Early infantile manifestations of incontinentia pigmenti mimicking acute encephalopathy

Objective

We retrospectively reviewed six patients with incontinentia pigmenti (IP) who had encephalopathic manifestations during early infancy.

Methods

We enrolled six patients who met the following criteria from the mailing list of the Annual Zao Conference: (1) diagnosis of IP; (2) encephalopathic manifestations with reduced consciousness and clusters of seizures by 6 months of age; and (3) no evidence of central nervous system infection or metabolic derangement.

Results

The onset of the encephalopathic events was within the first 2 months of life in all but one patient. All had clusters of focal clonic seizures. The duration of seizures was typically 5 min. The seizures ceased within 5 days in all patients. Various degrees of reduced consciousness were observed in association with the frequent seizures. Diffusion-weighted imaging during the acute phase showed reduced water diffusion in the subcortical white matter, corpus callosum, basal ganglia, thalami, and internal capsule in two patients. Scattered subcortical white matter lesions were observed on fluid-attenuated inversion-recovery images in two patients.

Conclusions

The encephalopathic manifestations in patients with incontinentia pigmenti were characterized by seizure clusters and reduced consciousness, albeit of relatively short duration. Magnetic resonance imaging abnormalities were predominant in the subcortical areas in most patients.     

Penetrating keratoplasty and cataract surgery

Of 46 eyes undergoing simultaneous penetrating keratoplasty and cataract extraction, 25 grafts (54%) remained clear with an average follow-up of 37 months. This low success rate can be party explained by the fact that more than 50% of our patients had unfavourable ocular conditions preoperatively. In case of simultaneous procedures, methods of cataract extraction (ICCE or ECCE) did not have any significant affect on the rate of clear grafts. In eyes with favourable ocular conditions preoperatively, the rates of clear grafts were similar (approximately 75%) in cases of simultaneous operation (21 eyes) and separate operation (8 eyes).     

Development Of The Human Mesencephalic Trigeminal Nucleus: A Morphometric Study

Anatomical development of the human mesencephalic trigeminal nucleus was assessed quantitatively and qualitatively, using serial sections of brains, with the help of a computer-assisted image-analyser. Five fetuses at 16, 18, 21, 23 and 27 weeks of gestation; five neonates at 30, 32, 33, 35 and 40 weeks of gestation; a two-month-old infant and a 63-year-old adult were studied. Morphometric analyses of the mesencephalic trigeminal nucleus revealed that orocaudal columnar length and neuronal areas gradually increased with gestational age. Diversity of neuronal areas appeared after 32 weeks of gestation, but neuronal numbers and shapes showed little change throughout prenatal and postnatal life.     

Functional neural reorganization following ischemic stroke in the precentral knob: an fMRI study]

We evaluated a 63 year-old, right-handed woman by functional MRI (fMRI) in the early and the recovery stages following a cerebral infarction in the right precentral knob. An activated signal in the ipsilateral sensorimotor cortex and contralateral supplementary motor cortex during deteriorated hand grasping (left) was observed in the early stages, whereas being unable to detect in the functionally recovered period. When simultaneous bilateral hand grasping was performed, the activated signal around infarct region was enlarged in recovered period. The functionally neural reorganization processes relating recovered hand movement after localized cortical infarction (precentral knob) was suggested.     

Clinical characteristics of seafood allergy and classification of 10 seafood allergens by cluster analysis]

The aim of this study was to investigate the clinical characteristics of children who showed sensitization to any type of seafood and to classify the 10 seafood allergens based on IgE reactivities by a cluster analysis. In children with bronchial asthma (BA) and/or atopic dermatitis (AD), we defined the 'seafood' group as 23 patients having any type of seafood specific IgE antibody (CAP system). We analyzed the clinical features, the serum total IgE and each allergen specific IgE level. In addition, ten seafood allergens were also classified by a cluster analysis. Three patients revealed immediate hypersensitivity to some seafood. The frequency of patients with AD and the total IgE in the seafood group were high and the patients in this group were tend to be sensitized to multiple allergens. Seafood allergens were classified into 4 groups, 1) salmon, sardine, horse mackerel and mackerel, 2) cod and tuna, 3) octopus and squid, and 4) crab and shrimp, by a cluster analysis. These findings corresponded to the biological classification and the classification by the reported common allergens among various types of seafood. Based on our findings, this classification is therefore considered to be useful when selecting allergens to screen for sensitization to seafood.     

