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.

[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.

[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.

[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.

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.

Comparison of Short-Term Outcomes Between Hand-assisted Laparoscopic Distal Gastrectomy and Laparoscopy-assisted Distal Gastrectomy in Gastric Cancer.

Hand-assisted laparoscopic surgery is considered to provide the benefits of laparoscopic surgery in various diseases. However, limited information is available regarding the feasibility of hand-assisted laparoscopic distal gastrectomy (HALDG)-a subset of hand-assisted laparoscopic surgery-as a gastric cancer treatment. This study aimed to evaluate the usefulness of HALDG compared with laparoscopy-assisted distal gastrectomy (LADG). Consecutive patients who underwent HALDG (n=58) or LADG (n=90) for stage I gastric cancer between 2005 and 2016 were eligible. Operative time was significantly shorter and blood loss was significantly higher in HALDG than in LADG (P<0.001, both). Postoperative aminotransferase levels were significantly lower in HALDG than in LADG (P<0.001). There was no significant difference in perioperative complications, a number of analgesics, postoperative C-reactive protein levels, and 3-year relapse-free and overall survival rates between the groups. This study suggests that HALDG is a safe and feasible approach and could become an effective option for stage I gastric cancer treatment.

Xanthogranulomatous pyelonephritis with psoas abscess and renocolic fistula.

Xanthogranulomatous pyelonephritis (XGP) is an uncommon inflammatory disease of the kidney 1. Diffuse XGP is a rare condition which may spread into the pelvic cavity leading to fatal complications from a psoas muscle abscess and/or renocolic fistula 2. In diffuse type, nephrectomy and excision of the fistula is the recommended treatment.

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.

Simultaneous diagnosis of familial achalasia: report of two cases.

BACKGROUND: Achalasia is a rare disease with a morbidity of 1 in 100,000, for which the exact mechanism of pathogenesis has not been clarified due to the small total number of patients. We herein report on our experience with two cases of familial achalasia in which the involvement of genetic inheritance was suspected. CASE PRESENTATION: These cases consist of a man in his thirties and his mother in her sixties. The son consulted the Department of Gastrointestinal Medicine at our institute with dysphagia, and an upper gastrointestinal endoscopy revealed a gastric submucosal tumor with a maximal diameter of approximately 50 mm. Achalasia was also strongly suspected due to the enlargement of the esophagus to the maximum transverse diameter of 55 mm by esophagography along with delayed clearance of barium. A detailed interview revealed prolonged mild dysphagia in his mother. Therefore, high-resolution manometry was carried out in both patients. As a result, peristaltic disorder was observed in the esophageal body in both the mother and son, leading to a definitive diagnosis of achalasia. For the son, total gastrectomy including the lower esophagus with Roux-en-Y reconstruction was performed. His postoperative course was uneventful, and the patient was discharged from hospital in remission on the 9th day following surgery and is currently undergoing follow-up as an outpatient. CONCLUSIONS: We hereby report on a very rare case of familial achalasia that we experienced which may suggest a genetic element in the onset of achalasia, and reviewed the literature.

Successful treatment of proton pump inhibitor induced sporadic fundic gland polyps with an argon plasma coagulator in a patient with polycythaemia vera.

INTRODUCTION: Proton pump inhibitor (PPI) use is associated with the development of fundic gland polyps (FGPs); discontinuing PPIs is associated with regression of FGPs. Here, we report a rare case of non-respondent FGPs after discontinuation of PPI that were successfully treated using an argon plasma coagulator (APC). PRESENTATION OF CASE: We present the case of a 68-year-old woman with a history of polycytheamia vera. She also had gastroesophageal reflux disease (GERD) and had been taking 10 mg of omeprazole daily for the past three years. Esophagogastroduedenoscopy (GF) revealed over 100 pedunculated polyps in the gastric body and fundus. Histological examination of the specimens showed dilated oxyntic glands with flattened parietal and mucous cells. Based on these findings and the clinical history, a diagnosis of FGPs was made. Omeprazole use was then discontinued. Repeat GF performed 6 months and 1 year later showed a significant increase in the number and size of the polyps. APC treatment was performed every 6 months for 3 years. Further GF showed a significant decrease in the number and size of the FGPs 4 years after discontinuing PPI. DISCUSSION: We conclude that PPI use is a strong risk factor for the development of FGPs and discontinuing PPI is associated with regression of FGPs, but not in patients with polycythaemia vera. However, the mechanism involved in the interaction between FGP and polycytheamia vera remains unknown. CONCLUSION: Non-respondent FGPs after discontinuation of PPI use may be successfully treated using APC.

Laparoscopic-Assisted Percutaneous Endoscopic Gastrostomy Combined with CT-GC.

