Distribution and progression of cerebral amyloid angiopathy in early-onset V30M (p.V50M) hereditary ATTR amyloidosis.

Background: Cerebral amyloid angiopathy (CAA) is becoming the most common and serious complications in long-lived hereditary ATTR amyloidosis patients. It is therefore imperative to elucidate the characteristics of ATTR-type CAA and develop useful biomarkers.

Methods: We enrolled 34 ATTRv amyloidosis patients with the V30M (p.

Non-invasive detection and differentiation of cardiac amyloidosis using Tc-pyrophosphate scintigraphy and C-Pittsburgh compound B PET imaging.

Purpose: To investigate the utility of the combined use of C-Pittsburgh compound B (C-PiB) positron emission tomography (PET) imaging and Tc-pyrophosphate (Tc-PYP) scintigraphy for detection and differentiation of three major types of cardiac amyloidosis, i.e. immunoglobulin light chain (AL), hereditary transthyretin (ATTRv), and wild-type transthyretin (ATTRwt) amyloidosis.

Late-onset Transthyretin (TTR)-familial Amyloid Polyneuropathy (FAP) with a Long Disease Duration from Non-endemic Areas in Japan.

We herein report the case of an 84-year-old woman with transthyretin (TTR) Val30Met-associated familial amyloid polyneuropathy (FAP-ATTR Val30Met), representing a very old case. The patient had muscle weakness and sensory disturbances in her extremities caused by severe peripheral neuropathy. She also had vitreous opacity and orthostatic hypotension, and pyrophosphate scintigraphy showed a myocardial accumulation.

[PRIMARY LOCALIZED AL AMYLOIDOSIS OF THE BLADDER MIMICKING RECURRENCE OF BLADDER CARCINOMA].

Primary bladder amyloidosis is a rare disease, with approximately 200 cases documented in the literature. We herein present a 85-year-old Japanese man who has undergone a transurethral resection of a bladder tumor (TURBT) and has regularly been followed up after surgery. Since cystoscopy revealed mucosal irregularity, he has got a TURBT again for a suspicion of recurrence.

Visualization of multiple organ amyloid involvement in systemic amyloidosis using C-PiB PET imaging.

Purpose: To investigate the utility of Pittsburgh compound B (PiB) positron emission tomography (PET) imaging for evaluating whole-body amyloid involvement in patients with systemic amyloidosis.

Methods: Whole-body C-PiB PET was performed in seven patients with systemic immunoglobulin light-chain (AL) amyloidosis, seven patients with hereditary transthyretin (ATTRm) amyloidosis, one asymptomatic TTR mutation carrier and three healthy controls. The correlations between clinical organ involvement, radiological C-PiB uptake and histopathological findings were analysed for each organ.

Cerebral amyloid angiopathy in posttransplant patients with hereditary ATTR amyloidosis.

Objective: To investigate the prevalence and clinical features of posttransplant CNS symptoms in patients with hereditary ATTR amyloidosis and their Pittsburgh compound B (PiB)-PET imaging correlates.

Methods: We monitored prevalence and type of CNS symptoms in 53 consecutive posttransplant patients with hereditary ATTR amyloidosis. (11)C-PiB-PET was performed in 15 patients with various disease durations.

Neural plasticity of neonatal hypoglossal nerve for effective suckling.

The adaptive movement of the tongue after unilateral lesion of the hypoglossal (XII) nerve during the early postnatal days is essential for recovery of milk intake. The present study investigated the basic mechanisms underlying such adaptation, focusing on the neural plasticity that allows effective suckling. After resection of the ipsilateral XII nerve on P1, 1,1'-dioctadecyl-3,3,3',3'-tetramethylindocarbocyanine perchlolate (DiI), a postmortem neuronal tracer, was applied to the contralateral uninjured XII nerve on P4 and P7.

Neonatal estrogen decreases neural density of the septum-midbrain central gray connection underlying the lordosis-inhibiting system in female rats.

Neurons in the lateral septum (LS) with projecting axons to the midbrain central gray (MCG) exert an inhibitory influence on lordosis. The number of such neurons is greater in female than in male rats. In this experiment, effects of neonatal estrogen on the density of the LS-MCG connections and on lordosis behavior were examined in female rats.