Amyotrophic lateral sclerosis: thyrotropin-releasing hormone and histidyl proline diketopiperazine in the spinal cord and cerebrospinal fluid

In spinal cords from seven amyotrophic lateral sclerosis (ALS) patients and four controls, we found no difference in thyrotropin-releasing hormone (TRH) concentration relative to protein content, but there was a reduction per tissue wet weight in ALS. Immunohistochemical localization of TRH in ALS cord was unaltered. Histidyl proline diketopiperazine (HisPro-DKP), a possible metabolite of TRH, was significantly elevated per protein content in ALS. CSF levels of TRH and HisPro-DKP were unchanged. These findings suggest that TRH neurons are not primarily affected in ALS, but TRH and tissue protein are lost together as the disease progresses.     

Early school adjustment problems: some perspectives and a project report

A kindergarten and elementary school intervention program for dealing with early school adjustment problems is described. It is designed to accommodate greater individual differences in classrooms, improve and augment regular support, and provide specialized staff development and interventions. Initial findings are presented, and the evolution of the program so as to address problems of school newcomers is discussed.     

Abdominal aortic aneurysms and thoracic aortic atheromas.

BACKGROUND: Abdominal aortic aneurysm (AAA) is associated with atherosclerosis elsewhere. Thoracic aortic atheromas (ATHs) seen on transesophageal echocardiography (TEE) are an important cause of stroke and peripheral embolization. The purposes of this study were to determine whether an association exists between AAA and ATHs and to assess the importance of screening patients with ATHs for AAA. METHODS: For the retrospective analysis, 109 patients with AAA and 109 matched controls were compared for the prevalence of ATHs on TEE and for historical variables. For the prospective analysis, screening for AAA on ultrasonography was performed in 364 patients at the time of TEE. RESULTS: Results of the retrospective analysis showed that ATHs were present in 52% of patients with AAA and in 25% of controls (odds ratio [OR] = 3.3; P =.00003). There was a significantly higher prevalence of hypertension, myocardial infarction, heart failure, smoking, and carotid or peripheral arterial disease in patients with AAA. However, only ATHs were independently associated with AAA on multivariate analysis (P =.001). Results of the prospective analysis showed that screening at the time of TEE in 364 patients revealed AAA in 13.9% of those with ATHs and in 1.4% of those without ATHs (P <.0001; OR = 11.4). CONCLUSIONS: (1) There is a strong, highly significant association between abdominal aneurysm and thoracic atheromas. (2) Patients with AAA may be at high risk for stroke because of the concomitance of thoracic aortic atheromas. (3) The high prevalence of abdominal aneurysm in patients with thoracic atheromas suggests that screening for abdominal aneurysm should be carried out in all patients with thoracic atheromas identified by TEE.     

Dental chair intracerebral hemorrhage

A 52-year-old woman had a fatal intracerebral hemorrhage after dental manipulation. Normotensive in the past, the initial blood pressure was high but rapidly returned to normal. Necropsy showed no vascular malformation or evidence of hypertensive vascular disease. Clinical and experimental data show that stimulation of trigeminal fibers can cause important changes in blood pressure and pulse.     

Abdominal pain in the primary care setting

The characteristics of and final diagnoses for patients presenting with abdominal pain were investigated. This retrospective study examined 556 charts of patients 18 years of age and older who presented over a two-year period to three family practice offices. The charts were abstracted for demographic factors, symptoms, physical findings, laboratory data, final diagnosis, and number of visits for abdominal pain. The final diagnosis was documented by radiologic, laboratory, surgical, or pathologic specimen confirmation except for the following diagnoses: acute gastroenteritis, pelvic inflammatory disease, irritable bowel syndrome, and abdominal pain, etiology undetermined. No cause for the abdominal pain was found for approximately one half of the cases. Most patients were female even when gynecologic problems were excluded. Nine percent of abdominal pain patients were admitted to the hospital for evaluation or surgery. An average of 1.8 tests were ordered per patient. Almost one half of the patients were seen only once for the problem. The results suggest that a large percentage of the patients who present with abdominal pain have a self-limited illness for which no definitive diagnosis is found.     

Progressive myopathy in hyperkalemic periodic paralysis

A progressive degenerative myopathy has been well described in hypokalemic periodic paralysis but is not as widely recognized in hyperkalemic periodic paralysis. We studied four families with the latter disease in which some members developed a progressive myopathy. Episodes of paralysis were prolonged, lasting for months in some cases, and in one case paralysis was sufficiently severe to require ventilatory support. The progressive myopathy tended to develop at a time when attacks of paralysis were decreasing in frequency. Muscle biopsy specimens showed variability in fiber size, internal nuclei, and fibers with vacuoles. Electron microscopy showed myofibrillary degeneration and tubular aggregates. An abnormal biopsy specimen was more common in older patients. Our experience suggests that a progressive myopathy is as common in hyperkalemic periodic paralysis as it is in the hypokalemic disorder.