Three cases of chondrodermatitis nodularis helicis (CDNH) in patients with the limited form of systemic sclerosis are described. Two of the three cases were noted during regular follow-up visits and the third case was found as part of a survey of the 21 other patients with systemic sclerosis routinely seen ...

We report the case of an infant who presented with isolated cutaneous manifestations of Langerhans cell histiocytosis before the evolution of systemic features. In the transition period, at 9 months of age, nail unit changes became prominent, and persisted throughout the duration of systemic treatment. A change in clinical features ...

Colour Doppler sonography (CDS) is becoming an increasingly popular method of examining the deep venous system of the lower limb, particularly in the investigation of possible deep venous thrombosis (DVT). We describe a further application of CDS observed in three intravenous (i.v.) drug abusers who presented with symptoms mimicking a ...

A black girl was born with flexion contractures and experienced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasia, perianal nodules, torticollis, diarrhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences. ...

A system for the monitoring and review of a large number of cases seen for assessment and outpatient treatment by a multidisciplinary child and adolescent psychiatric service is described. The system permits monitoring of all cases to closure, and clinical review of treatment for those cases whose outcomes are judged ...

Polyarteritis nodosa (PN) is frequently cause of both central and peripheral neurological disorders. However, involvement of the central nervous system is rare at the beginning of the disease. In this paper we report a case of a 38-year-old woman hospitalized in our Neurosurgical Service because of left hemiparesis in presence ...

The MR findings of a biopsy-proved case of primary angiitis of the central nervous system are described and compared with previously reported cases. Multiple punctate and linear areas of gadopentetate dimeglumine enhancement were present in the brain stem and white matter and appear to correspond to the inflammatory reaction of ...

Munchausen syndrome by proxy (MSP) is a recently described form of child abuse in which a caregiver fabricates or induces symptoms in a child in order to attract medical attention. The epidemiology, clinical features, diagnosis and the spectrum of MSP are reviewed. Central nervous system symptoms are a common presentation ...

This case report describes a systemic reaction occurring in a 12-year-old female following presumed envenomation by a brown recluse spider (Loxosceles reclusa). The systemic reaction included self-limited hemolysis necessitating blood transfusion. The clinical course and management are described and compared with those of previously reported cases of systemic loxoscelism.

Scleroderma is primarily a cutaneous disorder but systemic involvement is common and neurological complications are increasingly recognized but previously poorly documented. Few cases of peripheral nervous system involvement in scleroderma have been reported. The peripheral nervous system, however, is rich in connective tissue and sclerodermal involvement may not be as ...

The UltraSTAR structured data entry system is now in routine use for reporting ultrasound studies at Brigham and Women's Hospital, having been used for 3722 reports in its first ten months of service. Reports entered through GUI-based forms are uploaded via HL7 to a radiology information system and distributed through ...

Over the past 10-15 years, workers using conventional computing approaches have attempted to provide an accurate assessment of fetal condition during labour based on the cardiotocogram (CTG) alone. These have not proved successful perhaps because the correct interpretation of fetal condition also requires physiological knowledge, specific patient information, knowledge of ...

Three cases of rebound positional nystagmus (RPN) are discussed in the present study. In all cases, spontaneous nystagmus was absent in the primary position, but positional nystagmus appeared in the side down position, and then nystagmus in the reversed direction appeared when the patient was returned to the primary position. ...

OBJECTIVE: Primary angiitis of the central nervous system (PACNS) has been considered a rare and highly fatal disorder when untreated. In recent years the disease has been increasingly diagnosed by cerebral angiography, often without histologic confirmation. We have questioned whether cases of PACNS diagnosed on the basis of angiography alone ...

Transitional forms of the recent classes of vertebrates are only known in paleontology. The well described examples are: Eusthenopteron foordi (Crossopterygii), Ichthyostega and Acanthostega (Labyrinthodontia) between Osteichthyes and Amphibia, Seymouria baylorensis (Amphibiosaria) between Amphibia and Reptilia, Archaeopteryx lithographica (Archaeornithes) between Reptilia and Aves, and the mammal-like reptiles Pelycosauria, Therapsida and ...

We describe a 50-year-old Chinese woman who had severe gastrointestinal manifestations from systemic sclerosis complicated by spontaneous pneumoperitoneum in the absence of either visceral perforation or pneumatosis cystoides intestinalis. This is a rare complication of systemic sclerosis; only four other cases have been reported. Recognition of this condition is important ...

The prognosis in 52 patients with juvenile rheumatoid arthritis (JRA) was studied. There were 35 cases of systemic onset, 12 of polyarticular onset and 5 of pauciarticular onset. Thirteen systemic cases developed a polycyclic course with chronic polyarthritis. Many monocyclic JRA in systemic cases subsided within 1 year. There were ...

