Echolalia in patients with primary progressive aphasia.

OBJECTIVE: This study aimed to examine echolalia and its related symptoms and brain lesions in primary progressive aphasia (PPA). METHODS: Forty-five patients with PPA were included: 19 nonfluent/agrammatic variant PPA (nfvPPA), 5 semantic variant PPA, 7 logopenic variant PPA, and 14 unclassified PPA patients. We detected echolalia in unstructured conversations. An evaluation of language function and the presence of parkinsonism, grasp reflex, imitation behaviour, and disinhibition were assessed. We also measured regional cerebral blood flow (rCBF) using single-photon emission computed tomography. RESULTS: Echolalia was observed in 12 nfvPPA and 2 unclassified PPA patients. All patients showed mitigated echolalia. We compared nfvPPA patients with echolalia (echolalia group) to those without echolalia (non-echolalia group). The median age of the echolalia group was significantly lower than that of the non-echolalia group, and the echolalia group showed a significantly worse auditory comprehension performance than the non-echolalia group. In contrast, the performance of repetition tasks was not different between the two groups. The prevalence of imitation behaviour in the echolalia group was significantly higher than that in the non-echolalia group. The rCBFs in the bilateral pre-supplementary motor area and bilateral middle cingulate cortex in the echolalia group were significantly lower than those in the non-echolalia group. CONCLUSIONS: These findings suggest that echolalia is characteristic of nfvPPA patients with impaired comprehension. Reduced inhibition of the medial frontal cortex with release activity of the anterior perisylvian area account for the emergence of echolalia.

Sentence composition ability in two patients with non-fluent/agrammatic variant primary progressive aphasia.

Agrammatism is one of the core clinical features of non-fluent/agrammatic variant primary progressive aphasia, and it has traditionally been considered the hallmark of non-fluent aphasia in Western countries. However, agrammatic speech may remain undetected in Japanese patients because of the agglutinative structure of the language and high flexibility in word order. In the present study, we aimed to analyze agrammatism in the speech production of Japanese patients with aphasia due to neurodegenerative disease using an anagram test generated by our laboratory. Four patients were recruited from the dementia clinic at Tohoku University Hospital between December 2014 and August 2015: two patients with non-fluent/agrammatic variant primary progressive aphasia, one with semantic variant primary progressive aphasia, and one with probable Alzheimer's disease experiencing episodic memory impairment accompanied by transcortical sensory aphasia. All patients underwent thorough neurological and neuropsychological testing before performing a Japanese anagram task based on the Northwestern Anagram Test. Our findings indicated that the two patients with non-fluent/agrammatic variant primary progressive aphasia exhibited poorer performance on the anagram task than the remaining two patients. Therefore, the anagram test used in the present study may aid in detecting output aspects of agrammatism in Japanese patients with aphasia, although future studies are required to develop a standardized version of test.

Damage to the Left Precentral Gyrus Is Associated With Apraxia of Speech in Acute Stroke.

BACKGROUND AND PURPOSE: Apraxia of speech (AOS) is a motor speech disorder, which is clinically characterized by the combination of phonemic segmental changes and articulatory distortions. AOS has been believed to arise from impairment in motor speech planning/programming and differentiated from both aphasia and dysarthria. The brain regions associated with AOS are still a matter of debate. The aim of this study was to address this issue in a large number of consecutive acute ischemic stroke patients. METHODS: We retrospectively studied 136 patients with isolated nonlacunar infarcts in the left middle cerebral artery territory (70.5±12.9 years old, 79 males). In accordance with speech and language assessments, the patients were classified into the following groups: pure form of AOS (pure AOS), AOS with aphasia (AOS-aphasia), and without AOS (non-AOS). Voxel-based lesion-symptom mapping analysis was performed on T2-weighted images or fluid-attenuated inversion recovery images. Using the Liebermeister method, group-wise comparisons were made between the all AOS (pure AOS plus AOS-aphasia) and non-AOS, pure AOS and non-AOS, AOS-aphasia and non-AOS, and pure AOS and AOS-aphasia groups. RESULTS: Of the 136 patients, 22 patients were diagnosed with AOS (7 patients with pure AOS and 15 patients with AOS-aphasia). The voxel-based lesion-symptom mapping analysis demonstrated that the brain regions associated with AOS were centered on the left precentral gyrus. CONCLUSIONS: Damage to the left precentral gyrus is associated with AOS in acute to subacute stroke patients, suggesting a role of this brain region in motor speech production.

