Correlation between clinical symptoms and striatal DAT uptake in patients with DLB

Objective

It is widely known that there is low striatal ¹²³I-FP-CIT dopamine transporter-single photon emission tomography (DAT-SPECT) uptake in patients with dementia with Lewy bodies (DLB). We assessed the correlation between symptom and regional low DAT uptake in the striatum.

Methods

Patients with Alzheimer’s disease (AD) (n?=?95) and patients with DLB (n?=?133) who underwent DAT-SPECT were enrolled. We examined the correlation between symptoms [cognitive function decline, fluctuations, visual hallucinations, parkinsonism, and REM sleep behavior disorder (RBD)] and regional striatal DAT uptake in the patients with DLB.

Results

When comparing the DLB patients with or without fluctuations, visual hallucinations, or RBD, there were no significant differences in DAT uptake in any regions of the striatum. DLB patients with parkinsonism had significantly lower DAT uptake in entire striatum, entire putamen, and anterior putamen compared to DLB patients without parkinsonism. Moreover, there was weak but significant correlation between severity of parkinsonism and DAT uptake in entire regions of the striatum in patients with DLB. There was no significant correlation between cognitive function and DAT uptake in any regions of the striatum in patients with DLB.

Conclusions

In patients with DLB, only parkinsonism is associated with a reduction in striatal DAT uptake.

     

Cardiovascular dysautonomia in de novo Parkinson's disease

BACKGROUND: Clinical symptoms of Parkinson's disease (PD) include not only motor distress, but also autonomic dysfunction. OBJECTIVE: To clarify the progression of autonomic nervous dysfunction in PD. METHODS: The subjects were 44 patients with de novo PD. Autonomic nervous function, including cardiac sympathetic gain, was evaluated on the basis of cardiac radioiodinated metaiodobenzylguanidine (MIBG) uptake, the response to the Valsalva maneuver, and spectral analyses of the RR interval and blood pressure. RESULTS: Decreased cardiac MIBG uptake was found even in patients with early stage PD. MIBG uptake gradually decreased with increased disease severity. Hemodynamic studies using the Valsalva maneuver revealed that patients with early stage PD had reduced baroreceptor reflex sensitivity (BRS) in phase II, but not phase IV. Blood pressures normally rose in phases II and IV, but the increments decreased with disease progression. In early stage PD, the low frequency power of the RR interval (RR-LF) and the ratio (LF/HF) of RR-LF to the high frequency component of the RR interval (RR-HF) were significantly lower than the respective control values, despite no significant difference in RR-HF; these variables decreased with disease progression. CONCLUSION: Our results show that latent sympathetic nervous dysfunction without parasympathetic dysfunction, especially that involving the sinus node, is already present in early stage de novo PD. It is unclear whether the responsible lesion is central or peripheral.     

Isolated cortical tuber in an infant with genetically confirmed tuberous sclerosis complex 1 presenting with symptomatic West syndrome

Tuberous sclerosis complex (TSC) is an autosomal dominant hereditary disorder caused by mutations in either TSC1 on chromosome 16 or TSC2 on chromosome 9, clinically characterized mainly by facial angiofibroma, epilepsy, and intellectual disability. Cortical dysplasias, subependymal nodules, and subependymal giant cell astrocytoma are characteristic central nervous system lesions among 11 major features in the current clinical diagnostic criteria for TSC. We encountered an unusual case of genetically confirmed TSC1 presenting with symptomatic West syndrome due to an isolated cortical dysplasia in the left occipital lobe of a six‐month‐old male infant who did not meet the clinical diagnostic criteria for TSC. The patient underwent left occipital lesionectomy at age 11 months and has been seizure‐free for nearly six years since then. Histological examination of the resection specimen revealed cortical neuronal dyslamination with abundant dysmorphic neurons and ballooned cells, consistent with focal cortical dysplasia (FCD) type IIb. However, the lesion was also accompanied by unusual features, including marked calcifications, dense fibrillary gliosis containing abundant Rosenthal fibers, CD34‐positive glial cells with abundant long processes confined to the dysplastic cortex, and multiple nodular lesions occupying the underlying white matter, consisting exclusively of ballooned cell and/or balloon‐like astrocytes with focal calcifications. Genetic testing for TSC1 and TSC2 using the patient's peripheral blood revealed a germline heterozygous mutation in exon 7 (NM_000368.5: c.526dupT, p.Tyr176fs) in TSC1. Isolated FCD with unusual features such as calcification, dense fibrillary gliosis, Rosenthal fibers and/or subependymal nodule‐like lesions in the white matter may indicate the possibility of a cortical tuber even without a clinical diagnosis of TSC. Identification of such histopathological findings has significant implications for early and accurate diagnosis and treatment of TSC, and is likely to serve as an important supplementary feature for the current clinical diagnostic criteria for TSC.     