Amyloidosis is a manifestation of a group of diseases resulting from the variable infiltration of multiple organs by a fibrillar protein called amyloid. Hepatic involvement in amyloidosis is common both in the primary and in the secondary forms, whereas clinically-dominant liver amyloidosis is relatively rare. The authors describe a case ...

Harrison (1918: J. Exp. Zool. 25: 413-461) described a developmental field as an "equipotential self-differentiating system." The present study was undertaken to address the question: To what extent can be pre-limb territory of a chick embryo be considered a developmental field? To what extent is the chick pre-limb territory an ...

We performed a population-based study of over 100,000 users of diclofenac, naproxen, or piroxicam to identify cases of important blood, skin, central nervous system, kidney, pancreas, or pulmonary disorders caused by these drugs. In three cases a causal relation seemed likely; one of hemolytic anemia attributed to diclofenac, one of ...

The classification of hemifacial microsomia (HFM) aids in diagnosis, treatment planning, prognostic predictions and data evaluation. The aetiological and phenotypic heterogeneities of HFM, however, make its classification problematic. This study used data from 50 patients to examine the classification of HFM and to compare two systems: OMENS and SAT. The ...

Progeria is a rare syndrome, with an estimated incidence of 1 per 250,000 births. Although children with progeria have the appearance of premature aging or senility, the term is misleading because reported cases of progeria have not manifested most physical or biochemical aspects of old age. Many children with progeria ...

Incidental epidermolytic hyperkeratosis (EH) has been reported in a variety of lesions. We describe here incidental EH in a patient with progressive systemic sclerosis (PSS). We reviewed 108 skin biopsies from our cases of PSS and localized scleroderma, but this finding was seen only in the present case. Focal acantholytic ...

A 59-year-old woman showed chronic progressive autonomic failure consisting of orthostatic hypotension and dyshidrosis. No signs of Parkinsonism, cerebellar or peripheral neuropathy were observed. Autonomic nervous function tests and findings of biopsied sural nerve suggested that this case had both sympathetic and parasympathetic dysfunctions, mainly postganglionic. A decrease in the ...

We observed cases of hearing loss in which hearing deteriorated when steroid therapy was discontinued and improved upon re-administration. We identified these as cases of steroid-responsive sensorineural hearing loss (SNHL). The diagnostic criteria for determining steroid-responsive SNHL were stated, and 10 cases were examined. The 10 cases of steroid-responsive SNHL ...

This article presents a brief review of the morphology and physiology of molluscan statocysts. These sense organs provide the animals with information on orientation and, in some cases, velocity of movement. They vary in sophistication from rudimentary, to organs almost as complex as those found in higher vertebrates. The morphology ...

A summary review of leukocyte function in 42 published cases of glycogen storage disease Ib is presented. Polymorphonuclear and monocyte dysfunctions were evidenced in the majority of cases, whereas lymphocytes appeared to be unaffected. Phagocyte dysfunctions comprised in vivo mobilization and motility, in vitro random and directed migration, and one ...

Eleven patients with chronic progressive sensory ataxic neuropathy were examined clinicopathologically. Three cases were associated with primary Sjögren's syndrome (SS-SAN) and the others were considered to be idiopathic (ISAN). The major clinical symptom in both was loss of proprioceptive and kinesthetic sensation with some impairment of superficial sensation, with multifocal ...

A case of brachial plexus block is presented, following a bee sting in the posterior triangle of the neck. The onset of neurological symptoms was rapid as was their subsequent resolution. Delayed peripheral neurological symptoms believed to have an immunological basis have been reported in response to stings from bees ...

In our study, the characteristics of 114 patients with systemic sclerosis (SSc) are discussed with emphasis on the subgroup of cases whose onset of disease occurred above the age of 60 years. Seven out of the 9 cases showed symptoms of diffuse cutaneous systemic sclerosis with an extensive skin involvement, ...

Prototheca species are ubiquitous, aerobic, unicellular algae closely related to the green algae Chlorella. Their involvement in human disease--in both immunocompetent and immunocompromised patients--has been reported with increasing frequency. The wide array of presentations has included cutaneous, subcutaneous, mucosal, bursal, catheter-related, and (in rare instances) systemic disease. We report a ...

An acquired form of hydrocephalus due to aqueductal stenosis developing as a sequela of mumps virus infection of the central nervous system is presented. Percutaneous third ventriculostomy and interventriculostomy (aqueduct cannulation) were performed using a flexible fiberoptic ventriculoscope. The aqueduct was blocked with amorphous material but was cleared with the ...

OBJECTIVE: To report a case of central and extrapontine myelinolysis with unusual behavioural manifestations that developed after slow (not more than 0.5 mmol sodium per litre per hour) correction of hyponatraemia. CLINICAL FEATURES: A 51-year-old Caucasian woman with hyponatraemia caused by psychogenic polydipsia experienced a delayed onset of behavioural changes ...

