Cerebral hypoperfusion and leukomalacia

Tunga penetrans is a burrowing flea that is prevalent in Central and South America, the Caribbean, tropical Africa, India, and Pakistan, and rarely is imported to the United States. Tungiasis results from the cutaneous infestation of humans by the gravid female flea, T. penetrans. We report a case of tungiasis in a female who had been on a missionary expedition along the Brazilian Amazon River. The clinical presentation, life cycle, differential diagnosis, military and historical aspects, and treatment are discussed.     

Kawasaki disease: a dangerous acute childhood illness

Kawasaki disease is an acute febrile illness most commonly seen in children under the age of 5. It is characterized by fever, rash, cervical lymphadenopathy, bilateral nonexudative conjunctivitis, oropharyngeal mucosal changes, and erythema of the hands and feet followed by desquamation. However, a child with Kawasaki disease may not exhibit all of these symptoms. The disease resembles many other childhood illnesses, such as measles and scarlet fever, and misdiagnosis is common. Left untreated, Kawasaki disease has potential life-threatening consequences; 20% to 25% of children develop coronary artery aneurysms as a result. Although no specific laboratory tests exist that identify Kawasaki disease definitively, there are clinical and laboratory findings that guide diagnosis and treatment. Treatment includes the hospitalization of the child and subsequent administration of high doses of aspirin and intravenous immunoglobulin. With recovery, aspirin doses are reduced and the child may be monitored at home with outpatient follow-up. It is imperative that the health care provider be aware of the symptoms of Kawasaki disease in order to make the diagnosis and treat the child before cardiac sequelae ensue.     

Kawasaki disease

We analysed the cytoskeletal proteins and extracellular matrix components of in vitro cultured BMSC both in resting state and after activation with IFN gamma and TNF alpha, using an immunoperoxidase procedure. BMSC expressed fibronectin, alpha-actin, beta-tubulin, vimentin and vinculin while cytokeratinpan, GFAP, neurofilament, desmin and laminin were not expressed. This pattern of expression was not affected by addition of TNF alpha and IFN gamma, but differs from human tonsil stromal cells for laminin expression and alpha-actin localization.     

Kawasaki disease

This study was conducted to investigate the functional and morphological aspects of orthotopic lung xenograft rejection in a concordant hamster-to-rat donor-recipient species combination. By postoperative day (POD) 3, allotransplanted grafts demonstrated good aeration, but infiltrates were seen in all the xenotransplanted lungs. Antihamster lymphocytotoxic antibody titers increased to 5.2 +/- 1.1 by POD 3 (P < 0.05 vs POD 1) and reached 7.0 +/- 0.8 by POD 5 (P < 0.05 vs PODs 1 and 3). The CD4+/CD8+ ratio in peripheral blood lymphocytes increased significantly on POD 3 (P < 0.05 vs untransplanted), but decreased by POD 5 (P < 0.05). Histologically, on POD 3 the xenotransplanted grafts were characterized by perivascular cellular infiltrates and edema. Cytologically, the cells consisted of small round lymphocytes, monocytes, and occasional neutrophils. Immunohistochemical analysis showed heavy IgM and C3 deposits in the vascular endothelium, without any IgG deposits. Allotransplanted grafts showed moderate W 3/25 + (28.3 +/- 17.3%) and MRC OX8 + (38.7 +/- 0.7%) cellular infiltrations on POD 3, but ED1 + (8.0 +/- 3.7%) cells were rare. Conversely, in the xenotransplanted grafts, ED1 + (34.2 +/- 16.4%) cells were more prevalent than MRC OX8 + (18.1 +/- 16.5%) cells on POD 3, at P < 0.01 and P < 0.05 vs allograft, respectively. These results indicate that both antidonor antibodies and macrophages/monocytes play an important role in the concordant lung xenograft rejection.     

Cerebral hypoperfusion in orthostatic hypotension with globally denervated myocardium

A 57-yr-old woman had frequent syncope when rising from a seated position. Her blood pressure fell from 140/80 mmHg to 60-70/40 mmHg while changing positions. Iodine-123-metaiodobenzylguanidine ([123I]MIBG) did not accumulate in the heart, whereas 201Tl-Cl (201Tl) did. Raise-up 99mTc-hexamethyl-propyleneamine oxime (99mTc-HMPAO) brain SPECT revealed decreased activity in the bilateral frontal areas, and subsequent supine 99mTc-HMPAO brain SPECT revealed filling in these areas, indicating that the cerebral blood flow (CBF) was transiently decreased in the frontal areas more than others in a standing position. The plasma norepinephrine (NE) level of this patient was normal during supine rest, but when she stood up, failure to increase the plasma level of NE uncovered a sympathetic nervous dysfunction. The CBF abnormality in patients with orthostatic hypotension may be due to a "functional" hemodynamic mechanism that induces orthostatic stress. This patient had transient hypoperfusion in the frontal areas when standing, without organic cerebral arterial stenosis. Only CBF in the frontal areas revealed relative hypoperfusion. These regions might be highly susceptible to a change in blood flow. The causes of orthostatic hypotension of this patient were autonomic failure with a disturbance of the sympathetic nerve endings, which was revealed by 99mTc-HMPAO brain SPECT and cardiac [123l]MIBG imaging.     

