Correlation of clinical improvement with pontine lesions in opsoclonus-myoclonus syndrome

A total of 868 patients aged 25 to 68 with various types of occlusion and dentition defects were examined. 212 (24.4%) patients presented with a shortened interalveolar distance (height of occlusion). Abnormal abrasion of hard dental tissues, dentition defects in the lateral sections of dental arches, abnormal occlusion (deep or prognathic), reduced tolerance of periodontal tissues, and dysfunction of the masticatory muscles (bruxism) were found to be the pathogenetic factors leading to the development of this condition. Dysfunction of the masticatory muscles was detected in 41.5% of patients with shortened interalveolar distance and dysfunction of the temporomandibular joint in 9.9%; moreover, in many of them traumatic occlusion and disorders in the regional circulation in the periodontium of teeth exposed to overexercise were observed. Orthodontic treatment of patients with shortened interalveolar distance was carried out in two stages: the first stage consisted in functional and adaptation restructuring of the maxillodental system by repair of the height of occlusion and normalization of the mandibular position on a plastic cup which the patients wore for 3 months, and then the second stage ensued, at which they were fitted with dentures. Good results were attained in 95.7% patients.     

Marfan syndrome with coronary artery lesions in a North American Indian

From a preliminary evaluation, many of the disadvantages of the conventional arch bars appear to be overcome with this new technique. Of particular importance is that no anesthetic is required during placement of the arch bars except when needed for aligning displaced segments or open reductions. The laboratory work can be delegated to a technician, and operating time rarely exceeds an hour. Further evaluation is being done.     

Familial granulomatous arthritis (Blau syndrome) with granulomatous renal lesions

PURPOSE: To identify the BIGH3 gene mutation in 10 unrelated Japanese individuals with granular corneal dystrophy. METHODS: Genomic DNA was obtained from each patient's leukocytes. Exons 4 and 12 of the BIGH3 gene were amplified by polymerase chain reaction and were directly sequenced. RESULTS: Nine of these patients were found to have the R124H mutation, whereas only one had the R555W mutation. Slit-lamp examination showed that the granular corneal dystrophy associated with each mutation is different. CONCLUSIONS: These results, together with our previous findings, show that the classic form of granular corneal dystrophy associated with the R555W mutation is rare in Japanese patients, whereas granular corneal dystrophy accompanied by amyloid deposits and associated with the R124H mutation, Avellino corneal dystrophy, is more common. Direct examination may be insufficient in the proper diagnosis of corneal dystrophy, and BIGH3 mutation analysis may be required.     

Guillain-Barré syndrome: morphological lesions and their relations with clinical manifestations

Acute demyelinization of the peripheral nervous system is a characteristic feature of Guillain-Barré syndrome. Lesions of the spinal roots predominate with more or less diffuse multifocal peripheral demyelinization. Inflammatory lesions of the distal peripheral nervous system are less common. Segmentary demyelinization of nerve fibers accompanying inflammatory lesions show characteristic penetration of macrophages within a normal appearing myelin sheath. The myelin on the segments concerned is rapidly destroyed by phagocytosis and removed with the macrophages. Once the debris has been removed, the axons are remyelinized after proliferation of the Schwann cells. In monophasic cases, the entire processes occurs over a 2-3 week period allowing remyelinization and rapid functional recovery. In prolonged cases, flare-ups of demyelinization, non remyelinization or damage to the axons can retard recovery. The mechanism and impact of axon damage which accompanies demyelinization at various degrees and which may predominate in certain cases, is discussed here.     

Variables discriminating between cryptogenic glomerular lesions in adults with the nephrotic syndrome

A univariate analysis of individual clinical and biochemical values of adult patients with cryptogenic nephrotic syndrome has shown that significant differences exist between patients with proliferative glomerulonephritis, 'minimal change' nephritis and membranous nephropathy. For any given adult patient with the condition, the most likely clinical diagnosis is proliferative disease and the next, minimal change. These two diagnoses together account for most cases. The best clinical discriminants between them are the systolic blood pressure and plasma cholesterol concentration. If the systolic pressure is greater than 145 mm Hg proliferative disease is more likely, but if the cholesterol is greater than 530 mgm/100ml, a minimal change lesion is more likely. A scattergram for combining these variables in clinical practice is given, showing a zone of uncertainty where renal biopsy would be indicated. Although single variables do not permit discrimination between membranous nephropathy and the other two groups, it is suggested that analytical techniques where combinations of variables are used may be helpful, and should be developed.     

