Testing basic visual functions in the evaluation of extrapyramidal side effects of antipsychotic agents

A female patient presenting with B-CLL and coincident eosinophilia-myalgia syndrome (EMS) after ingestion of L-tryptophan is described. The manifestations of EMS disappeared completely during treatment with cyclophosphamide/prednisone. and there was an intermittent clinical remission of CLL with absence of the monoclonal B-cell population. A few years later, the B-CLL relapsed, but without sign and symptoms of EMS. Whereas other eosinophilic syndromes such as eosinophilic fasciitis, panniculitis, or cellulitis Wells have been found to occur in relation to malignant underlying diseases, only a single patient with malignant fibrous histiocytoma following EMS has been described. There are no reports about an increased occurrence of B-CLL or other non-Hodgkin's lymphomas combined with or following EMS or related to L-tryptophan itself. The variant types of eosinophilic syndromes occurring due to malignant disorders, the differentiation from EMS, and the possible association between B-CLL and L-tryptophan-related EMS are discussed.     

5-Fluorouracil dose intensification and granulocyte-macrophage colony-stimulating factor in cisplatin-based chemotherapy for relapsed squamous cell carcinoma of the head and neck: a phase II study

We reviewed the clinical record of all patients admitted to Saga Medical School Hospital during the most recent 10 years and found that 17 (0.03%) were diagnosed as having necrotizing fasciitis. Bacteriological examination demonstrated that Vibrio vulnificus was the pathogen responsible in five patients (29%). The disease caused by V. vulnificus occurred in the warmer half of the year. All of the patients had underlying chronic liver dysfunction, and three of them had previously consumed raw seafood. In these patients, the predominant skin lesions were oedema and subcutaneous bleeding, such as ecchymosis and purpura, while superficial necrosis was not recognized. Three patients died of systemic complications. By contrast, all of the five patients with necrotizing fasciitis caused by Streptococcus pyogenes had the disorder in winter, and only one of them had chronic liver dysfunction. In skin lesions, subcutaneous bleeding was rare but necrosis was seen often. Despite the high incidence of systemic complications, no patients with streptococcal necrotizing fasciitis died. These findings suggest that the clinical features of necrotizing fasciitis caused by V. vulnificus are different from those of necrotizing fasciitis caused by classical pathogens, and that the two should be differentiated as early as possible to improve the prognosis.     

Response to methotrexate in a patient with idiopathic eosinophilic fasciitis, morphea, IgM hypergammaglobulinemia, and renal involvement

A 35-year-old man with idiopathic eosinophilic fasciitis (EF) and morphea developed renal disease characterized by microscopic hematuria, nephrotic range proteinuria, and rapidly progressing hypertension, an association that has not previously been reported in EF. Initial clinical symptoms of EF began in July 1989; peripheral eosinophilia peaked at 30% in August 1990; an abnormal urinalysis was first observed in March 1992 and subsequently a renal biopsy was performed. Renal biopsy demonstrated focal segmental glomerulosclerosis and a subepithelial immune-type deposit. Partial fasciectomy and a course of methotrexate resulted in overall functional improvement of his extremities. Proteinuria and hematuria was reduced during methotrexate therapy.     

Spectrum of hypereosinophilia syndrome based on 2 clinical case reports

BACKGROUND: Two severe cases of hypereosinophilic syndrome with cardiopulmonary symptoms, erythema nodosum, eosinophilic ileocolitis and paresthesia are reported. Definition, etiology, pathophysiological mechanisms, types, complications and therapeutic approaches of this relatively rare clinical disorder are discussed on the basis of these two cases. The distinction between eosinophilia and hypereosinophilic syndrome is clearly defined. DIFFERENTIAL DIAGNOSIS: The 2 cases of hypereosinophilic syndrome demonstrate the difficulty to decide whether they represent the idiopathic or the reactive type of hypereosinophilic syndrome. If the assumption is made in favor of the reactive type of hypereosinophilic syndrome the assumed prognosis could eventually be too promising. In case of an assumed idiopathic type of hypereosinophilic syndrome the search for causing factors like allergens could be left out. The decision concerning the type of hypereosinophilic syndrome of the 2 cases reported was made with high probability. In the case of idiopathic hypereosinophilic syndrome, eosinophilic leukemia was excluded by bone marrow biopsy. CONCLUSION: In order to be able to make a diagnosis it is necessary to search carefully for causing and influencing factors of the hypereosinophilic syndrome such as allergens or medicaments and to eliminate them. The hypereosinophilic syndrome must be put under frequent control in order to avoid complications or to start with therapeutic approaches in time.     

