Persistent brain damage in reversible cerebral vasoconstriction syndrome on 99mTc-ethyl cysteinate dimer single-photon emission computed tomography: A long-term observational study.

BACKGROUND: Blood-brain barrier (BBB) breakdown is considered a key step in the pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS); however, its temporal course remains unclear. Based on the characteristics and dynamics of 99mTc-ethyl cysteinate dimer (99mTc-ECD) as a tracer, 99mTc-ECD single-photon emission computed tomography (SPECT) can detect not only hypoperfusion but also BBB breakdown and/or brain tissue damage. Therefore, this study aimed to investigate this course using 99mTc-ECD SPECT. METHODS: Between 2011 and 2019, we enrolled seven patients (one male and six female patients) with RCVS without ischemic or hemorrhagic stroke or posterior reversible encephalopathy syndrome. 99mTc-ECD SPECT was performed repeatedly in each patient. SPECT data were statistically analyzed using an easy Z-score imaging system. RESULTS: Thunderclap headache was the initial symptom in all the patients and was most commonly triggered by bathing (three patients). All the patients exhibited vasoconstriction and reduced cerebral uptake of 99mTc-ECD during the acute stage. Follow-up assessment from 3 to 16 months showed that reduced cerebral uptake persisted in all the patients, even after the vasoconstriction had resolved. CONCLUSION: Reduced cerebral uptake of 99mTc-ECD persisted in the late stage of RCVS, even after vasoconstriction and headache subsided. BBB breakdown and/or brain tissue damage may underlie this phenomenon. 99mTc-ECD SPECT is an effective neuroimaging method to detect brain functional abnormalities, reflecting BBB breakdown or tissue damages, throughout the treatment course of RCVS.

Posterior reversible encephalopathy syndrome presenting with thrombotic microangiopathy triggered by malignant hypertension: a case report and literature review.

Background: Posterior reversible encephalopathy syndrome (PRES) is a disease characterised by reversible subcortical vasogenic oedema, neurological symptoms and abnormal findings on head imaging. It is recognised as one of the most prominent organ disorders in hypertensive emergencies but is rarely associated with thrombotic microangiopathy (TMA). Case presentation: A woman in her 40s with untreated hypertension had occasional headaches in the past 4 months. The headaches worsened during the 3 weeks prior to admission. On the day of admission, the patient presented with severe headache accompanied by frequent vomiting. MRI of the head revealed oedematous changes in the brainstem, including the subcortical, cerebellum and pons. Fundus examination revealed hypertensive retinopathy with papilloedema. Blood tests indicated thrombocytopenia, renal dysfunction and haemolytic anaemia, and a blood smear confirmed fragmented erythrocytes. Coombs' test, and tests for ADAMTS13 activity and infectious and autoimmune diseases were negative. The patient was diagnosed with PRES, secondary to malignant hypertension (MH) and associated with TMA. Antihypertensive therapy promptly improved the clinical symptoms, blood pressure, and the abnormal MRI and blood test findings. The patient was discharged from the hospital 20 days after admission. Conclusions: We report a rare case of PRES that was associated with TMA and triggered by MH. Antihypertensive therapy was effective in alleviating the associated adverse clinical symptoms. Differentiation of underlying diseases is essential for early intervention, since treatment depends on factors causing TMA.

Palinopsia as an initial symptom of cerebral amyloid angiopathy-related inflammation.

•We report the first case of cerebral amyloid angiopathy-related inflammation (CAA-RI) presenting palinopsia initially.•Palinopsia is generally caused by intracranial diseases involving the parietal and occipital areas.•CAA dominantly affects parietal and occipital lobes, therefore palinopsia could be an important phenomenon of the disease.

Ten-Year Evaluation of the TOYOTA Prehospital Stroke Scale for Tissue Plasminogen Activator Intravenous Therapy in the Real World.

BACKGROUND: Emergency medical services are an important part of acute stroke management. We devised a prehospital stroke scale, the TOYOTA prehospital stroke scale for tissue plasminogen activator (t-PA) intravenous therapy (TOPSPIN) for triaging patients with ischemic stroke and especial indications for intravenous t-PA therapy in December 2006; this scale comprises 5 items including consciousness, atrial fibrillation, language disorder, disturbance of the upper extremities, and disturbance of the lower extremities. The aim of this study was to analyze the results of 10 years of TOPSPIN implementation and to distinguish ischemic stroke from hemorrhagic stroke in the real world. METHODS: We prospectively enrolled consecutive patients who were transferred to our hospital and evaluated by Toyota city ambulance services using the TOPSPIN from December 2006 to January 2017. We examined definite diagnosis at the time of hospital discharge (ischemic stroke, hemorrhagic stroke, or stroke mimic), positive-predictive value of the stroke, the rate of the recanalization therapy, and clinical differentiation among patients with hemorrhagic stroke, ischemic stroke, and stroke mimics. RESULTS: A total of 1,482 consecutive patients were enrolled; 1,134 (76.5%) were patients with stroke (628 ischemic-type, 34 transient ischemic attack-type, and 472 hemorrhagic-type) and 348 (23.5%) without stroke (80 with seizure, 42 with syncope, 27 with hypoglycemia, and 199 other). Among 628 patients with ischemic stroke, 130 (20.7%) received intravenous recombinant t-PA treatment, endovascular therapy, or both. The presence of atrial fibrillation, older age, lower blood pressure, and lower total TOPSPIN score was more commonly associated with ischemic stroke than with hemorrhagic stroke. In multivariable logistic regression analysis, the presence of atrial fibrillation was independently associated with ischemic stroke (OR 2.33; 95% CI 1.61-3.40). CONCLUSIONS: The TOPSPIN is a simple prehospital stroke scale that includes an assessment of atrial fibrillation. Detection of atrial fibrillation in the prehospital stage may point to a higher probability of ischemic stroke.

