization of the right ICA (OR, 18.1; 95% CI, 4.0-101.9) were independently associated with AAD. CONCLUSION: In emergency AIS/TIA patients, right anterior circulation infarct and poor visualization of the right ICA on cerebrovascular imaging are potential imaging markers of AAD.

Urgent Detection of Acute Type A Aortic Dissection in Hyperacute Ischemic Stroke or Transient Ischemic Attack.

BACKGROUND AND PURPOSE: Urgent diagnosis of acute Stanford type A aortic dissection (AAD) in patients with acute ischemic stroke (AIS) or transient ischemic attack (TIA) is sometimes challenging. We assessed predictive values for markers of AAD in patients with AIS or TIA. METHODS: Consecutive patients with AIS or TIA with AAD who presented to our emergency room within 4.5 hours of symptom onset between 2007 and 2014 were compared with patients without AAD seen between 2012 and 2014. RESULTS: Data were obtained for 24 patients with AIS or TIA with AAD (15 women; mean age, 75 ± 12 years) and 812 patients without AAD (305 women; mean age, 73 ± 12 years). Compared with patients without AAD, patients with AAD displayed significantly higher systolic blood pressure (SBP) laterality (30 ± 20 mm Hg versus 12 ± 11 mm Hg), initial D-dimer concentration (median 38.1 µg/mL versus 1.3 µg/mL), and mediastinal width-to-chest width (M/C) ratio on x-ray (.35 ± .05 versus .29 ± .05), and more frequently showed common carotid artery (CCA) dissection on carotid ultrasonography (84% versus 1%) and pericardial effusion on echocardiography (43% versus 0%). Sensitivity and specificity to identify AAD were 80% and 75% for SBP laterality 17 mm Hg or greater; 100% and 86% for D-dimer concentration 4.1 µg/mL or greater; 75% and 76% for M/C ratio .32 or greater; 84% and 99% for CCA dissection; and 43% and 100% for pericardial effusion, respectively. CONCLUSIONS: High D-dimer level may provide the most reliable screening test for AAD in patients with AIS or TIA. CCA dissection on ultrasonography appears to represent the most disease-specific finding and shows acceptable sensitivity.

Transient Prosopometamorphopsia Restricted to the Left Eye Caused by Ischemia at the Right Splenium of the Corpus Callosum.

We herein report a patient who developed transient prosopometamorphopsia restricted to the left eye caused by ischemia of the right splenium of the corpus callosum. A 66-year-old right-handed woman suddenly noticed that the left eyes of people she encountered appeared markedly adducted to their noses. On emergent admission, neurological and ophthalmological examinations revealed no abnormalities. Diffusion-weighted magnetic resonance imaging showed a small, hyperintense lesion at the right splenium of the corpus callosum. In this case, information on the right visual field projected to the left occipital lobe might have been obstructed on transmission to the right hemisphere through the splenium of the corpus callosum.

Characteristic Posterior-dominant Lower Limb Muscle Involvement in Limb-girdle Muscular Dystrophy due to a DNAJB6 Phe93Leu Mutation.

A 41-year-old man presented with gradually progressing proximal-dominant lower limb atrophy and weakness. His brother, mother and maternal aunt had the same symptoms. A physical examination and muscle imaging (CT and ultrasound) showed selective muscle involvement of the bilateral paraspinal, gluteus and posterior groups of lower limb muscles. Based on the characteristic muscle involvement pattern, the clinical findings and the muscle biopsy results, we made a straightforward diagnosis of limb-girdle muscular dystrophy (LGMD) due to a DNAJB6 Phe93Leu mutation based on a targeted gene analysis. In the differential diagnosis of adult-onset LGMD syndromes, in addition to investigating the family history, it is important to perform an extensive physical examination to determine the pattern of muscle involvement, and to perform a muscle biopsy. Our case suggests that posterior-dominant lower limb muscle impairment with gluteus and truncal muscle involvement and the detection of rimmed vacuoles on a muscle biopsy could be clues for the diagnosis of LGMD due to DNAJB6 mutations.

Rapid Identification of Type A Aortic Dissection as a Cause of Acute Ischemic Stroke.

BACKGROUND AND PURPOSE: Patients with acute aortic dissection (AAD) sometimes present predominantly with neurological symptoms from cerebral ischemia. Such stroke patients must not receive thrombolysis therapy, which can be fatal. However, patients remain at risk if there is a failure to notice concurrent AAD. We aimed to clarify the characteristics of AAD patients with stroke to identify markers for early AAD detection before thrombolysis. METHODS: Using the single-center database of Stanford type A-AAD patients between 2007 and 2013, we selected those presenting with acute focal neurological deficits, presumably due to cerebral ischemia. Results of physical, radiological, and blood examinations were assessed in AAD patients with stroke. RESULTS: Of 226 AAD patients, 23 (10%) had stroke secondary to AAD. Of the 23 patients, 21 (91%) were primarily examined by stroke physicians and 2 (9%) by cardiologists. Thirteen patients (57%) were potential candidates for intravenous thrombolysis. Only 11 patients (48%) complained of chest/back pain. Positive findings indicating AAD included occlusion or intimal flap of the common carotid artery on carotid ultrasound in 18 (90%) of 20 patients, elevated serum d-dimer values (≥6.9 µg/mL) in 18 (78%) of 23, left hemiparesis as a neurological symptom in 17 (74%) of 23, systolic blood pressure differential above 20 mmHg between the arms in 15 (71%) of 21 patients, and mediastinal widening on chest radiograph in 10 (67%) of 15 patients. All 14 patients who underwent complete evaluation showed 2 or more positive diagnostic findings. CONCLUSIONS: The combination of physical, radiological, and laboratory findings may be a useful rapid-screening method for AAD as a cause of acute ischemic stroke.