Idiopathic hypereosinophilic syndrome with eosinophilic myositis, peripheral neuropathy and central nervous system involvement

Idiopathic hypereosinophilic syndrome (HES) is a rare disorder marked by a sustained overproduction of eosinophils and a predilection for damage to multiple organ systems. Its neurologic involvement ranges from the central to the peripheral nervous system, and can be associated with eosinophilic myositis. We report a 68-year-old woman who had eosinophilia, eosinophilic dermatitis and eosinophilic pneumonia. She also suffered from numbness and weakness of the lower limbs. Because of long-lasting (> 6 mo) eosinophilia (> 1.5 x 10(9)/L) in the peripheral blood and the fact that no other underlying causes of eosinophilia and neurologic involvement could be identified, a diagnosis of idiopathic hypereosinophilic syndrome was made. The muscle biopsy showed infiltration of inflammatory cells, including a few eosinophils (Liu's stain). Magnetic resonance images, motor evoked potentials, somatosensory evoked potentials and nerve conduction velocities also showed abnormalities in the central and peripheral nervous systems. The pathogenesis and treatments of HES are discussed in this report.     

Clinical correlates of granulomas in muscle

We evaluated the clinical and myopathological features of all patients with granulomas in muscle biopsy specimens identified over a 5-year period (1992-1996) at the Washington University Medical Center. Ten patients were found to have granulomas in their muscle biopsy specimens. Of these, eight patients had myopathic changes. Seven had dysphagia as a major functional difficulty during the course of their disease. None had elevated levels of serum creatine kinase (CK). Four of the patients with myopathy had systemic sarcoidosis and relatively severe proximal weakness with functional disability. Treatment with corticosteroids was followed by marked improvement in strength and functional disability. The four other patients with myopathy had no systemic signs of sarcoidosis. Weakness was especially prominent distally in three of these patients. The two patients in this group treated with corticosteroids did not improve. The final two patients, who had granulomas in muscle but no myopathic changes, had clinical syndromes of mononeuritis multiplex and eosinophilic fasciitis (Shulman syndrome). We conclude that granulomatous myopathy, in the presence or absence of systemic sarcoidosis, is commonly associated with dysphagia (87%) and a normal serum CK. Clinical features in patients with sarcoidosis included severe proximal weakness with functional disability that often responded to corticosteroid treatment. Granulomatous myopathy without systemic sarcoidosis was associated with milder, but more predominantly distal weakness.     

Chromosome studies in "preleukemia". III. Myelofibrosis

Cytogenetic studies were done on 18 patients with myelofibrosis or the closely related syndrome, undifferentiated myeloproliferative disorder (MPD). Clones of cells with chromosome abnormalities were demonstrated in the blood of eight individuals, including two with a history of radiation therapy and two with "acute myelofibrosis". Trisomy 8 was present in the latter two patients, but otherwise, there was no consistent cytogenetic pattern or correlation with specific hematologic findings. Sixteen of these patients have been followed for more than 1 year or until death; none has progressed to leukemia. The results indicate that chromosome abnormalities are relatively common in this disorder, but as with polycythemia vera, and unlike some other "preleukemic" states, the aberrant clones in myelofibrosis do not appear to indicate that clinical leukemia is imminent.     

Further evidence that the Hajdu-Cheney syndrome and the "serpentine fibula-polycystic kidney syndrome" are a single entity

The Hajdu-Cheney syndrome (HCS) is a rare autosomal dominant disorder. It comprises a coarse face, short neck, hirsutism, joint laxity, and normal intelligence. Bone dysplasias, include acro-osteolysis, bathrocephaly, and vertebral anomalies. In 1988, Exner [1988: Eur J Pediatr 147:544-546] coined the term "serpentine fibula-polycystic kidney syndrome" (SFPKS) when he reported on a girl with short stature, unusual facial appearance, polycystic kidneys, and elongated curved fibulae. He postulated that it was a new entity different from the Melnick-Needles syndrome. Since his report, five similar cases have been published. Similarities between both HCS and SFPKS were noticed first by us and then by other authors. In this report we show that many clinical and radiological characteristics are shared by the HCS and the SFPKS and hypothesize that they represent a single entity with a variable degree of expression.     