PURPOSE: Despite the widespread use of percutaneous endoscopic gastrostomy (PEG) tubes, their placement may be associated with a variety of complications, including gastrocolic fistula. MATERIALS AND METHODS: In total, seven high-risk individuals diagnosed using computed tomography (CT)-gastrocolonography (GC) underwent laparoscopic-assisted PEG (LAPEG) placement. Study endpoints included the success of LAPEG under local anesthetic and intravenous sedation, inability to thread the PEG tube, the eventual tube location, the number of tube adjustments needed, adverse events, the operating time, and PEG tube-related infection. RESULTS: In total, 135 PEG procedures were performed during this study. Successful CT-GC was achieved in all 135 patients, and we successfully used a standard PEG technique to place the gastrostomy tube in 128 patients (95%). In seven patients (5%), the LAPEG technique was used because the transverse colon became interposed between the abdominal wall and the anterior wall of the stomach. LAPEG procedure-related minor complications were observed in two patients. CONCLUSIONS: LAPEG combined with CT-GC can be used for patients with difficult anatomical orientations and may minimize the risk of complications in PEG placement.

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.

On the Utility of MIBG SPECT/CT in Evaluating Cardiac Sympathetic Dysfunction in Lewy Body Diseases.

BACKGROUND: Abnormal cardiac uptake of 123I-metaiodobenzylguanidine (123I-MIBG) is a diagnostic marker of Lewy body diseases (LBDs), e.g., Parkinson's disease (PD) and dementia with Lewy bodies (DLB). Planar imaging is generally used to assess cardiac sympathetic dysfunction in 123I-MIBG scintigraphy; however, its clinical utility requires further improvement. We hypothesized that the co-registration of single-photon emission tomography (SPECT) and computed tomography (CT) images would improve the diagnostic accuracy of 123I-MIBG cardiac scintigraphy for LBDs. This study sought to evaluate the effects of SPECT/CT imaging on 123I-MIBG cardiac scintigraphy for diagnosing LBDs. METHODS: We retrospectively investigated data of 54 patients (consecutive 18 patients in each PD, DLB, and idiopathic normal pressure hydrocephalus [iNPH] groups) who underwent 123I-MIBG cardiac scintigraphy (planar and SPECT/CT) because of suspected LBDs at the Tohoku University hospital from June 2012 to June 2015. We compared the diagnostic accuracies of the conventional planar 123I-MIBG method and SPECT/CT methods (manual and semi-automatic). RESULTS: In the conventional planar analysis, 123I-MIBG uptake decreased only in the DLB group compared with the iNPH group. In contrast, the SPECT/CT analysis revealed significantly lower 123I-MIBG uptake in both the PD and DLB groups compared with the iNPH group. Furthermore, a receiver operating characteristic analysis revealed that both the manual and semi-automatic SPECT/CT methods were superior to the conventional planar method in differentiating the 3 disorders. CONCLUSIONS: SPECT/CT 123I-MIBG cardiac scintigraphy can detect mild cardiac sympathetic dysfunction in LDBs. Our results suggest that the SPECT/CT technique improves diagnostic accuracy for LBDs.

Nonocclusive mesenteric ischemia: fulminant pancolitis.

NOMI is mesenteric hypoperfusion with reactive vascular spasms. Changes in the color of the mucosa may reflect the severity of the ischemia of the colon and the severity of prognosis. Even with surgery, the mortality rate is 75%. Diagnosis requires a high degree of clinical suspicion.

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.

Clinical characteristics of idiopathic normal pressure hydrocephalus with Lewy body diseases.

BACKGROUND: Comorbidity of idiopathic normal pressure hydrocephalus and neurodegenerative diseases presents a diagnostic challenge. The aim of this study was to elucidate the clinical features of iNPH patients who are possibly comorbid with Lewy body diseases. METHODS: In this study, we retrospectively analyzed the records of consecutive 127 patients with definite idiopathic normal pressure hydrocephalus. (123)I-Metaiodobenzylguanidine myocardial scintigraphy was undertaken to 21 patients with idiopathic normal pressure hydrocephalus because of suspicion for comorbid Lewy body diseases. RESULTS: As a result, 7 of the 21 patients with idiopathic normal pressure hydrocephalus exhibited cardiac sympathetic abnormality. Idiopathic normal pressure hydrocephalus patients with cardiac sympathetic dysfunction had the constellation of clinical findings, such as younger onset, less severe urinary dysfunction, kinesie paradoxale, and cogwheel rigidity. CONCLUSIONS: Our results suggested that comorbidity of idiopathic normal pressure hydrocephalus and Lewy body diseases are not rare condition and careful screening for potentially-curative surgery is important especially in atypical cases.