Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration.

We report the case of a 54-year-old woman with mental retardation who developed frontotemporal dementia and amyotrophic lateral sclerosis (ALS) in the presenium. She presented with dementia at age 48, and motor neuron signs developed at age 53. She had no family history of dementia or ALS. Postmortem examination disclosed histopathological features of ALS, including pyramidal tract degeneration, mild loss of motor neurons, and many Bunina bodies immunoreactive for cystatin C, but not ubiquitin-positive inclusions. Unusual features of this case included severe neuronal loss in the substantia nigra and medial globus pallidus. The subthalamic nucleus, limbic system, and cerebral cortex were well preserved. In addition, neurofibrillary tangles (NFTs) were found in the frontal, temporal, insular, and cingulate cortices, nucleus basalis of Meynert, and locus coeruleus, and to a lesser degree, in the dentate nucleus, cerebellum, hippocampus, and amygdala. No ballooned neurons, tufted astrocytes, or astrocytic plaques were found. Tau immunostaining demonstrated many pretangles rather than NFTs and glial lesions resembling astrocytic plaques in the frontal and temporal cortices. This glial tau pathology predominantly developed in the middle to deep layers in the primary motor cortex, and was frequently associated with the walls of blood vessels. NFTs were immunolabeled with 3-repeat and 4-repeat specific antibodies against tau, respectively. Although the pathophysiological relationship between tau pathology and the selective involvement of motor neurons, substantia nigra, and globus pallidus was unclear, we considered that it might be more than coincidental.

A case of incarcerated femoral hernia in an infant.

The authors describe the case of an incarcerated femoral hernia in an infant. A 4-month-old female infant was hospitalized with an incarcerated right groin hernia. Attempts to reduce the mass were unsuccessful, so an emergency surgery was performed. During surgery, the hernial sac seemed to protrude under the inguinal ligament. The authors opened the hernial sac and observed a dark red small intestine. The authors did not resect the intestine because its color gradually improved after the constriction was released. The hernial sac passed under the inguinal ligament, and the infant was diagnosed with incarcerated femoral hernia.

Nonocclusive mesenteric ischemia after aorto-iliac reconstruction of a ruptured abdominal aortic aneurysm: report of a case.

We report a case of nonocclusive mesenteric ischemia (NOMI), which developed after Y-grafting for a ruptured abdominal aortic aneurysm (AAA). A 71-year-old man was referred to our hospital with severe abdominal pain and signs of shock. Computed tomography revealed a ruptured AAA, and emergency aneurysmectomy and aortic grafting were performed. However, on postoperative day 7, a large amount of pus was seen oozing from the surgical wound. An emergency laparotomy revealed segmental, highly diffuse necrotic changes of the small intestine, but pulsation of the marginal artery in the necrotic region was palpable and blood flow was well audible by Doppler ultrasound. These findings were consistent with a diagnosis of NOMI.