Morphologic and immunohistochemical characterization of the hepatic lesions associated with European brown hare syndrome

Liver lesions were studied in 40 free-living adult European brown hares (Lepus europaeus) and varying hares (Lepus timidus) of both sexes that had died in Sweden with the viral infection European brown hare syndrome (EBHS). The lesions were characterized by their histopathologic, immunohistochemical, and electron microscopic findings. Periportal to massive coagulation necrosis was a distinctive feature of EBHS. Lytic necrosis, inflammation, fatty degeneration, and cholangitis occurred variably. Accumulation of basophilic granules in the cytoplasm of hepatocytes was commonly observed; these lesions corresponded ultrastructurally to mitochondrial calcification. Viral antigen was revealed in the cytoplasm and nucleus of hepatocytes and in the cytoplasm of macrophages.     

Space-occupying lesions as a cause of tarsal tunnel syndrome

In diagnosing the etiology of tarsal tunnel syndrome, the practitioner must be aware of space-occupying lesions as a possibility. These masses, rarely found beneath the laciniate ligament, can occur. Satisfying results have occurred after removal of these lesions. Careful dissection will assure preservation of the nerve and prevent sensory or motor loss.     

Cerebral white matter lesions in sudden infant death syndrome

In 21.6% of infants who died of sudden infant death syndrome, the cerebral white matter showed areas of leukomalacia. Of those infants with congenital heart disease, 24.8% had lesions, whereas 4.4% of infants who died from known acute causes had lesions. The sites of the cerebral white matter lesions, subcortical or periventricular, seem to be related to the age of the infant.     

Adrenal lesions in calves dying from endotoxin shock, with special reference to the Waterhouse-Friderichsen syndrome

The influence of hypoxemia on the brain content of several organic acids and NH+4, AND ITS RELATIONship to the accompanying hypocapnia was studied in unanesthetized rats subjected to hypoxemia for periods ranging between 2 hours and 7 days. Under acute conditions, 'mild' hypoxemia (FO2 = 6--7%), these increases were greater and accompanied by increased gamma-aminobutyric acid (GABA) and decreased glutamic and aspartic acid levels; glutamine and NH+4 remained normal. When hypocapnia was prevented, 'severe' hypoxemia induced only a rise in GABA and slight elevations in lactic and alpha-ketoglutaric acid. During prolonged severe hypoxemia, the effects on the brain amino acids were maintained throughout, indicating that they are independent from the intracerebral pH which should progressively normalize. The effect on lactic acid gradually disappeared. The results show that during hypocapnic hypoxemia the rise in brain GABA is hypoxemia dependent, the decrease in glutamic and aspartic acid is hypocapnia dependent and the increase in lactic acid is in a large way alkalosis dependent.     

HB-GAM is a cytokine present in Alzheimer''s and Down''s syndrome lesions

OBJECTIVE: Our purpose was to evaluate peripheral joint laxity during pregnancy and to determine whether serum relaxin levels are associated with increased joint laxity. STUDY DESIGN: A prospective observational study was performed. RESULTS: A significant increase in joint laxity was found in five of seven peripheral joints over the course of the pregnancy and post partum. There was no correlation with serum relaxin levels. There were no significant differences in joint laxity on the basis of parity, age, or prepregnancy exercise levels. CONCLUSIONS: Peripheral joint laxity is noted to increase as pregnancy progresses. The cause of this change is undetermined.     

Gingival lesions in a patient with dermochondrocorneal dystrophy (Fran?ois syndrome). A case report

This report is concerned with gingival manifestations associated with a case of dermochondrocorneal dystrophy (DCCD) or Fran?ois syndrome occurring in a 42-year-old woman. Our Department treated this patient for 15 years. Oral examination of this case revealed a diffuse enlargement and severe inflammation of the attached gingiva. Systemic findings were similar to those reported in the literature for patients with DCCD. Firm papules 3 mm wide, localized on the face and on the dorsal surface of the hands, were associated with corneal involvement and progressive and severe articular disorder. Because they recurred after surgical ablation, the gingival lesions became an important problem in the management of the patient. After 10 years of unsuccessful treatment limited to scaling, oral hygiene control and mouth rinses with 0.2% chlorexidine solution, the patient was submitted to extraction of the remaining teeth, remodelling osteoplasty and cutaneous graft. An acrylic full denture was inserted. In a follow-up of 7 years, good results for the oral health of the patient were seen.     

