Neurological manifestations of bronchogenic carcinoma were studied in 50 cases, 42% of whom showed neurological abnormalities with 6% having more than one type. Recurrent laryngeal nerve paralysis (20%) was the commonest, phrenic nerve paralysis (2%), paraneoplastic syndrome (12%), Pancoast's syndrome (10%) and metastatic involvement of central nervous system (4%) were ...

An 18-year-old woman with systemic lupus erythematosus developed neuropsychiatric disorders, including aseptic meningoencephalitis, organic brain syndrome and seizure. A series of computed axial tomography scans revealed the progression of marked atrophy of the right cerebral hemisphere for a period of 3 years without occlusion or stenosis of large vessels on ...

Allylnitrile, crotononitrile and 2-pentenenitrile induce this behavioral syndrome, which includes persistent random circling, head twitching and backward running. The effects of neurotropic agents, with a selectivity for the alpha 1 or alpha 2 adrenoceptor, were examined on the nitrile-induced behavioral abnormalities. The alpha 1 agonist phenylephrine, the alpha 2 agonist ...

Four patients suffered a distinctive neurologic syndrome after undergoing profound hypothermia and complete circulatory arrest for congenital heart lesion repair. Symptom onset was delayed 24-120 hours postoperatively. The syndrome consists of choreoathetosis and oral-facial dyskinesias, hypotonia, affective changes, and pseudobulbar signs (CHAP). Precise anatomic localization is uncertain. Magnetic resonance imaging ...

A 50-year-old woman with systemic lupus erythematosus was admitted in an obtunded state. An EEG recorded while she was unconscious demonstrated one episode of rhythmic sharp wave activity in the left occipital area that lasted for 5 minutes and was followed later in the same recording by an episode of ...

A syndrome of dorsal midbrain dysfunction in association with a central nervous system anaerobic diphtheroid infection is described. Two cases of infection with Propionibacterium acnes manifested as shunt malfunctions with a clinical dorsal midbrain syndrome. Magnetic resonance images showed increased signal in the midbrain tectum which has decreased slowly over ...

An 8-year-old boy with reticular erythematous mucinosis syndrome had erythematous plaques on his chest, face, and arms for three years. Sun exposure resulted in pruritus and increased lesions. Histologic examination revealed a perivascular mononuclear cell infiltrate with hematoxylin and eosin staining, positive staining material between the dermal collagen bundles with ...

Mentally handicapped adults with and without Down's syndrome performed single movements and movement sequences following both verbal and demonstration cueing conditions. While the type of cue did not influence the performance of control subjects, Down's syndrome individuals exhibited more error following verbal cueing. These findings could not be explained on ...

It has been emphasized that an increased oxidative damage can exist in Down syndrome (DS), and that superoxide dismutase (SOD-1) seems to play a role in the pathogenesis of this disorder. We have studied the antioxidant system SOD-1, catalase, glutathione peroxidase (GSH-Px) and reduced glutathione (GSH) in erythrocytes of DS ...

Nine of 122 patients dead from AIDS in central London presented with neurological disease, confirmed pathologically in seven. Seven had no other major systemic manifestations. AIDS needs to be considered in the differential diagnosis of meningitis, dementia, diffuse and focal encephalopathies, brainstem syndromes, myelopathy, visual failure and peripheral nerve syndromes. ...

Immunohistochemical analysis was performed on the annular erythema associated with Sjögren syndrome. This type of annular erythema is characterized by a doughnutlike appearance with an elevated border and central pallor; it is distinct from subacute cutaneous lupus erythematosus. Histologically this erythema is characterized by coat sleeve-like infiltration of lymphocytes around ...

We have described previously the clinical features of a unique group of anti-Ro(SS-A) antibody positive Sjogren's patients who have cutaneous features of lupus erythematosus, most commonly subacute cutaneous lupus erythematosus, defined as the Sjogren's/lupus erythematosus overlap syndrome. Three of these patients are also mothers of infants with the neonatal lupus ...