Urine mercury levels in Kawasaki disease

Chronic idiopathic neutrophilia is a rarely recognized finding in otherwise healthy subjects. Described here is a case, not previously reported; in which the patient also had congenital asplenia. Studies carried out to determine the mechanism for the neutrophilia are also described. The results show that the patient's neutrophilia was associated with an enlarged circulating pool of neutrophils, increased production and utilization of neutrophils and a short blood granulocyte survival. The findings differ completely from those observed in patients with chronic idiopathic neutrophilia and intact spleens. The results suggest that (1) the mechanism for neutrophilia in patients with chronic idiopathic neutrophilia varies and depends upon the presence or absence of the spleen, and (2) the spleen has a role in the control of neutrophil production, distribution and utilization.     

Predicting coronary aneurysms in Kawasaki disease

Intravenous immunoglobulin is used to prevent coronary artery involvement in patients with acute Kawasaki disease. In this issue of the journal, Beiser et al provide a scoring system that is invoked 24 hours after gamma globulin therapy to predict risk for coronary involvement. This instrument would be useful for pediatric cardiologists and pediatricians in planning later management of patients with Kawasaki disease and counseling parents regarding long-term outcome.     

Ga-67 imaging: Kawasaki disease

The authors describe a child with Kawasaki disease (mucocutaneous lymph node syndrome), in whom myocardial inflammation associated with the disease was readily detected with Ga-67 imaging.     

Serum HDL-cholesterol and lipoprotein fraction in Kawasaki disease (acute mucocutaneous lymph node syndrome)

Serum HDL-cholesterol level and lipoprotein fraction were studied in 50 patients with Kawasaki disease (acute mucocutaneous lymph node syndrome), ranging in age from 4 months to 7 years, with an average of 2 years and 6 months. There were 31 males and 19 females. In 7 of these patients coronary arterial aneurysms were revealed by aortogram. In all 50 patients analysis were carried out periodically from the onset of the disease to the convalescent stage, and the following results were obtained: 1) Serum total cholesterol level showed mild change throughout the course of the disease. 2) Average HDL-cholesterol level was 29.6 +/- 11.0 mg/dl in the first week of the disease, 26.8 +/- 8.9 from 1 to 2 weeks and 47.9 +/- 12.6 after 6 weeks. There was significant difference between the HDL-cholesterol levels of first 2 weeks and after 4 weeks (p less than 0.01). 3) The recovery of HDL-cholesterol level was slow in both clinically severe patients and the patients with coronary aneurysms as compared with that in clinically mild patients (p less than 0.05). These findings suggest that there may be some relationships between the alteration of serum lipoprotein and the severity of systemic vasculitis in Kawasaki disease, especially which may play some role in clinical severity and the occurrence of coronary arterial lesions. However, further study is needed to clarify the cause of changes in serum HDL-cholesterol and lipoprotein.     

Cerebral blood flow velocity decreases during cognitive stimulation in Huntington's disease

OBJECTIVE: To examine whether activation of cerebral blood flow velocity during cognitive stimulation, as measured by transcranial Doppler ultrasonography (TCD), is altered in patients with Huntington's Disease (HD). BACKGROUND: Previous research suggests that resting cerebral blood flow in symptomatic and asymptomatic HD patients is reduced from expected premorbid levels. The effects of cognitive activation on this relative hypoperfusion in HD has not been studied extensively. METHODS: We measured TCD flow velocity during rest and cognitive testing with the Porteus Maze Test and the Trails Test in 12 normal control subjects and 10 gene-positive HD patients. Percent change (i.e., flow during testing/resting) of flow velocity in the anterior and middle cerebral arteries were compared between groups. Correlations among percent flow velocity change, a disability rating scale, and cognitive test scores were calculated. RESULTS: In control subjects, anterior cerebral artery flow velocity and middle cerebral artery velocity increased during cognitive testing (p=0.001). HD patients showed a smaller blood flow velocity increase in the anterior cerebral arteries during the Porteus Maze Test (p < 0.001) and the Trails Test, Part B (p < 0.001). In the left anterior cerebral artery, flow velocity fell an average of 4.2% below resting baseline levels during the Porteus Maze Test, and 1.2% below resting levels during the Trails Test. The magnitude of the cerebral blood flow velocity changes in the anterior cerebral artery correlated with a number of cognitive test scores and with a rating scale of functional disability. In addition, logistic regression was able to discriminate the HD patients from the control group based on blood flow velocity changes (p=0.0025). When HD patients were divided into more (i.e., HD with chorea; n=4) and less impaired (i.e., without chorea; n=6) groups, both showed significant decreases in left anterior cerebral artery flow velocity during visual spatial executive cognition testing compared with control subjects. CONCLUSIONS: These results suggest that activation of visual spatial executive functions cause decreased flow velocity in the anterior cerebral artery, but not the middle cerebral artery, in HD patients. These changes are related to test performance and functional capabilities.