Akane Nogami

Distribution of α-synuclein in the spinal cord and dorsal root ganglia in an autopsy cohort of elderly persons.

BackgroundLewy body–related α-synucleinopathy (LBAS, the abnormal accumulation of pathologic α-synuclein) is found in the central and peripheral nervous systems, including the spinal cord, dorsal root ganglia, and sympathetic ganglia, of Parkinson’s disease patients. However, few studies have focused on the distribution of LBAS in the spinal cord, primary sensory neurons, and preganglionic sympathetic nerves.ResultsWe analyzed 265 consecutive subjects with LBAS who underwent autopsy at a general geriatric hospital. LBAS in the spinal cord was significantly associated with that in the lower brainstem regions that are directly connected to the spinal cord (i.e., the medullary reticular formation and locus ceruleus), but it was not associated with the olfactory bulb–amygdala system, which is not directly connected to the spinal cord, suggesting that the lower brainstem is a key structure regarding the spread of LBAS to the spinal cord. In the primary sensory neurons, most subjects with LBAS in the dorsal root ganglia had LBAS in the dorsal root, and all subjects with LBAS in the dorsal root also had LBAS in the dorsal horn, suggesting that LBAS spreads retrogradely from the axonal terminals of the dorsal horn to the somata of the dorsal root ganglia via the dorsal root. In the preganglionic sympathetic nerves, the LBAS in the sympathetic ganglia preceded that in the nucleus of the intermediolateral column of the thoracic cord, suggesting that LBAS spreads retrogradely through the preganglionic sympathetic nerves.ConclusionsLBAS in the spinal cord was associated with the lower regions of the brainstem, but not with the olfactory bulb or amygdala. LBAS may spread centrifugally along the primary sensory neurons, whereas it may spread centripetally along the preganglionic sympathetic nerves.

Limb-shaking TIA: Cortical myoclonus associated with ICA stenosis

Limb-shaking associated with steno-occlusion of the internal carotid artery (ICA) was first reported by Miller Fisher1 in 1962, and is characterized by brief, jerky, coarse, involuntary movements involving an arm or leg. Limb-shaking TIA is an under-recognized manifestation of an intracranial and extracranial carotid occlusion or severe stenosis. Although hemodynamic compromise has been suggested to be associated with shaking movements, the pathogenic nature of this symptom remains unclear. Neurophysiologic evaluation of this movement disorder would be useful, but the attack is rare and typically lasts less than 5 minutes.2 Thus, few reports have investigated this hyperkinetic phenomenon under neurophysiologic assessment.3

Spontaneous Bilateral Cervical Internal Carotid and Vertebral Artery Dissection in a Japanese Patient without Collagen Vascular Disease with Special Reference to Single-Nucleotide Polymorphisms

Spontaneous cervical artery dissection (sCAD) is a major cause of ischemic stroke in young adults. Frequently, sCAD involves multiple neck arteries, accounting for 13%-28% of the total sCAD cases. However, little is known about factors related to multiple sCAD. In this case, a 52-year-old man was admitted due to headache without aura. There was a personal history of migraine with aura and a family history of similar symptoms. The patients younger brother had a left vertebral artery (VA) dissecting aneurysm and underwent endovascular occlusion of his parent artery at the age of 48. Magnetic resonance imaging of our admitted patient showed hyperintensities in the right internal carotid artery (ICA) without acute infarction, and magnetic resonance angiography revealed a narrowing of the right ICA. Angiography was then performed, which showed a trace of dissection of the left ICA and both VAs as well as the right ICA. The patient did not fulfill any major criteria of collagen vascular disease such as Ehlers-Danlos syndrome type IV or Loeys-Dietz syndrome. The data in our patient are quite similar to those reported in patients with single-nucleotide polymorphism (SNP) of PHACTR1. Obtaining the patients informed consent, we analyzed a common SNP variation in the rs9349379[G] allele (PHACTR1), which has been reported to be associated with a lower risk of sCAD.