Patients eventually diagnosed as having the fibromyalgia syndrome often have symptoms which suggest alternate diagnoses such as peripheral neuropathy, spondylitis, metabolic myopathy, polymyalgia, early rheumatoid arthritis, early systemic lupus erythematosus or a chronic fatigue syndrome. Delay in diagnosis of fibromyalgia often proves costly and frustrating to the patient and may ...

The recent Australian epidemic of localized fibrositis syndrome is used to highlight the role of compensation and medicolegal systems on the clinical features and outcome of fibrositis syndrome. It is suggested that psychological factors are of great importance when this syndrome occurs after minor injury or de novo in the ...

This study presents particular clinical manifestations in 7 patients with autoiMmune diseases: rheumatoid purpura with right crural nerve paresis (1 case), Stevens-Johnson syndrome with encephalomyeloradicular syndrome (1 case) and left Wallenberg syndrome (1 case), rheumatoid arthritis with right parieto-occipital syndrome (1 case) and Gowers local panatrophy (1 case), systemic lupus ...

The lumbar spine is an impressive mechanical system but it can fail from injury or abuse. This paper reviews four recognisable back pain syndromes, which result from mechanical failure, symptomatic disc protrusion, root entrapment syndrome, segmental instability and neurogenic claudication--their clinical presentation, and what is understood about their multifactoral aetiology.

Preliminary observations suggested that arterial and arteriolar necrosis in vasculitis of the peripheral nerve leads to local haemorrhage and subsequent deposition of haemosiderin. This pigment is more readily recognized in the nerve by the sensitive Perls' test for iron than by relying on recognizing its yellow colour. To support the ...

We describe a patient with systemic lupus erythematosus (SLE) and overlapping syndrome who had repeated gastrointestinal (GI) symptoms such as nausea, vomiting and malabsorption. With a subacute process, she developed paralytic ileus and contracted bladder, and died of perforation of the ileum. When 13 reported cases with lupus cystitis were ...

Prior neurologic illness or CNS insult of any kind is known to increase the vulnerability to neurotoxicity of lithium. In this event the occurrence of neurotoxicity does not correlate with serum lithium levels. The authors describe a patient with hemiparesis who developed the syndrome of irreversible lithium-effectuated neurotoxicity (SILENT) while ...

The histopathological observations in the case of a 24 year old woman with Chediak-Higashi syndrome are described. There were characteristic features of cytoplasmic giant granules in various cells and lymphohistiocytic infiltration in various tissues. Amyloid deposits, which have not been reported previously in human Chediak-Higashi syndrome, were systemically noted and ...

We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti-Sj-T antibodies found in the original Rowell's syndrome ...

Dizziness as defined herein will include an illusion of motion caused by various degrees of ischemia to the vestibular pathway or its interconnecting pathways. "Syndrome," such as the lateral medullary syndrome, denotes a macroinfarct, while a microinfarct or an area of incomplete infarct (where there may develop an incomplete degeneration ...

A woman with Hodgkin disease presented a neoplastic syndrome of unusual magnitude affecting both the CNS and PNS. The severity of the clinical pattern contrasted strongly with the paucity of neuropathological findings. The report is followed by a brief discussion of the etiological and clinical problems of paraneoplastic syndromes affecting ...

Fourteen of 52 unselected patients with systemic lupus erythematosus (SLE) (27%) had ligamentous derangement demonstrated by either Jaccoud's syndrome and/or patellar tendon elongation. Three cases had only Jaccoud's syndrome, 4 isolated patellar tendinous laxity, while the remaining 7 presented both findings. Jaccoud's syndrome and/or tendinous laxity were not associated to ...

We investigated the prevalence and type of neurologic involvement in Behçet's syndrome in a prospective protocol. Of 323 consecutive patients with Behçet's syndrome seen during a 12-month period, 46 underwent neurologic evaluation because of headaches and/or neurologic symptoms and signs. Only 17 (5.3%) were found to have involvement of the ...

