We report a case of a 25-month-old girl presented to us for the evaluation of a severe delayed psychomotor development who also has pigmentary abnormalities. Linear and whorled hyperpigmentations following Blaschko's lines were noticed on her entire body except on her face, palms, soles, eyes and mucous membranes, which closely resembled those found in hypomelanosis of Ito, but inversely pigmented. Histologic examination revealed basal layer hyperpigmentation without incontinence of pigment or dermal melanophages. Chromosomal analysis of cultured peripheral leukocytes and fibroblasts from the hyperpigmented and the hypopigmented skin revealed normal female karyotype with no evidence of mosaicism or chimerism. This entity represents a kind of neurocutaneous syndrome-referred to by some authors as linear and whorled nevoid hypermelanosis.
Case Report ; *Child Development ; Child, Preschool ; Female ; Human ; Melanosis/*pathology/*physiopathology ; Nevus/*pathology ; *Psychomotor Performance ; Syndrome

Amyloidosis is the definition for a group of diseases that have, in common, the infiltration of one or more tissues by an abnormal protein material-the amyloid substance, which is detected histologically by their green polarization color after Congo red staining. Despite increased interest on basic nature of amyloidosis by recent immunohistochemical or experimental study, the knowledge about the incidence and neurotoxic effect of cerebral amyloid or concomitant occurrence with brain tumor is still inchoate. We examined the incidence and clinico-pathologic characteristics of the patients with amyloid deposits in supratentorial meningiomas. Particularly about their neurotoxic effect to adjacent brain is considered one of the possible cause of seizure in 33 patients who underwent resection surgery for meningioma at the Keimyung University during the past three years. The pathological review and subgrouping by histologic type were done in all 33 specimens with sufficient size of paraffin block, defined by their morphology and polarization color after Congo red staining for diagnosis of amyloid deposits localized in the tumor. Immunohistochemical studies using monoclonal antibodies for amyloid-A protein(AA) and beta-amyloid(A beta) were evaluated to identify subtypes of amyloidosis. The rate of incidence of amyloid deposit in meningioma was 21%, i.e. seven out of thirty three cases. All laboratory findings and clinical studies did not suggest a systemic form. Seizure occurrence was one out of seven cases(14%), which was of no statistical significance. Immunohistochemical study for AA subtype was all negative, but showed all positive for A beta protein around the vessels. Recent reports has also demonstrated that Amyloid precusor protein(APP) and A beta is related in Alzheimer's disease, hereditary cerebral hemorrhage with amyloidosis-Dutch type(HCHWA-D) and amyloid angiopathy. Our research data indicates that the incidence of amyloid deposit is as high as 21% in supratentorial meningiomas. It seems that it is one of the possible cause of seizure. Nonsystemic microdeposits of amyloid and their subtype and it's relationship to neurotoxic effect in meningiomas remain to be confirmed by immunoelectron microscopic examination or immunohistochemical methods.
Alzheimer Disease ; Amyloid* ; Amyloidosis ; Antibodies, Monoclonal ; Brain ; Brain Neoplasms ; Cerebral Hemorrhage ; Congo Red ; Diagnosis ; Humans ; Incidence ; Meningioma* ; Paraffin ; Plaque, Amyloid* ; Seizures