A case of seizures induced by abstract reasoning

We describe a case of reflex seizures induced by abstract reasoning but not other cognitive processes. The patient, a 46-year-old man, experienced myoclonic seizures whenever he played shogi (Japanese chess). To identify the critical thought processes responsible for inducing his seizures, we monitored his clinical seizures and epileptiform discharges while he performed comprehensive neuropsychological tests, including the Wechsler Adult Intelligence Scale—Revised (WAIS-R), spatial working memory, mental rotation, and Wisconsin Card Sorting Test (WCST) tasks. A myoclonic seizure occurred only during the WCST. Generalized 3- to 5-Hz spike-and-slow-wave bursts occurred repeatedly during the Block Design subtest of the WAIS-R and the WCST, whereas no discharges occurred during other subtests of the WAIS-R including the calculation, spatial working memory, and mental rotation tasks. These results indicate that abstract reasoning, independent of other cognitive processes, could induce the patient’s epileptiform discharges, suggesting that his reflex seizures might be a distinct subtype of nonverbal thinking-induced seizures.     

Concordance and Discordance Between Brain Perfusion and Atrophy in Frontotemporal Dementia

The aim of this study was to determine if a dissociation between reduced cerebral perfusion and gray matter (GM) atrophy exists in frontotemporal dementia (FTD). The study included 28 patients with FTD and 29 cognitive normal (CN) subjects. All subjects had MRI at 1.5 T, including T1-weighted structural and arterial spin labeling (ASL) perfusion imaging. Non-parametric concordance/discordance tests revealed that GM atrophy without hypoperfusion occurs in the premotor cortex in FTD whereas concordant GM atrophy and hypoperfusion changes are found in the right prefrontal cortex and bilateral medial frontal lobe. The results suggest that damage of brain function in FTD, assessed by ASL perfusion, can vary regionally despite widespread atrophy. Detection of discordance between brain perfusion and structure in FTD might aid diagnosis and staging of the disease.     

Angiospermic Changes of Coronary Aneurysms in Kawasaki Disease

The fate of coronary aneurysms in Kawasaki disease or MCLS was examined by follow-up studies on 9 patients with multiple bilateral aneurysms in the coronary arteries demonstrated by coronary arteriography. Their ages at the time if first examination ranged from 0.3 to 11 years (mean, 4.1 years). The mean interval between the onset of acute illness and the initial examination was 15.8 months, and the mean interval between the first and second examination was 17.9 months. Eight of the nine patients received anticoagulants between examinations. The second coronary arteriogram showed morphologic changes of the aneueysm from the time of the first examination: changes were seen in the right coronary artery area in 8 patients (89%) and in the left coronary artery area in 7 patients. These morphologuic changes seemed to be mainly secondary to thrombus formation, calcification, stenosis and/or obstruction of the aneuryms. Thus, coronary aneuryms in Kawasaki disease were transformed despite the use of anticoagulants. It is considered that multiple large coronary aneurysms will not healed or disappear, but are simply transformed as a result of their thrombosis and/or calcification.     

The effect of paroxetine on Taijinkyofusho: a report of three cases

Taijinkyofusho is a culture-related syndrome conceptualized in Japan. While previous studies suggest its psychopathological similarities to social phobia and obsessive-compulsive disorder, introspection regarding shame and low self-esteem is particularly linked to Japanese culture. We present three cases of Taijinkyofusho: Cases 1 and 2 show neurotic features while Case 3 shows delusional thoughts. Paroxetine was used for treatment but was productive in only the first two cases. Phobic and obsessive thought patterns were altered in Cases 1 and 2, suggesting that the significant core symptoms were responding to the treatment. In the future, large-scale pharmacological studies will be necessary to investigate treatment outcomes Taijinkyofusho. Such studies would contribute to providing information for effective treatment as well as for examining relationships between Taijinkyofusho and related disorders.