Totally implantable venous access port via the femoral vein in a femoral port position with CT-venography.

BACKGROUND AND PURPOSE: We aimed to determine the success rate and any complications using a percutaneous approach to the femoral vein (FV) for placement of a totally implantable access port (TIVAP), with a preoperative assessment of the femoral and iliac veins using computed tomography-venography (CT-V). METHODS: A prospective study of 72 patients was conducted where placement of a TIVAP was attempted via the right FV, with the port placed in the anterior thigh, when subclavian vein or jugular vein access was contraindicated. Preoperative assessment of the femoral venous plexus was performed with CT-V in 72 patients. RESULTS: CT-V success was achieved in 72 of 72 patients (100%). The average distance between the inguinal ligament and the saphenofeomral (FV-GSV) junction was 42.8 ± 12.9 mm. The FV approach had a 97% successful cannulation rate. Two patients had a thrombosis in either the femoral vein or the great saphenous vein. One procedural complication (1.4%) and one initial complication (1.4%) occurred. Late complications occurred in four patients (5.7%). CONCLUSIONS: The percutaneous FV approach with CT-V guidance is an option for patients with multiple central venous cannulations, as well as those with bilateral breast cancer, or those undergoing hemodialysis. J. Surg. Oncol. 2016;114:1024-1028. © 2016 Wiley Periodicals, Inc.

Computed tomography (CT) venography using a multidetector CT prior to the percutaneous external jugular vein approach for an implantable venous-access port.

BACKGROUND AND PURPOSE: The objective of this study was to determine the success rate and complications of using the percutaneous approach of the external jugular vein (EJV) for placement of a totally implantable venous-access port (TIVAP) with a preoperative estimate of the detailed anatomical orientation of the cervical venous plexus using computed tomography venography (CT-V). METHODS: A prospective cohort study of 45 patients in whom placement of a TIVAP was attempted via the right EJV was conducted. The preoperative anatomical estimation of the cervical venous plexus was performed with CT-V using a Multidetector Helical 16-section CT. The angulation between the right EJV and the right subclavian vein, anterior jugular vein, transverse cervical vein, and suprascapular vein was estimated. RESULTS: CT-V success was achieved in 45 of 45 patients (100 %). A plexus of veins under the clavicle was most commonly responsible for the insertion of the central venous catheter. The EJV approach resulted in a successful cannulation rate of 93 %. No initial complications of pneumothorax or carotid artery puncture occurred during insertion procedures. Late complications occurred in three patients. These included one port erosion (2 %), one catheter occlusion (2 %), and one wound hematoma (2 %). Catheter-related infections were observed in one patient (2 %). CONCLUSIONS: The percutaneous EJV approach with CT-V guidance is an optional method for patients with multiple central venous cannulations, those in hemodialysis, or those with long catheter indwelling periods.

Evaluation of three types of platforms in single-incision laparoscopic surgery for performing transanal endoscopic microsurgery (SILSTEM).

BACKGROUND/AIMS: Transanal endoscopic microsurgery using a platform for single-incision laparoscopic surgery (SILSTEM) is safe for excising rectal lesions. We tested three types of platforms. METHODOLOGY: Nine patients underwent SILSTEM by one surgeon. Tumors located 5­15 cm from the anal verge were eligible. After measuring their dimensions, length, and trocar channels, the platforms tested were the SILSTM port (SP), EZTM access (EA), and GelPOINTTM Path (GP). Clinicopathology, intraoperative parameters, and postoperative outcomes were recorded. RESULTS: Six men and three women (median age 63 years) underwent SILSTEM using platform SP in three patients, EA in four, GP in two. Median operation time was 128 min (range 71­313). Median blood loss was 3 ml (range 1­71). Pathology confirmed adenocarcinoma in five patients, adenoma in three, and carcinoid in one. Patients were discharged within 2­13 days postoperatively. There was no postoperative fecal incontinence or soiling. Overall median follow-up was 13.3 months (range 1.3­27.2). There were no recurrences. CONCLUSION: SILSTEM can effectively resect rectal tumors using any of three platforms. Large prospective trials are needed to define the advantages, disadvantages, and indications for each platform and to draw conclusions regarding operation time, anorectal function, and costs.

[Dystypia in a patient with subcortical ischemic stroke].