A system is described running on any IBM-compatible personal computer operating under MS-DOS which permits an expert in pediatric dysmorphology to formulate dysmorphic features and syndromes in a knowledge base, and a user to enter, analyze, store and retrieve case data. For each case, the system calculates the Bayesian probability ...

Immunotactoid glomerulopathy is a distinct clinico-pathological entity which has recently been defined. The term immunotactoid refers to highly organized immune depositions appearing as rod-like microtubular structures in ultrastructural examination. We describe a patient with mixed connective tissue disease who demonstrates characteristic features of immunotactoid glomerulopathy. The diagnosis was made after ...

A 70 year old male patient admitted for coronary bypass surgery presented with a procainamide-induced lupus syndrome. This syndrome included a LLAC with a positive IgM ACA titer as well as a factor XII inhibitor. These drug-induced inhibitors were superimposed upon the patient's congenital deficiency of factor XI. The methods ...

X-ray photography was employed to analyse the postural syndromes following unilateral global and selective lesions of the vestibular apparatus in guinea pigs. Following hemilabyrinthectomy, head rotation in the horizontal plane resulted mainly from rotation of the cervical vertebrae whereas lateral head tilt was due to the rotation of thoracic vertebrae ...

The Kluver-Bucy syndrome has not been previously reported as a complication of systemic lupus erythematosus (SLE). A 48-year-old female is described who sustained several cerebral infarcts, some of which were bitemporal, due to SLE vasculopathy. She developed a complex behavioural picture consisting of global aphasia, left-side neglect, hyperorality, hypermetamorphosis and ...

A dermatomyositis-like syndrome developed in a patient treated with a nonsteroidal anti-inflammatory agent (NSAI), niflumic acid, and regressed after the cessation of treatment. Previously an eruption had occurred under treatment with another NSAI, diclofenac. Our report shows that NSAI can induce not only lupus-like syndromes but also other connective tissue ...

Recent reports suggest that the higher frequency components (around 2 Hz) of accommodative fluctuations may arise from the accommodative 'plant' and may have no role in the control of the accommodation system. The dynamic responses of accommodation in two cases with Adie's syndrome were recorded. The results showed that the ...

Elemental gold, administered parenterally and orally, is used to a moderate extent to treat rheumatoid arthritis, Sjögren's syndrome and nondisseminated lupus erythematosus. Deposition of gold salts are observable in ocular tissues during chrysotherapy. These tissues are the cornea, conjunctiva, skin, lens and retina. This case report describes ocular chrysiasis and ...

Cardiac manifestations of the mucopolysaccharidoses often include valvular regurgitation, but stenotic lesions are quite rare. This report describes a 30-year-old man with mucopolysaccharidosis type II (Hunter's syndrome) and systemic lupus erythematosus who developed severe progressive aortic stenosis and died. Autopsy examination revealed evidence of various cardiac mucopolysaccharide disease including valvular ...

A case in which anomalous systemic venous drainage occurred in association with the hypogenetic lung syndrome (scimitar syndrome) is described. The chest radiograph appearances of the anomalous systemic vein mimicked an anomalous pulmonary or scimitar vein. Angiography demonstrated that the patient also had a small anomalous pulmonary vein draining and ...

A 46 year old white woman with active systemic lupus erythematosus developed a skin rash 8 hours after intravenous urography with the non-ionic contrast medium iopamidol. Severe Stevens-Johnson syndrome with erythema multiforme, intrahepatic cholestasis, pulmonary infiltrates and acute renal failure ensued, leading to her death. Although non-ionic contrast media are ...

The literature on Werner's syndrome is scarce, and to our knowledge, no documented evidence is available to substantiate central nervous system involvement in this multisystem disease. We present a case of Werner's syndrome associated with recurrent delusional psychosis in the presence of cognitive impairment and computerised tomography (CT) radiological changes ...

Thrombocytopenia has been documented infrequently in association with congenital heart block or lupus dermatitis in the neonatal lupus erythematosus syndrome. We report the cases of two infants with transient neonatal thrombocytopenia born to mothers with connective-tissue disease. Both mother/infant pairs were Ro(SS-A) antibody positive. Although the finding of neonatal thrombocytopenia ...

