Predominant Clinical Symptoms Research Articles

Pseudomeningoencephalitic presentation of pediatric Guillain-Barré syndrome.

Guillain-Barré syndrome is an acute, autoimmune polyradiculoneuropathy that improves with immune-modulating treatment if instituted early in the illness. Preliminary diagnosis relies on the clinician's recognition of the typical symptoms and signs as supporting evidence of the illness, such as nerve conduction studies, which may not be available emergently. We report eight children with Guillain-Barré syndrome in whom the initial presentation was atypical and suggested a primary central nervous system illness. In these patients, the predominant clinical symptoms included drowsiness, headache, irritability, and meningismus, although the classic features of Guillain-Barré syndrome (weakness, hyporeflexia) were also present. The atypical presentation caused delay in diagnosis in some cases. It is important to recognize this variant of pediatric Guillain-Barré syndrome to ensure expeditious diagnosis and treatment.

Solitary fibrous tumour of the pleura: surgical treatment.

Solitary fibrous tumours (SFT) of the pleura are rare tumours originated from the mesenchimal tissue underlying the mesothelial layer of the pleura. This tumours present unpredictable clinical course probably related to their histological and morphological characteristics. Twenty-one patients affected by SFT of the pleura were referred to us for surgical resection from September 1984 to April 2000. They were 15 males and six females with median age of 51 (range 15--73) years. Nine patients (43%) were symptomatic and predominant clinical symptoms or signs were dyspnoea (19%), coughing (14.3%), chest pain (28.5%), finger clubbing (14.3%) and hypoglycaemia (14.3%). Hypoglycaemia was related to a pathological incretion of insulin-like growth factor 2 by the tumour. Chest radiograph and computed tomography of the chest revealed intra-thoracic homogeneous sharply delineated round or lobulated mass sometimes associated with ipsilateral pleural effusion (19%) or causing pulmonary atelectasis with opacification of the complete hemithorax (19%). Surgical excision required 14 posterolateral thoracotomies, six anterior thoracotomies and one video-assisted thoracoscopy. Thirteen tumours arose from visceral pleura and wedge resection was performed, seven tumours arose from parietal pleura and extrapleural resection was carried out without any chest-wall resection, one tumour growth within the upper left lobe and required lobectomy. Tumours weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. At cut section seven cases (34%) revealed focal necrosis and hemorrhagic zones and on light microscopy six cases (28.5%) were characterized by high mitotic count: characteristics related with uncertain clinical behaviour. Immuno-histochemical reactions were in all cases positive for CD34. In all our patients resections were complete. Paraneoplastic syndromes like hypoglycaemia and clubbing receded after surgery. No intraoperative or perioperative medical or surgical complications occurred. Median chest-drain duration timed 3 (range 2--5) days and median hospital stay was 5 (range 4--7) days. Perioperative mortality rate was 0%. Median follow-up was 68 (range 2--189) months: during this period patients were submitted to chest X-ray with 6-months interval to evaluate possible local recurrence. Only one patient experienced tumour recurrence after 124 months follow-up: the tumour was suspected after observation of finger clubbing. The tumour was detected and excised by redo-thoracotomy. Surgical resection of benign solitary fibrous tumours is usually curative, but local recurrences can occur years after seemingly adequate surgical treatment. Malignant solitary fibrous tumours generally have a poor prognosis. Clinical follow-up and radiological follow-up are indicated for both benign and malignant solitary fibrous tumours.

Awareness and early detection of cutaneous melanoma: an analysis of factors related to delay in treatment.

Factors associated with the detection of cutaneous melanomas and reasons for delay in diagnosis were investigated in 429 patients with histologically proven melanoma operated on between January 1993 and June 1996. Patients were interviewed using a standardized questionnaire. In 25% of patients, treatment was delayed for more than 1 year from the time they first noticed a suspicious pigmented lesion. Melanoma was detected by the patients themselves in 67% of women and 45% of men. The three predominant clinical symptoms of melanoma were change in colour (darker), increase in size and increase in elevation of a pigmented lesion. The role of sun exposure and of naevi as risk factors for melanoma, as well as the potential benefit of early treatment, were known by 87%, 66% and 82% of the patients, respectively. However, melanoma awareness had no impact on the time period between first observation of skin changes and treatment. Among the factors associated with delay in melanoma diagnosis, an initial incorrect diagnosis as a benign lesion by the physician first visited (in 18% of all cases) had the highest significance. Patients detecting their lesions themselves were treated significantly later than patients in whom others had remarked on changes in a naevus. Furthermore, melanomas of the head and neck were treated later than melanomas at other body sites. Further efforts to educate both the public and the medical profession are essential to ensure earlier treatment for cutaneous melanomas.