A 58-year-old, right-handed man noticed difficulty in typing and speech. On day 3 after onset, the day of admission, he had frontal lobe dysfunction including verbal fluency impairment and impairment of recent memory, although he did not have apraxia or visual agnosia. Moreover, he had difficulty typing in romaji, especially words containing contracted or double consonant sounds, although he was able to do this before onset by visually checking the keyboard. He had mild dysgraphia. MRI showed an infarct in the genu and posterior limb of the left internal capsule. SPECT revealed low-uptake lesions in the left frontal lobe. In the present case, we consider that the subcortical infarction disrupted the network between the thalamus and frontal lobe, resulting in dystypia due to difficulty with recalling romaji spelling.

A decade with anomic primary progressive aphasia.

Some patients with primary progressive aphasia (PPA) demonstrate only anomia. The lack of longitudinal observations of anomic PPA precluded us from determining whether progressive anomic aphasia was simply an early stage of semantic or logopenic variants, or a relatively independent variant. Herein, we report the 10-year clinical course of a patient with PPA who presented with pure anomic aphasia for 9 years. He is a right-handed man with anomia, who noticed word-finding difficulty at age 73. He was admitted to the hospital at age 77. On admission, the patient showed pure anomic aphasia with preserved other language function. Episodic memory and visuospatial function were preserved. Magnetic resonance imaging (MRI) revealed left temporal lobe atrophy. At 82 years of age, the patient presented with pure anomic aphasia. At 83 years old, he showed mild impairment in word comprehension and semantic memory, in addition to anomia. MRI demonstrated further atrophy in the bilateral anterior temporal lobes, predominantly on the left side. This case suggests the possibility of slowly progressive, late-onset anomic PPA, which could be differentiated from the early stage of semantic or logopenic variants.

Single-incision laparoscopic surgery used to perform transanal endoscopic microsurgery (SILSTEM) for T1 rectal cancer under spinal anesthesia: report of a case.

Transanal endoscopic surgery has slowly gained widespread acceptance among colorectal surgeons, despite the need for specific training and the high costs of specialized instrumentation. At the other extreme, some laparoscopic surgeons recommend single port access surgery using a single-incision laparoscopic surgery port. Single-incision laparoscopic surgery was applied to perform transanal endoscopic microsurgery in a patient with T1 rectal cancer under spinal anesthesia. The patient was a 74-year-old man who presented with a 2-cm elevated lesion in the right anterior portion of the rectum. Ordinary laparoscopic instruments were used to perform submucosal resection. The tumor was completely excised from the rectal wall with the use of an ultrasonic surgical scissors. The patient recovered uneventfully and was discharged 4 days after the operation. There was no fecal incontinence or soiling during the postoperative follow-up. Colonoscopy at 4 months after the operation showed no recurrence of either adenocarcinoma or adenoma.

[Tactile hallucination and delusion associated with broad brain infarction in the right middle cerebral artery territory: a case report].

A 64-year-old woman experienced a broad brain infarction in the right middle cerebral artery territory with left hemiparesis and left unilateral spatial neglect. She was treated by endovascular thrombectomy and decompressive craniectomy. During the subacute phase of the brain infarction, she became alert and insisted, "My eldest daughter is at my feet and I can touch her body with my feet." The patient's abnormal experience was accompanied by visual information, such as her daughter's physical characteristics and clothing, which could not be directly confirmed, and a false ideation that she had two eldest daughters. Although the patient was aware that her experience was abnormal, she was unable to deny her strong beliefs regarding the experience. We concluded that a series of the patient's abnormal experiences consisted of both tactile hallucinations and delusions. In this case, the subjects of the delusions associated with the patient's tactile hallucinations were limited to close relatives. Finally, it was clinically important that introspection by the patient revealed details of the nature of her hallucinations.

Suppression of melanin production by expression of HSP70.