Advised follow-up after emergency treatment of adolescents with violence-related injuries

INTRODUCTION: The finding of eosinophilic ascites is unusual. It requires the search for the main etiology, i.e., parasitic or malignant disease, vasculitis or hypereosinophilic syndrome. The diagnosis of exclusion is either mucosal, muscular or serous eosinophilic gastroenteritis. This last type, the most unusual--as less than 50 cases have been documented until now--is associated with eosinophilic ascites. EXEGESIS: We report a new case of serous eosinophilic gastroenteritis that occurred in a 23-year-old woman. This case was unusual because of its clinical history, as abdominal pain fits (along with the occurrence of ascites) were associated with urticaria fits. The lack of eosinophils in both the blood counts and the various digestive biopsies were unusual too. The disease evolution was favorable with corticosteroid therapy; however, a minimal dose of 8 mg/day was necessary to control the disease symptoms. CONCLUSION: Because of its association with urticaria fits, this case emphasizes the need for differential diagnosis in patients with hypereosinophilic syndrome and food allergy.     

Eosinophilic cholecystitis, appendiceal inflammation, pericarditis, and cephalosporin-associated eosinophilia

A patient with eosinophilic cholecystitis and accompanying eosinophilic appendiceal inflammation, eosinophilic pericarditis, and peripheral eosinophilia is described. Review of the nine previously reported cases of eosinophilic cholecystitis suggests that this is the first case with closely associated eosinophilic appendiceal inflammation and pericarditis as manifestations of a systemic hypereosinophilic syndrome. The possible etiologic role of cephalosporin hypersensitivity is discussed.     

Children with Asperger syndrome

In 1994 Hans Asperger (1906-80), an Austrian physician, described a group of children with impaired social interaction and communication abilities. The name of this disorder today is Asperger's syndrome, and it is currently defined under the category of pervasive developmental disorder in DSM-IV and ICD-10. In this article the following aspects of Asperger's syndrome are focused on: personality, epidemiology, etiology, examination, differential diagnosis, management and prognosis. The article is based on a literature study. Asperger's syndrome seems to be considerably more common than "classic" autism. The syndrome is much more common in boys than in girls. The clinical characteristics of Asperger's syndrome are probably influenced by many factors, including organic and genetic factors. Asperger's syndrome is the term applied to the highest functioning end of the autism scale. There are several commonalities between Asperger's syndrome and autism, namely impairment of social interaction and communication abilities, and range of interests and activities. Differences exist primarily in the degree of impairment in language and cognitive development. Differential diagnosis, examination and management are discussed. There is a need for further research. It is important that the diagnostic criteria for Asperger's syndrome are as uniform as possible, and that they do not overlap with infantile autism.     

Inhibition of interleukin-5 with a monoclonal antibody attenuates allergic inflammation

IL-5 is a prominent and perhaps an essential element in the induction of allergic inflammation in human asthma and other allergic diseases. Despite the strong biochemical and clinical correlates between lung eosinophilia and asthma, there is no clear understanding of how eosinophils exacerbate asthma. Antigen administration to sensitized animals produces eosinophilic infiltration that is very similar to that in man, and is prevented by administration of a neutralizing monoclonal antibody against IL-5. Mice in which the IL-5 gene is absent are unable to mount eosinophilic responses to antigen and do not sustain lung damage, but otherwise develop normally. The study of the biology of IL-5 has not only clarified the links between eosinophilia and airway hyperreactivity, but also strongly suggests that anti-IL-5 therapy may be an effective, safe, and novel way of treating human asthma and perhaps other eosinophilic diseases. There are many different potential approaches to the inhibition of IL-5, but the one most likely to provide "proof of principle" in "asthma in the wild" in man is a monoclonal antibody against IL-5.     

Impact of imported beverages on fluoridated and nonfluoridated communities

In the last 10 years an increasing number of cases of group A streptococcal toxic shock syndrome have appeared in various clinical settings. The manifestation of this syndrome includes rapidly progressive multiorgan failure and soft-tissue necrosis. This report presents a case of streptococcal toxic shock syndrome caused by Streptococcus pyogenes with severe necrotizing fasciitis of the abdominal wall following hysterectomy. Aggressive surgical intervention with debridement of all necrotic tissue necessitated resection of the complete abdominal wall (skin, subcutaneous tissue, muscle and peritoneum). The abdominal wall defect was covered with free myocutaneous flaps and split-skin grafts. Optimal treatment, including adequate antibiotic therapy and radical surgical intervention, is an indispensable prerequisite of successful outcome.     