Functional morphology of nasal blood vessels in humans.

"Secretion" and "obstruction" as predominant clinical symptoms in rhinology affect a great number of patients with disorders of the nose. According to clinical experience as well as morphological investigations, the endonasal vascular system is most likely to be involved in these functional mechanisms. In the present study, we report morphological findings on the angioarchitecture of human nasal mucosa. Meticulous investigation of the structure and ultrastructure of nasal mucosal capillaries revealed differences in the appearance of the endothelial lining. Especially the morphological feature of attenuated fenestrated endothelia in these vessels might be correlated with the functional behaviour under various physiological and pathological conditions. Inspection of the vascular wall of nasal swell bodies revealed differences in the orientation as well as the shape of muscle cells in different parts of this vascular system. The functional role of special morphological features known as muscular bolsters and intervascular muscle fibres for the swelling mechanism of the nasal mucosa is emphasized. Our results enabled us to define the muscular structures that are most probably responsible for constriction and dilatation of nasal swell bodies.

Increased density of dopamine D2 receptors in the putamen, but not in the caudate nucleus in early Parkinson's disease: a PET study with [ 11C]raclopride

Striatal dopamine D2 receptors were studied, using positron emission tomography (PET), in 10 patients with early Parkinson's disease without any antiparkinsonian medication and in 14 healthy controls. [ 11C]Raclopride was used as ligand and an equilibrium method was applied. The maximum count of receptors ( B max) and their dissociation constant ( K d) were calculated according to the Scatchard principle. In parkinsonian patients, the B max of D2 receptors was increased in the putamen contralateral to the predominant symptoms, as compared to the opposite putamen, by 33% ( p = 0.0008). In the caudate nucleus no significant side to side differences was noted. On comparison with age-matched healthy controls, B max values in the putamen ( p = 0.0012) but not in the caudate nucleus contralateral to the side of predominant clinical symptoms were increased in PD patients. The K d values were unchanged. The difference in putaminal B max values between the opposite hemispheres correlated with the difference in the severity of parkinsonian motor symptoms between the two body sides ( r = 0.69, p = 0.03). The present results show that there is both a relative and absolute increase in the number of dopamine D2 receptors in the putamen, but not in the caudate nucleus in early Parkinson's disease.

Wernicke's encephalopathy in non-alcoholics: An autopsy study

In a 5-year autopsy material constituting 6 964 autopsies, there were 52 cases of Wernicke's encephalopathy of which 12 (23%) occurred in non-alcoholics. Among 18 cases with active (acute) disease, 7 cases (39%) were found in non-alcoholics. Only 4 cases of active Wernicke's disease were diagnosed clinically, all of them in alcoholics. The predominant clinical symptoms were disorientation and depressed levels of consciousness, whereas eye symptoms were recorded in only 3 cases. None of the non-alcoholics were given specific thiamine therapy, whereas some alcoholics received large doses of the vitamin as a routine procedure. However, the thiamine therapy was often instituted too late. It is concluded that active Wernicke's encephalopathy should be considered in all patients with prolonged malnutrition and that disorientation and depressed levels of consciousness may be the predominating symptoms of the disease. Even the slightest suspicion of Wernicke's encephalopathy should prompt immediate administration of large doses of thiamine parenterally.

Natural course of primary biliary cirrhosis. I. A morphological, clinical and serological analysis of 103 cases.

Clinical, biochemical and serological data obtained in 103 patients with primary biliary cirrhosis (PBC) were analysed with respect to the four defined morphological stages. Evaluation of the initial biopsies (99 needle biopsies/4 wedge biopsies) revealed that most patients were in stage I (focal bile duct destruction). Unequivocal distinction between stages I and II was possible in most cases, while considerable overlapping of criteria was observed in stages II to IV. Morphological cholestasis, a characteristic sign of stage IV was already found in 7% of PBC I cases. Four out of 12 autopsy specimens showed micronodular biliary cirrhosis (Hanot's type) and eight specimens had a macronodular type of biliary cirrhosis. No predominant clinical symptoms were found in patients with PBC I or II, but pruritus was observed in about 30%. Increased serum alkaline phosphatase (AP) and IgM levels as well as a positive antimitochondrial antibody (AMA) test were typical features of all stages in up to 80-90%, but patients with normal AP or IgM or negative AMA have been observed, especially in stages I and II. Five of 57 patients at stage I had increased bilirubin levels and in three patients IgM and IgG were simultaneously elevated in stage I. The natural course of PBC, as it is reflected in histological staging, was studied in 30 patients in whom biopsies were regularly taken over a period of 2-18 years. About 80% of PBC I-cases lasted between 1 and 7 years before reaching stage II, while another 5-10 years were necessary for the development of stage III-IV. Thus it appears that in the vast majority of patients PBC lasts about 10-15 years and in some instances even more than 20 years. The finding in stage I and II of normal AP indicates a benign course, while morphological and biochemical cholestasis seems to be associated with a rather progressive course.