Multiple Cerebral Infarctions in a Patient with Adenomyosis on Hormone Replacement Therapy: A Case Report.

A 59-year-old woman was admitted to our hospital because of repeated episodes of bilateral hand weakness. She had a 10-year history of combined estrogen-progestin therapy for menopausal symptoms. Magnetic resonance imaging on admission showed multiple hyperintense lesions in bilateral cerebral and cerebellar cortices on diffusion-weighted imaging. Transesophageal echocardiography showed thrombus formation on the aortic valve and moderate aortic insufficiency. Laboratory test demonstrated elevated CA125 (334.8 U/mL) and D-dimer (7.0 µg/mL) levels. Trousseaus syndrome (cancer-related hypercoagulation) was considered, but various examinations showed only uterine adenomyosis and no evidence of cancer. Multiple cerebral infarctions were considered to be caused by Trousseaus syndrome-like condition associated with uterine adenomyosis. CA125 and coagulation markers should be measured in adenomyosis patients treated with hormone replacement therapy, because a mucinous tumor and coagulation markers may be good markers for the risk of thromboembolism in such patients.

Reduction of Small Fibers of Thoracic Ventral Roots and Neurons of Intermediolateral Nucleus in Parkinson Disease and Dementia with Lewy Bodies

BACKGROUND AND OBJECTIVE Loss of intermediolateral nucleus (IML) neurons is considered to play a major role in orthostatic hypotension (OH) of multiple system atrophy (MSA). In Parkinson disease (PD) and dementia with Lewy bodies (DLB), autonomic phenomena such as OH are common and attributed to dysfunction of sympathetic, parasympathetic, and visceral autonomic neurons. However, apart from MSA, few reports have focused on the neuropathologic aspects in PD and DLB. Here we assessed IML degeneration as well as the fine myelinated fibers (FFs; maximum diameter less than 3 μm) considered to be preganglionic sympathetic nerve fibers derived from IML neurons in PD, DLB, MSA, and age-matched normal controls (NC). METHODS We counted IML neurons and measured the diameter and number of myelinated fibers of the ventral root at the level of the 12th thoracic segment. RESULTS Compared to NC, number of IML neurons and density of FF were significantly reduced in PD (53% and 67%), DLB (47% and 71%) and MSA (27% and 42%). Compared to combined group of PD and DLB without OH (OH-), IML neurons in combined group of PD and DLB with OH (OH+) were significantly reduced (77%). Compared to NC, FF densities in OH-, OH+ were significantly reduced (74% and 59%). The mean ratio of small to large myelinated fibers in OH+ (1.18), but not that in OH-(1.58), was significantly lower than that in NC (3.17). CONCLUSIONS We present neuropathological evidence that IML neurons and FFs in the ventral root are reduced in PD and DLB and that the reduction was more severe in the combined group of OH+ than in OH-.