Focal Perivascular Alterations of White Matter in Herpes Simplex Encephalitis —A Histological and Immunocytochemical Study—

Abstract: Several cases of human herpes simplex encephalitis treated with Vidarabin have been investigated with the histological and immunocytochemical techniques. Cases with a subacute evolution revealed areas of focal perivascular myelin destruction in the white matter. The distribution of herpes simplex antigen did not show any preferential localization of the virus in perivascular oligoglia! cells. In contrast, a spatial and temporal relationship has been found between the appearance of immunoglobulin-bearing cells around the vessels and that of areas of focal perivascular myelin damage. Therefore, it is postulated that the areas of focal destruction of myelin are not related to the cytotoxic effect of the virus but are rather dependent on the immune response of the host.     

Myosin light-chain kinase inhibitor, 1-(5-chlornaphthalene-1-sulfonyl)-1H-hexahydro-1,4-diazepine (ML-9), inhibits catecholamine secretion from adrenal chromaffin cells by inhibiting Ca2+ uptake into the cells

For determination of whether myosin light-chain kinase (MLCK) is involved in the secretory mechanism of adrenal chromaffin cells, the effect of a preferential inhibitor of the enzyme, 1-(5-chlornaphthalene-1-sulfonyl)-1H-hexahydro-1,4-diazepine (ML-9), on catecholamine secretion from cultured bovine adrenal chromaffin cells was studied. ML-9 did not affect basal catecholamine secretion, but inhibited catecholamine secretion stimulated by acetylcholine, high K+, veratridine or palytoxin. At similar concentrations to those inhibiting the secretion of catecholamine, ML-9 also inhibited increased [45Ca]2+ uptake by the cells induced by these stimulants. However, it did not inhibit catecholamine secretion induced by the Ca2+ ionophore A23187. Moreover, it did not affect catecholamine secretion from digitonin-permeabilized cells induced by a micromolar Ca2+ concentration in the presence of Mg ATP. These results indicate that ML-9 inhibits catecholamine secretion from adrenal chromaffin cells by inhibiting the transmembrane Ca2+ uptake mechanism, but not by inhibiting the intracellular Ca2+-dependent mechanism. The possible role of MLCK in stimulus-secretion coupling in adrenal chromaffin cells is discussed.     

Differences in contact lens-induced responses in the corneas of Asian and non-Asian subjects.

PURPOSE: To investigate ethnic differences in corneal endothelia bleb formation and corneal swelling during contact lens wear under closed-eye conditions, with the use of lenses of varying oxygen transmissibilities (Dk/L) in Asian and non-Asian subjects METHODS: Asian and non-Asian contact-lens naive subjects were fitted with one of four different types of contact lenses at each 20-minute session. During lens wear, the eyelid was closed Endothelial bleb formation was determined by specular microscopy or confocal microscopy immediately on eye opening. Some Asian contact-lens naive subjects were asked to wear one of two types of lenses (Dk/L values of 24 and 175) on each eye for 1 hour Corneal swelling was determined immediately after opening of the eyes. RESULTS: Asian subjects had a significantly higher degree of endothelial bleb formation than the non-Asian population for all closed eye conditions, p=0.0001. There was no significant difference in endothelial bleb formation between closed eves with or without lenses in the non-Asian population. There was a significantly higher degree of bleb formation with low Dk/L lenses, < or = 40, compared to high Dk/L lenses, > or = 110, in Asian subjects, p=0.0001. CONCLUSIONS: There is a significant difference between Asian and non-Asian subjects in the corneal response to stress. Under a closed eyelid only contact lenses with very high levels of Dk/L do not cause cornea stress in Asian patients.