Skin hyperpigmentation disorders due to abnormal melanin production induced by ultraviolet (UV) irradiation are both a clinical and cosmetic problem. UV irradiation stimulates melanin production in melanocytes by increasing intracellular cAMP. Expression of heat shock proteins (HSPs), especially HSP70, is induced by various stressors, including UV irradiation, to provide cellular resistance to such stressors. In this study we examined the effect of expression of HSP70 on melanin production both in vitro and in vivo. 3-Isobutyl-1-methylxanthine (IBMX), a cAMP-elevating agent, stimulated melanin production in cultured mouse melanoma cells, and this stimulation was suppressed in cells overexpressing HSP70. IBMX-dependent transcriptional activation of the tyrosinase gene was also suppressed in HSP70-overexpressing cells. Expression of microphthalmia-associated transcription factor (MITF), which positively regulates transcription of the tyrosinase gene, was up-regulated by IBMX; however, this up-regulation was not suppressed in HSP70-overexpressing cells. On the other hand, immunoprecipitation and immunostaining analyses revealed a physical interaction between and co-localization of MITF and HSP70, respectively. Furthermore, the transcription of tyrosinase gene in nuclear extract was inhibited by HSP70. In vivo, UV irradiation of wild-type mice increased the amount of melanin in the basal layer of the epidermis, and this increase was suppressed in transgenic mice expressing HSP70. This study provides the first evidence of an inhibitory effect of HSP70 on melanin production both in vitro and in vivo. This effect seems to be mediated by modulation of MITF activity through a direct interaction between HSP70 and MITF.

Prevention of UVB radiation-induced epidermal damage by expression of heat shock protein 70.

Irradiation with UV light, especially UVB, causes epidermal damage via the induction of apoptosis, inflammatory responses, and DNA damage. Various stressors, including UV light, induce heat shock proteins (HSPs) and the induction, particularly that of HSP70, provides cellular resistance to such stressors. The anti-inflammatory activity of HSP70, such as its inhibition of nuclear factor kappa B (NF-kappaB), was recently revealed. These in vitro results suggest that HSP70 protects against UVB-induced epidermal damage. Here we tested this idea by using transgenic mice expressing HSP70 and cultured keratinocytes. Irradiation of wild-type mice with UVB caused epidermal damage such as induction of apoptosis, which was suppressed in transgenic mice expressing HSP70. UVB-induced apoptosis in cultured keratinocytes was suppressed by overexpression of HSP70. Irradiation of wild-type mice with UVB decreased the cutaneous level of IkappaB-alpha (an inhibitor of NF-kappaB) and increased the infiltration of leukocytes and levels of pro-inflammatory cytokines and chemokines in the epidermis. These inflammatory responses were suppressed in transgenic mice expressing HSP70. In vitro, the overexpression of HSP70 suppressed the expression of pro-inflammatory cytokines and chemokines and increased the level of IkappaB-alpha in keratinocytes irradiated with UVB. UVB induced an increase in cutaneous levels of cyclobutane pyrimidine dimers and 8-hydroxy-2'-deoxyguanosine, both of which were suppressed in transgenic mice expressing HSP70. This study provides genetic evidence that HSP70 protects the epidermis from UVB-induced radiation damage. The findings here also suggest that the protective action of HSP70 is mediated by anti-apoptotic, anti-inflammatory, and anti-DNA damage effects.

Neural correlates of psychotic symptoms in dementia with Lewy bodies.

The aim of this study was to investigate the association between psychotic symptoms in dementia with Lewy bodies and brain perfusion on single photon emission tomography. Based on factor analysis in 145 patients, psychotic symptoms were classified into five symptom domains (factor 1 to 4-related symptoms and delusions). The relationship between each symptom domain and brain perfusion was assessed in 100 patients with dementia with Lewy bodies, while accounting for the effects of age, sex, dementia severity, parkinsonism and dysphoria. Factor 1 symptoms (Capgras syndrome, phantom boarder, reduplication of person and place and misidentification of person) represented misidentifications, and were significantly related to hypoperfusion in the left hippocampus, insula, ventral striatum and bilateral inferior frontal gyri. Factor 3 symptoms (visual hallucination of person and feeling of presence) represented hallucinations of person and were related to hypoperfusion in the left ventral occipital gyrus and bilateral parietal areas. Delusions of theft and persecution were associated with relative hyperperfusion in the right rostral medial frontal cortex, left medial superior frontal gyrus and bilateral dorsolateral frontal cortices. This study revealed that different psychotic symptoms in dementia with Lewy bodies were associated with distinguishable cerebral networks. Visual hallucinations were related to dysfunction of the parietal and occipital association cortices, misidentifications were related to dysfunction of the limbic-paralimbic structures and delusions were related to dysfunction of the frontal cortices. Our findings provide important insights into the pathophysiological mechanisms underlying psychotic symptoms in dementia with Lewy bodies.

[Non-aphasic misnaming: a case report and literature review].