[A case of Hashimoto's encephalopathy successfully treated with oral steroid therapy, resistant to high-dose methylprednisolone, plasma exchange and intravenous immunoglobulin].

A 30-year-old woman was admitted to the first institution with subacutely progressive aphasia and depression. Despite of lacking conclusive evidence on magnetic resonance imaging, cerebrospinal fluid examination, or electroencephalogram, we tentatively diagnosed her disease as limbic encephalopathy due to its acute progression. High-dose methylprednisolone was started on admission. However, symptoms did not improve. To make matters worse, psychiatric symptoms, such as hallucinations and emotional incontinence, appeared on the same day. Additional treatment with plasma exchange and intravenous immunoglobulin administration was also ineffective. Therefore, we could not manage the patient in a general ward due to severe psychiatric symptoms. The patient was transferred to a psychiatric ward in the second institution. She received both psychiatric treatment and steroid therapy, including a second course of intravenous high-dose methylprednisolone, followed by long-term oral prednisolone. Her symptoms gradually improved. A final diagnosis of Hashimoto's encephalopathy was made based on the patient's clinical course and positive results for both serum anti-thyroid antibody and anti-NAE antibody. In our case, long-term oral steroid therapy under psychiatric treatment was effective for good outcome.

[A case of pure alexia and foreign accent syndrome following acute encephalopathy in the presence of anti-glutamate receptor antibodies].

A 41-year-old right-handed woman presented abnormal behavior two weeks after suffering from headache and fever. Anti-glutamate receptor antibodies in the serum and cerebrospinal fluid were positive and we diagnosed anti-glutamate receptor antibody-related encephalopathy. The patient improved after administration of corticosteroid and was discharged without neurological deficit. After discharge, pure alexia and foreign accent syndrome-like language disturbance appeared consecutively. The serial fluorodeoxyglucose-positron emission tomography scans suggested that pure alexia and FAS-like language disturbance may have been caused by low function of the occipital lobes and the left frontal lobe, respectively. FAS has been linked to various lesions in the brain. The background mechanism may therefore be heterogeneous. On the other hand, patients with this syndrome recover spontaneously with our case. FAS may therefore be a temporal phenomenon resulting from imbalance in language processing rather than a specific deficit.

Parasympathetic Dominant Autonomic Dysfunction in Charcot-Marie-Tooth Disease Type 2J with the MPZ Thr124Met Mutation.

We herein report the case of a 69-year-old woman with Charcot-Marie-Tooth Disease type 2J (CMT2J) who presented with Adie's pupil, deafness, and urinary disturbance in addition to motor symptoms. On autonomic investigation, the coefficient of variation of the R-R intervals was decreased, and a urodynamic analysis showed a hypotonic bladder. A heart rate variability analysis revealed a decreased high frequency component and low frequency/high frequency ratio. Orthostatic hypotension was not present, and the sympathetic skin response and cardiac scintigraphy using (123)I-metaiodobenzylguanidine were normal. A gene analysis showed a known heterozygous mutation associated with CMT2J in myelin protein zero exon 3, resulting in the substitution of threonine to methionine at position 124. Our case suggests that mainly the parasympathetic autonomic function is disturbed in CMT2J.

Acupuncture stimulates the release of serotonin, but not dopamine, in the rat nucleus accumbens.

Acupuncture has been introduced as one of the available therapies widely used in alternative medicine, but it has not achieved widespread acceptance with scientific evidence. Furthermore there are still many unanswered questions about the basic mechanisms of acupuncture. To investigate the neuropharmacological mechanisms of oriental acupuncture, we studied the acupuncture-induced changes of in vivo monoamine release in the rat brain. A microdialysis guide cannula was implanted into the nucleus accumbens (ACC), which plays an important role in the brain reward system. Acupuncture treatment at the unilateral or bilateral Shenshu (bladder urinary channel 23) acupoints, located on the both sides of the spinous processes on the lower back, was carried out for 60 min in freely moving rats, and the dopamine (DA) and serotonin (5-HT) contents of the microdialysates in the ACC were measured simultaneously. In rats subjected to acupuncture at bilateral Shenshu acupoints, increases of 5-HT release in the ACC were observed at 20 min of acupuncture treatment and continued until 40 min after acupuncture was ended. Acupuncture at a unilateral Shenshu acupoint increased the release of 5-HT at 20 min compared with that in the sham-control group. Five-HT release returned to the baseline level at 120 min. The effects of acupuncture at bilateral Shenshu acupoints on the release of 5-HT in the ACC were greater than that of unilateral acupuncture treatment. In contrast, DA release in the ACC was not changed following acupuncture treatment. Effective acupuncture increased and prolonged the activity of serotonergic neurons in the reward system pathway of the brain. This suggests that oriental acupuncture therapy may be effective for the treatment of emotional disorders, drug abuse and alcoholism.