[Continuous Blood Glucose Monitoring of Hyperglycemia in Acute Stroke Patients Receiving Enteral Tube Feeding].

Severe hyperglycemia often occurs when acute stroke patients receive enteral tube feeding. Hyperglycemia should be managed because it adversely affects stroke outcome. However, it remains unclear how blood glucose level (BGL) changes with enteral nutrition in acute stroke patients. Our purpose was to investigate features of the blood glucose curve in acute stroke patients receiving enteral feeding. We retrospectively evaluated acute stroke patients who developed hyperglycemia during enteral tube feeding and underwent CGM in our institution. We evaluated characteristics of blood glucose curves, fasting and peak BGL, and time to peak of BGL. Six patients were analyzed. The median fasting BGL was 125.5 mg/dL and the median peak BGL was 351.5 mg/dL. The blood glucose curve showed the following characteristics following: 1) BGL elevated slowly reach the peak in 180 to 240 minutes; 2) BGL decreased slowly or very little after its peak; 3) there was a large gap between fasting and peak BGL regardless of presence of diabetes mellitus. We grouped the curves into four types by time to peak and how BGL decreased after peak. (Received August 17, 2017; Accepted February 1, 2018; Published July 1, 2018).

A case of intracranial arterial dolichoectasia with 4 repeated cerebral infarctions in 6 months and enlargement of basilar artery.

A 78-year-old man was admitted to our hospital because of sudden right hemiparesis and dysarthria. His cranial MRI showed an area of hyperintensity in left pons on DWI and MRA revealed dilated, elongated and tortuous intracranial artery. We diagnosed as acute phase ischemic stroke and intracranial arterial dolichoectasia (IADE). Intravenous infusion of rt-PA was performed 157 minutes after the onset of symptoms, and his hemiparesis improved. However, he subsequently suffered from cerebral infarction 4 times in 6 months, and we treated him twice with thrombolytic therapy. Although thrombolytic therapy was effective in the short term and antithrombotic therapy was continued, he had bilateral hemiplegia and severe dysphagia because of repeated cerebral infarctions. Hence basilar artery was dilated with intramural hemorrhage over 6 months, and we discontinued antithrombolytic therapy. It is possible that antithrombolytic therapy affects enlargement of IADE. Antithrombolytic therapy for IADE should be done carefully.

[A case of amyloid-ß-related cerebral angiitis with ApoE ε4/ε2 genotype].

A 53-year-old male with a past medical history of hypertension and bipolar disorder gradually developed gait disturbance and cognitive dysfunction over half a year. His cranial MRI showed an area of hyperintensity in the right occipital lobe on T2 weighted images and the surface of the lesion was enhanced along the sulci. We diagnosed his condition as amyloid-ß-related angiitis (ABRA) based on brain biopsy. Repeated, frequent seizures resistant to several antiepileptic drugs (AEDs) occurred after the operation. Steroid therapy was effective and the symptoms, including the intractable seizures and MRI abnormalities dramatically improved. In contrast to the common wild type ε3/ε3 ApoE genotype, a majority of ABRA patients have ε4/ε4. However, in this case the rare ε4/ε2 type was detected. The ε4 allele is considered to promote Aß deposition on the vessel wall, and ε2 is speculated to trigger vessel ruptures or vascular inflammation. Although seizure is not a common complication of brain biopsy, it occurred repeatedly and responded poorly to AEDs in this case. Surgical stress in this patient with ε2 probably induced the uncontrolled seizures. ApoE genotype may be an effective and low-invasive marker in case of suspected ABRA and in predicting the risks of the complication from brain biopsy.

In vivo mesolimbic D2/3 receptor binding predicts posttherapeutic clinical responses in restless legs syndrome: a positron emission tomography study.

Although D2/3 agonists have been used as a first-line medication for idiopathic restless legs syndrome (iRLS), findings on D2/3 receptors have been inconsistent. Here, we aimed to clarify the contribution of D2/3 receptor function to the clinical symptoms of iRLS by comparing the binding potential (BP(ND)) of [(11)C]raclopride with clinical improvements after D2/3 stimulation by pramipexole. Eight drug-naïve, iRLS patients and eight age-matched healthy subjects were scanned with positron emission tomography (PET). After PET scans, all patients received pramipexole (0.125 mg) orally for 2 weeks. Patients were evaluated every day with several standardized clinical tests. The BP(ND) values were compared using regions of interest and voxel-based methods. Results showed that the mean magnitude of [(11)C]raclopride BP(ND) in the mesolimbic dopamine region (nucleus accumbens (NA) and caudate) was significantly lower in the iRLS group. No significant differences between groups were observed in the putamen. The NA [(11)C]raclopride BP(ND) levels correlated negatively with clinical severity scores and positively with the degree of posttreatment improvement in iRLS. The present results suggest that alterations in mesolimbic D2/3 receptor function reflect the pathophysiology of iRLS, and the baseline availability of D2/3 receptors may predict the clinical outcome after D2/3 agonist treatment.