Choc anaphylactique au suxaméthonium A propos de 18 cas

The authors have studied 18 cases of anaphylactic shock due to suxamethonium which occurred in eastern France during the period 1972–1980. The drug responsible was succinyl iodide in 15 cases and succinyl chloride in 3. Significant contributing factors were previous drug allergy, spasmophilia and skin hypersensitivity to histamine. Five subjects had never been previously anaesthetized. The predominant clinical symptoms were cardiovascular collapse (72 p. cent), cutaneous and mucous symptoms (72 p. cent) and bronchospasm (33 p. cent). Cardiac arrest was observed in 5 patients; it was postanoxic in 3 cases and primary in 2. The diagnosis of anaphylaxis was based on the simultaneous positivity of at least two of the specific immunoallergic tests : intradermal reaction to a 10 −5 to 10 −3 dilution of the drug; Prausnitz-Küstner test with Parish's variant and human basophil degranulation test. If only one test was positive, this had to be confirmed on two or even three occasions. Thus, 6 patients were tested once, 9 patients were tested twice and 3 patients were tested 3 times. In case of genuine anaphylaxis, suxamethonium must definitely be avoided, and the subject should be provided with an allergy card.

Gingivo‐periodontal manifestations in chronic benign neutropenia

A case of chronic benign neutropenia in a 7-year-old Caucasian male is reported. The predominant clinical symptoms were persistent gingivitis and periodontitis. The clinical, hematological, oral radiographical, histological, and therapeutical features of this uncommon disease are discussed.

An outbreak of ornithosis.

An outbreak of ornithosis occurred in Lidköping, a small town in Southwest Sweden, in January 1967 through May 1969. 24 proved cases were known, aged from 10 to 72 years, 19 patients gave a history of contact with birds (parrots, canaries, parakeets), 13 of them with birds from the same pet shop. Five patients had visited the pet shop only once before their illness. Attempts to prove infection with ornithosis agent in 7 birds from the pet shop were unsuccessful. Three patients, 2 of them in the same household, had no contact with birds and infection from person to person was strongly suspected. All patients had pneumonia. Fever, cough and headache were predominant clinical symptoms. There were relapses in 4 patients and 2 patients had symptoms of encephalitis. No deaths occurred.

Crohn's disease of the upper gastrointestinal tract.

Ten patients with Crohn’s disease of the upper gastrointestinal tract were studied. All patients had either gastric or duodenal involvement, or both, and all had typical Crohn’s disease elsewhere. In one patient, an esophageal lesion of Crohn’s type was present. Upper abdominal pain and nausea and vomiting were the predominant clinical symptoms; signs of gastric outlet obstruction signalled the onset of gastroduodenal involvement. In four patients not previously known to have Crohn’s disease, gastric outlet obstruction was the initial sign of the disease, but all four proved to have concurrent disease of the ileum or colon. The histologic changes in the upper gastrointestinal lesions were similar to those in the ileum and included transmural chronic inflammation, granulomas, and marked submucosal fibrosis. Other disease processes known to produce similar changes were carefully excluded.

Pulmonic manifestations of systemic lupus erythematosus (SLE): Report of five cases

1. 1. The pulmonic manifestations of systemic lupus erythematosis (SLE) can be the predominant clinical symptoms at any stage of the disease. 2. 2. The pulmonary findings can be bizarre and can simulate a variety of respiratory lesions. Unexplained, chronic, pulmonary involvement should make the clinician suspect that systemic lupus erythematosus is the basic disease. 3. 3. The most beneficial treatment of the pulmonary parenchymal lesion of SLE appears to be steroid therapy. Antibiotics used solely in two cases failed to alleviate the pulmonary pathologic process, but both patients improved after steroid treatment had been instituted. 4. 4. The significance of fungi in the pulmonary parenchymal tissue at necropsy is not understood at present, because they are found under widely differing circumstances. Fungi were found in the pulmonary parenchymal tissue of one patient who died before steroids were available for therapy and in that of another patient who had received both steroids and antibiotics over a relatively long period of time.