Severe head injury results in the suppression of cellular immunity associated with dysfunctioning of effector lymphocytes, such as helper T cells(CD4) (and cytotoxic T cells(CD8). Despite progress in the management of increased intracranial pressure following head injury, infection remains the most common complication and the primary cause of prolonged hospitalization and death. This study attempts to assess the cellular immune function following head injury according to the degree of severity, and to establish the clinically available parameters of cell mediated immune(CMI) function, which can then be used for coherent prediction of infection risk. Eighteem head injury patients without severe systemic injury, who divided into three subgroups depending on the severity of head injury, were estimated with the use of CMI multitest kit(Merieux Institute, France) to test delayed-type hypersensitivity(DTH) and enumerated the circulating lymphocyte subpopulation(pan T-cell marker CD3, helper T cell marker CD4, cytotoxic T cell marker CD8 and B-cell marker CD19) on the 1st, 7th, and 21th day of injury. Patients were monitored for evidence of infection for this period. Fourteen patients had no reaction to any antigens of the DTH skin test(anergy) and the remaining four patients had also some degree of anergy. Seven patients became infected and all of them were anergic. There were significant decrease of circulating effector T lymphocytes, both CD4-positive and CD8-positive cells, within 24 hours of injury in the mild as well as the moderate and severe head injury group. CD4-positive cells were nearly completely recovered by the 7th day of injury. CD8-positive cells had sustained significant decrease even after 3 weeks of injury. There was no significant change in pan T-cells(CD3-positive cells) and B-cells(CD19-positive cells). The results suggest that DTH skin test and effector T cell enumeration are both relatively simple and highly sensitive parameters for monitoring CMI function. Especially, anergy of DTH skin test can be used for indicator to predict risk of infection. Mild as well as moderate and severe head injuries may result in the suppression of cellular immunity associated with the dysfunctioning of effector T cell.
B-Lymphocytes ; Craniocerebral Trauma* ; Head* ; Hospitalization ; Humans ; Immunity, Cellular* ; Intracranial Pressure ; Lymphocytes ; Skin ; Skin Tests ; T-Lymphocytes

Epilepsy surgery has been demonstrated to be an effective alternative treatment for intractable partial or localization related epilepsy. Primary intracranial neoplasms and other structural lesions of the brain are important etiological factors in patients with partial seizure disorders. A neuroimaging identified lesion in patients with seizures, not necessarily medically refractory, may also be an indication for surgery in selected patients. Twelve patients operated on under local or general anesthesia for resection surgery underwent intraoperative recording(electrocorticogram) and/or functional mapping by electrical stimulation or somatosensory evoked potentials-(SSEPs) for identification of the secondary epileptogenic area and/or functional area; 2 meningiomas, 5 astrocytomas, 1 gangliocytoma, 1 abscess, 1 small AVM, 1 cysticercosis and one gliosis by previous intracerebral hemorrhage with middle cerebral artery(MCA) aneurysm. Among these, additional corticectomy or anterior temporal lobectomy was performed in eleven patients. All the patients did well after surgery with good outcomes as seizure free in nine(75%) out of 12 patients with 11.9 months of follow-up period, without any neurological deficits. Intraoperative recording and functional mapping of adjacent areas of the structural lesions of the brain are useful in surgery and can guide the extent of further resection.
Adolescent ; Adult ; *Brain Mapping ; Brain Neoplasms/physiopathology/*surgery ; Electroencephalography ; Evoked Potentials, Somatosensory ; Female ; Human ; Intraoperative Period ; Male ; Middle Age ; Seizures/physiopathology/*surgery ; Support, Non-U.S. Gov't

PURPOSE: This survey was aimed to examine the current dietary restrictions practices for neutropenic patients among hospitals with 500 and more beds. METHODS: Self-administered questionnaires were sent to 100 head or charge nurses of oncology or hemato-oncology wards by mail during October 2009, and 51 questionnaires were returned. The data were analyzed with descriptive statistics using SPSS (WIN version 12.0). RESULTS: Of the hospitals surveyed, 47.1% (n=24) had guidelines on the dietary restrictions, and 80.4% (n=41) placed patients with neutropenia on restricted diets. The major decisional criteria of the dietary restriction was absolute neutrophil count (30.5%) and cooking status (29.2%). The most commonly restricted foods were raw fish or fresh meat (98.0%), uncooked intestine (98.0%), raw eggs (98.0%), draught beer (96.1%), and fresh fruits or vegetables (86.3%). CONCLUSION: The results showed variation in pattern of dietary restrictions and lower rate of guideline among hospitals, so that the need for the standard dietary restriction guideline is high. However, the role of diet in the development of infection in neutropenic patients is still unclear, which makes it difficult to establish dietary restriction guideline. Therefore, additional research are required to identify the relationship between dietary factors and infections.
Beer ; Cooking ; Diet ; Eggs ; Fruit ; Head ; Humans ; Intestines ; Meat ; Neutropenia ; Neutrophils ; Nursing, Supervisory ; Ovum ; Postal Service ; Surveys and Questionnaires ; Vegetables