Grooming behavior in cats with pontile lesions and cats with tectal lesions.

Because an abnormal grooming behavior that is mediated by the superior colliculi is elicited from cats with pontile lesions, an ablation study of these structures was conducted in 40 cats to specify quantitatively the changes in grooming behavior. Ss that underwent the surgical procedure except for the lesion and Ss with lesions of the auditory and visual cortices served as controls. Time-lapse motion pictures of the Ss in their home cages were taken, and statistical analyses of the grooming behavior shown on the films indicated that Ss with pontile lesions and those with tectal lesions spent less time grooming, had shorter grooming bouts, and failed to exhibit the normal temporal pattern of grooming behaviors. Other studies revealed that Ss with pontile or tectal lesions were deficient in removing tapes stuck on their fur. A sensory-loss hypothesis appeared to account for some of the changes, but a deficit in endogenous control of the grooming behaviors also was indicated. The literature on grooming behavior related to peripheral vs endogenous control is reviewed, and the role of the superior colliculi as a higher order integrative center for complex behaviors is emphasized. (47 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

POEMS syndrome with vascular lesions: a role for interleukin-1beta and interleukin-6 increase--a case report

The authors describe the case of a 60-year-old man with POEMS syndrome associated with vascular lesions. The patient had osteosclerotic myeloma IgA (lambda), polyneuropathy, endocrinopathy, and skin changes. Subsequently, he developed gangrene of the lower limbs with no response to heparin therapy. The humoral study showed thrombocythemia, high levels of interleukin-1beta (IL-1beta) and IL-6 and of some coagulative/fibrinolytic and endothelial factors (von Willebrand factor, plasmin-antiplasmin complexes, plasminogen activator, and endothelial adhesion molecule ICAM-1). The authors suggest that these factors, induced by the increased levels of cytokines, could be responsible for microvascular damage, gangrene, and heparin resistance.     

Improvement of lesions shown on MRI and CT scan by administration of dichloroacetate in patients with Leigh syndrome

Brain lesions exhibited on MRI and CT scan in 2 patients with mitochondrial encephalomyelopathy representing Leigh syndrome were improved by administration of dichloroacetate (DCA). One patient had pyruvic acid dehydrogenase complex (PDHC) deficiency, the other had complex I deficiency. The efficacy of DCA was transient in the patient with the PDHC deficiency, lasting for about 2.5 months. The patient died at the age of 6, about 2 years after the initiation of DCA treatment. DCA administration was started in the patient with complex I deficiency when he was 15 months old and it is still effective at his present age of 24 months. His motor ability is developing, and he could walk without support at the age of 19 months. DCA administration should be tried in patients with mitochondrial diseases.     

Kindler syndrome: a rare cause of desquamative lesions of the gingiva

Kindler syndrome is a rare syndrome with cutaneous and intraoral manifestations. It has been suggested that there is an overlap between this syndrome and another called Weary syndrome. Only 68 cases of Weary and Kindler syndromes have been reported, with fewer solely attributed to Kindler syndrome. The salient cutaneous features are neonatal bullae, poikiloderma, photosensitivity, and acral atrophy. This article presents the clinical intraoral findings of two siblings of consanguineous descent diagnosed as having Kindler syndrome. Both had an erythematous and erosive appearance of the gingiva; one sibling had poor oral hygiene and a rapidly progressive form of periodontal disease; the other, whose oral hygiene was acceptable, had no detectable bone loss.     

Sensorimotor syndrome produced by lesions of the amygdala and lateral hypothalamus.

Experimented with 7 male Long-Evans hooded rats. Small unilateral lesions of the amygdala, lateral hypothalamus, or nearby structures resulted in an inability to orient and localize, by an appropriate movement of the head, stimuli in the contralateral visual and somatosensory fields. This is neither a primary sensory nor motor deficit. Conditioning experiments revealed that the linkage between contralateral sensory and motor fields has been disrupted, possibly reflecting damage to fibers which connect sensory and motor areas within a hemisphere. This sensorimotor syndrome can account for the placidity and decreased aggressive, social, and feeding behaviors commonly observed following lesions of these areas. (15 ref.) (PsycINFO Database Record (c) 2010 APA, all rights reserved)