The musculoskeletal system is involved in nearly all patients with SLE. Transient arthralgias or arthritis are common and, in some patients, there is a progression to rheumatoid-like nonerosive hand deformities (Jaccoud's syndrome). The major disabling type of joint disease in lupus is articular osteonecrosis, often induced by high-dose corticosteroids. Rare ...

We describe a young Hispanic man who presented with features suggestive of a diagnosis of systemic lupus erythematosus. Eventually, a bone marrow biopsy was performed, and it revealed the presence of hairy cell leukemia. This case of a lupus-like syndrome should be added to the list of reported rheumatic syndromes ...

The bilateral electrolytic lesion of the ventral mesencephalic tegmentum (VMT) induces, in the rat, behavioural deficits such as locomotor hyperactivity and disappearance of spontaneous alternation ('VMT syndrome'). When a specific 6-hydroxy dopamine (6-OHDA) destruction of the dorsal noradrenergic (NA) ascending pathway was superimposed to an electrolytic lesion of the VMT, ...

A young woman on chronic corticosteroid treatment for systemic lupus erythematosus developed a myasthenia-like syndrome 7 weeks after starting chloroquine therapy. Discontinuation of chloroquine allowed symptomatic and immunological remission within 6 months. Ocular symptoms reappeared following a second short course of chloroquine. A motor-point biopsy revealed a vacuolar myopathy with ...

Lycanthropy, the belief that one has been transformed into an animal (or behaviour suggestive of such a belief), has been described by physicians and clerics since antiquity, but has received scant attention in the modern literature. Some have even thought the syndrome extinct. However, in a review of patients admitted ...

In order to maximize biochemical, neurochemical, molecular, and pathologic information from patients with Rett syndrome, a uniform procedure has been developed for the conduct of the postmortem examination. Tissue should be prepared for freezing, for electron microscopy studies, and for standard histologic examination. For the purposes of uniformity, three central ...

The hypereosinophilic syndrome (HES) is a rare disease, characterized by a long lasting hypereosinophilia of the blood and the marrow and by the presence of varying internal symptoms and frequent neurological signs. These latter can be as well central: encephalomalacy, organic psycho-syndrome, as peripheral: polyneuropathy or mononeuropathia multiplex, autonomic neuropathy ...

The natural history of schizophrenia cannot be studied directly, but nevertheless can be inferred and conceptualized as a frame of reference for examining the heterogeneity of the syndrome and for evaluating the impact of treatment, environment, and other factors influencing its course. Alternative diagnostic systems, including DSM-III, imply differing, largely ...

This is a report of the study of progressive dementia associated with Sheehan's syndrome in a 61-year-old woman who had a 13-year history of insidious progressive decline in global cognitive abilities. At the onset, there were only slight difficulties with abstract thoughts, and a shortened attention span, but the symptoms ...

Results of clinical, serologic and histologic studies documenting an association between hereditary C6 deficiency and a connective tissue disease are provided. The propositus had systemic lupus erythematosus with prominent discoid features, Sjögren's syndrome and hyperthyroidism. Serum C6 was undetectable by radial immunodiffusion and hemolytic assays. Serologic and typing studies performed ...

A 66-year-old man with the lupus anticoagulant-hypoprothrombinemia syndrome was treated with cyclophosphamide and prednisone to correct a factor II deficiency prior to elective major surgery. Whereas the lupus anticoagulant activity persisted, functional factor II levels normalized and he underwent surgery without a bleeding diathesis. Immunosuppressive therapy may temporarily normalize factor ...

A strong correlation exists between the presence of an in vitro plasma coagulation inhibitor, named "lupus anticoagulant", and a clinical syndrome of recurrent arterial and venous thromboses, neurological abnormalities, repeated obstetrical complications, thrombocytopenia, a biologic false-positive serological test for syphilis, and a variety of rarer manifestations. This syndrome has predominantly ...