INCIDENCE AND PREDICTIVE FACTORS OF COGNITIVE DYSFUNCTION SIX MONTHS AFTER ACUTE STROKE

Background: There is general acknowledgement of the importance of early diagnosis of dementia, yet there are still high rates of undetected dementia internationally. The aim of this crosssectional study was to determine the sociodemographic characteristics associated with possible undetected dementia in a large sample of community-dwelling older New Zealanders. Methods: The sample consisted of older people (age 65) who had received the homecare version of the international Residential Assessment (interRAI HC 9.1) over a 2-year period and who were screened positive for possible dementia on the interRAI’s Cognitive Performance Scale. People with possible alternative explanations for cognitive impairment such as depression and other neurological conditions were excluded. The 5202 eligible individuals were categorized into two groups: (1) those with a recorded diagnosis of dementia (64%) and (2) those without a recorded diagnosis of dementia (36%). Logistic regression was used to evaluate the association between individual characteristics and possible undetected dementia. Results: The mean age for the possible undetected dementia group (UDG) was significantly higher than the diagnosed dementia group (DDG) (83.6 years, SD67.7 vs 82.6 years, SD67.0; p1⁄40.000). Compared with the DDG, subjects in the possible UDG were less likely to be married or in a de facto relationship (37.6% vs 54.5%, p1⁄40.000) and more likely to be living alone (42.8% vs 26.4%, p1⁄40.000) (OR1⁄42.01, CI1⁄41.75-2.30). Compared with the DDG, subjects in the possible UDG were significantly (i) less likely to have participated in social activities of long standing interest (42.6% vs 52.6%, p1⁄40.000) (OR1⁄41.37, CI1⁄41.211.56), (ii) more likely to have experienced major life stressors (52.1% vs 35.2% p1⁄40.000) (OR1⁄41.88, CI1⁄41.65-2.14), and (iii) more likely to report limited access to home or rooms in home (8.6% vs 5.9%, p1⁄40.000) (OR1⁄41.44, CI1⁄41.13-1.84). Ethnicity had a significance effect on the diagnosis of dementia (p1⁄40.012); Asian people has a greater risk of being in the possible undetected dementia group (OR1⁄41.91, CI1⁄41.33-2.74). Conclusions: Our results could help to identify the individual characteristics that play a role in the under-diagnosis of dementia in the local context, and guide interventions to promote early diagnosis in vulnerable groups. P2-300 INCIDENCE AND PREDICTIVE FACTORS OF COGNITIVE DYSFUNCTION SIX MONTHS AFTER ACUTE STROKE

Remote intracerebral hemorrhage followed by re-bleeding after intravenous thrombolysis in a patient taking a non-vitamin K antagonist oral anticoagulant: A case report

A 92-year-old woman was admitted to our stroke center 40 min after onset of left-sided weakness and dysarthria. She had a history of hypertension, diabetes mellitus, and atrial fibrillation, and she took rivaroxaban 10 mg once daily and dipyridamole 25 mg twice daily. Her blood pressure (BP) was 174/80 mmHg, and her heart rate was 76 beats/min with an irregular rhythm. The electrocardiogram showed atrial fibrillation. Neurological examination revealed a disturbance of consciousness (Glasgow Coma Scale: E3, V4, M6), dysarthria, leftsided weakness and sensory disturbance, and left unilateral spatial neglect. The total National Institutes of Health stroke scale (NIHSS) score on admission was 11. Laboratory data showed that the prothrombin time-international normalized ratio (PT-INR) was 1.44. Her estimated glomerular filtration rate was 40–50 ml/min, and her estimated creatinine clearance by the Cockcroft-Gault equation was 22–29 ml/min, based on laboratory and anthropometric data on admission through Day 3. Magnetic resonance imaging (MRI) on admission showed a hyperintense lesion in the right middle cerebral artery (MCA) territory on diffusion-weighted imaging and right MCA branch occlusion. A cardioembolic stroke was diagnosed because of the presence of atrial fibrillation, and intravenous (IV) tissue-plasminogen activator (t-PA) therapy was started 2 h after symptom onset and about 16 h after taking a rivaroxaban10mg tablet, based on our domestic guideline [1]. Her symptoms improved gradually after the IV t-PA therapy, but computed tomography (CT) 6 h after IV t-PA therapy showed an asymptomatic remote ICH in the right cerebellum (Fig. 1, C). There were no ischemic lesions or microbleeds in the right cerebellum on admission (therefore prior to IV t-PA therapy), as shown on MRI (diffusionand

Thrombolysis, Complete Recanalization, Diffusion Reversal, and Luxury Perfusion in Hyperacute Stroke.

A 59-year old man was admitted to our stroke care unit 1.8 hours after onset of cardioembolic stroke. Administration of issue-plasminogen activator achieved complete recanalization, and his lesion on diffusion-weighted imaging (DWI) disappeared and single photon emission computed tomography showed luxury perfusion. DWI reversal and luxury perfusion were sometimes observed in hyperacute stroke patients, especially timely reperfusion was achieved. However, the relationships between DWI reversal and luxury perfusion were not well known. Transient DWI reversal may be associated with luxury perfusion in patients treated with t-PA, via early complete recanalization achieved by thrombolysis.