A 71-year-old, right-handed woman was admitted to our hospital due to a sudden difficulty with conversation. On admission, she was alert, but had a euphoric mood, disorientation, and a disturbance of recent memory. Her speech was fluent. Her repetition and auditory word cognition were excellent, but she had a slight difficulty with naming visual objects. She frequently showed word-finding difficulty and irrelevant paraphasia during free conversation and a word fluency task. Her irrelevant paraphasia was observed more frequently when she was asked to explain her outbreak of anger at the hospital, i.e., it was situation-dependent. She also had anosognosia. MRI showed an infarct in the territory of the left tuberothalamic artery. Single-photon emission computed tomography revealed low-uptake lesions in the left thalamus and orbital frontal, medial frontal, and medial temporal lobes. The patient was diagnosed with non-aphasic misnaming. The clinical characteristics of patients with non-aphasic misnaming in the literature were reviewed. All of the patients with non-aphasic misnaming had word-finding difficulty and irrelevant paraphasia. Additionally, they had either emotional disturbance or anosognosia.

Giant Hepatic Cyst: A Possible Cause of Inferior Vena Cava Syndrome.

An 80-year-old man was transferred to our institution with lower limb edema and worsening dyspnea following the administration of diuretic medication. Transthoracic echocardiography and computed tomography revealed a giant hepatic cyst (176×190 mm) compressing his right atrium and inferior vena cava (IVC). Laparoscopic cyst deroofing combined with omental packing and subsequent tube drainage immediately alleviated all his symptoms. The procedure was uneventful, and he was discharged without any complications on postoperative day 9; he had no recurrent symptoms or hepatic cysts at the postoperative 2-month follow-up. Therefore, a giant hepatic cyst can cause IVC syndrome, and laparoscopic deroofing is a beneficial approach for the treatment of accessible cysts.

HSP70 inducers from Chinese herbs and their effect on melanin production.

Skin hyperpigmentation disorders as a result of abnormal melanin production induced by ultraviolet (UV) irradiation are both a clinical and a cosmetic problem. This melanin production is mediated by tyrosinase whose expression is positively regulated by microphthalmia-associated transcription factor (MITF). We recently found that expression of heat shock protein 70 (HSP70) inhibits melanin production. In this study, we searched for HSP70 inducers from Chinese herbs and selected an ethanol extract of Eupatorium lindleyanum (E. lindleyanum). Not only melanin production but also the activity and expression of tyrosinase were significantly suppressed in cells treated with E. lindleyanum extract as well as in HSP70-overexpressing cells. The expression of MITF was clearly suppressed in cells treated with E. lindleyanum extract but not in HSP70-overexpressing cells. These results suggest that E. lindleyanum extract suppresses the expression of tyrosinase and melanin production through both HSP70-dependent and HSP70-independent mechanisms.

Effectiveness of a team participation training course for laparoscopy-assisted gastrectomy.

BACKGROUND: Laparoscopy-assisted distal gastrectomy (LADG) for stomach cancer is increasingly performed in Japan and Korea. However, the procedure still is considered to be complicated, and the optimal education system for trainees has not been established to date. METHODS: The authors organized a 1-day professional training course termed the LADG Basic Lab Course for LADG beginners. The participants were required to apply as a team of two surgeons and two operating nurses. The training course consisted of lectures and a live porcine lab emphasizing use of the ultrasonically activated device and the flexible laparoscope as well as team cooperation. The quality and effectiveness of the course were evaluated 6-10 months (mean, 8.2 +/- 2.2 months) after the course using a survey form sent to a representative surgeon of each institution. RESULTS: From May 2007 to July 2008, a total of 80 colleagues (47 surgeons and 33 nurses) from 20 different centers in Japan participated in the training course. These surgeons represented 12.4 +/- 6.2 postgraduate years of education and had performed 2.7 +/- 4.9 LADGs before taking the course. In the follow-up evaluation, 12 institutions (60%) completed the survey forms. The mean operation time was reduced for eight respondents (66.7%). The number of LADGs performed per month increased in 50% of the respondents' institutions. The degree of lymph node dissection in LADG was extended for 66.7% of the respondents. The respondents answered that 100% of the first operators showed improvement in skills, as did 59.5% of the scope operators and 59.5% of the nurses. CONCLUSIONS: The training course was an effective means of introducing LADG to each institution. Training courses emphasizing explanations of key devices and teamwork may be effective for the introduction of advanced laparoscopic surgeries.