Granulomatous angiitis of the central nervous system occurred in a 43-year-old male presenting with recurrent intracerebral hemorrhage confirmed by computed tomography. A surgical specimen obtained at hematoma removal revealed granulomatous angiitis. Systemic investigation found no underlying cause for the hemorrhage. Although the incidence is very low, this condition should be ...

Nonteratomatous intrapulmonary neuroglial heterotopia not associated with birth trauma or frank vascular embolization has been described rarely. This article briefly reviews the literature, and presents two additional cases of intrapulmonary neuroglial heterotopia. We found 14 cases in the literature. Twelve of these cases had central nervous system disruption, where neuroglial ...

Systemic sclerosis sine scleroderma can present in some patients as pulmonary interstitial fibrosis. Until now ten cases with this particular clinical variant, all men, have been reported in the literature. The knowledge of systemic sclerosis sine scleroderma presenting as lung interstitial involvement is important in clinical practice for an early ...

The authors present two cases of systemic blastomycosis successfully treated with fluconazole. In one case, the disease involved the respiratory tract, and the central nervous system was presumed to be involved. The second case consisted only of pulmonary blastomycosis. Both patients were treated with oral fluconazole 200 mg twice per ...

Three cases of transient migratory osteoporosis treated with sympathetic blocks are described. Review of the literature in conjunction with the presentation and treatment of these 3 cases led to the formulation of a hypothesis of a common mechanism (mediated through the sympathetic nervous system) responsible for the profound osteoporosis and ...

We describe 3 cases of immune-mediated cytopenia occurring after bone marrow transplantation (BMT). In 1 case, only the platelet line was affected, whereas in the other 2 cases more than 1 cell lineage was involved simultaneously. Two of the cases presented with falling peripheral blood counts following apparently normal early ...

DESCRIPTION: There have been numerous case reports of adverse reactions involving monoamine oxidase inhibitors (MAOIs), including reactions that developed when one MAOI was substituted for another. Although most reports have usually described a switch from isocarboxazid or phenelzine to tranylcypromine, we report a case of acute central nervous system toxicity ...

In addition to the gravity-dependent position, we believe that other particular anatomical features may be important in the retention of stone debris in the lower calices after extracorporeal shock wave lithotripsy (ESWL). We analyzed the inferior pole collecting system anatomy in 146, 3-dimensional polyester resin corrosion endocasts of the pelviocaliceal ...

Chondrodysplasia Punctata is a rare condition in which there are numerous punctate areas of calcification in the epiphysis. The disease involves not only skeletal but also the cardiovascular, cutaneous, ocular and central nervous system. We present a newborn case with clinical as well as radiographic findings similar to Chondrodysplasia Punctata.

LiverSoar is a flexible knowledge-based system that can learn from its problem-solving experience in individual cases. Within an opportunistic abductive framework, it can diagnose liver diseases from findings in liver tissue biopsies. As more cases are encountered and more recognition knowledge is acquired, the system becomes prepared for most of ...

Multifocal gliomas fall into four main categories: diffuse, multiple, multicentric and multiple-organ. Multicentric gliomas are uncommon lesions of the central nervous system. Even more so are multicentric gliomas lying both above and below the tentorium (16 cases to date, as far we know). We report a clinical series of 7 ...

The association of xanthogranulomatous pyelonephritis and systemic amyloidosis is extremely rare. To our knowledge, despite innumerable cases of xanthogranulomatous pyelonephritis reported in the literature, this association has been described on only 2 occasions. Clinical, analytical and radiographic findings can be highly suggestive of the diagnosis. We report on 2 patients ...

Suppurative pylephlebitis is an unusual disease with an associated high mortality. Most cases are a complication of an intra-abdominal septic focus, but some may occur de novo. In the case we have presented, the diagnosis was made by modern imaging techniques that showed thrombus predominantly within the upper portal venous ...

The knowledge bases (KBs) of diagnostic decision support systems are often incomplete, and gaps in the KB could potentially lead systems to reach diagnoses that are implausible to physicians. To investigate this possibility we studied Iliad (Version 2.01), a computer consultant system that generates differential diagnosis across the domain of ...

Iniencephaly is a rare congenital malformation of central nervous system and axial skeleton. Eight cases were seen in the Department of Obstetrics and Gynaecology, Jinnah Post-Graduate Medical Centre over a 12 month period constituting 0.1% of all deliveries conducted, the estimated frequency being 1: 850 deliveries. The purpose of this ...

Central nervous system disease in cutaneous T cell lymphoma is uncommon and is usually not considered in standard therapeutic regimens. We report three patients who had cutaneous T cell lymphoma with involvement of the central nervous system and review the cases of 28 such patients reported in the literature. Potential ...

Twenty nine cases of central nervous system infection due to Monosporium apiospermum have been reported. Six of them occurred after an aspiration pneumonia following a near drowning. The case of a 53 year old man is reported: M apiospermum was isolated from cerebrospinal fluid, sputum, urine and from the mud ...