KARGA PUJA: A TRANSPERSONAL RITUAL OF HEALING IN TAMANG SHAMANISM

The karga healing ritual of the Tamang shamans is a dramatic event and a successful means of treating the major indigenous categories of mental illness, that is, soul loss and spirit possession, when they occur simultaneously in a patient. This clinical pathology is a cross-cultural variation of the categories of dissociative trance disorder and somatoform disorder described in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders of the American Psychiatric Association. Tamang shamans treat this relatively neurotic syndrome by evoking transpersonal experiences that are psychotherapeutic in that they alter the patient's relationships both in regard to the spiritual cosmos and to significant interpersonal relationships. Karga rituals evoke experiences analogous to "spiritual emergencies" and "rites of passage" with a death-and-rebirth scenario leading to psychological, social, familial, and spiritual renewal. Discussion is illustrated by a case vignette.     

Overinterpretation of gastroduodenal motility studies: two cases involving Munchausen syndrome by proxy

Two children were thought to have an atypical gastroduodenal motility disorder because of the history and clinical course; both had received parenteral alimentation because of claims of inability to tolerate enteral feedings, and both continued to have unusual medical problems during parenteral alimentation. Both children had motility studies that were interpreted by a pediatric gastroenterologist to be "abnormal" and "diagnostic" of a motility disorder, but each was eventually shown to have a behavioral abnormality related to Munchausen syndrome by proxy.     

Covering the "open abdomen": a better technique

"Damage control" in severe abdominal trauma, abdominal compartment syndrome, necrotizing fasciitis of the abdominal wall, and necrotizing pancreatitis often preclude closure of the fascia after laparotomy. Many techniques have been reported for temporary coverage of the exposed viscera, but most have had documented problems. We report the successful use, since 1989, of a temporary sutureless coverage. The viscera are covered with omentum when possible, then with a clear plastic sheet. Sump drains are placed over this layer. The entire abdomen is then covered with two layers of iodophor-impregnated adhesive plastic drape. The last 50 patients managed with this technique are reported. The most common indication (27 patients) was for treatment of severe abdominal trauma. There were no wound infections, fasciitis, or bowel obstruction. Eighteen patients died; no deaths were related to abdominal closure. Temporary abdominal covering with adhesive plastic sheeting is a rapid, safe, and readily available method for managing the open abdomen. This technique provides a physiologic milieu for the abdominal viscera, simplifies nursing care, and promotes safe closure of the abdomen at a later time.     

Giant antral ulcer: a rare presentation of eosinophilic gastroenteritis--case report and review of the literature

A 24-yr-old female presented with a giant gastric ulcer and anemia. She suffered from a transient infantile malabsorption syndrome with eosinophilia. The diagnosis of eosinophilic gastroenteritis associated with the gastric ulcer was made by endoscopic biopsy. Ulcer healing was refractory to medical therapy and partial gastrectomy was performed. Histologic examination revealed transmural eosinophilic infiltrates with mast cell infiltrates in the gastric wall. This case illustrates (1) an extremely rare presentation of eosinophilic gastroenteritis--giant, refractory, gastric ulcer; (2) a potential pathogenic role for mast cells in this syndrome; and (3) the chronic and relapsing nature of the syndrome.     

Manic-depressive disease

Manic-depressive illness (MDI) is a periodic major affective disorder defined by successive depressive and manic episodes, separated by free intercritic periods. Unipolar manic-depressive illness is defined by successive depressive episodes, whereas bipolar manic-depressive illness is defined by successive depressive and manic episodes. Clinical, familial and biological studies have demonstrated the heterogeneity of unipolar depression and its relationship with bipolar depression leading to questions about common etiopathogeny of those two disorders. Manic-depressive heterogeneity led to the identification of several subgroups defining "manic-depressive spectrum". The reunion of these different clinical entities is based on phenomenological, clinical and familial arguments. MDI is an endogenous pathology, as vulnerability to this disorder is mostly determined by genetic and/or biological factors. Treatment consist first on treatment of major episodes, based on curative and consolidation treatment and secondly on prophylactic treatment.     

In-vitro evaluation of nitrofurantoin as an alternative agent for metronidazole in combination antimicrobial therapy against Helicobacter pylori

We report the case of a patient aged 49 who presented with a picture of an acute respiratory distress syndrome developing over several days after the preventative treatment of a burn using chloroquine (Nivaquine). The combination of clinical data, radiological and clinical as well as the broncho-alveolar lavage, enabled a diagnosis of acute eosinophilic pneumonia to be made according to Allen's criteria. The outcome after stopping the cloroquine and treating with corticosteroids was rapidly favourable and without any relapse. Based on this observation, we recall the characteristics of acute eosinophilic pneumonia as well as the causes which are currently established. Their potentially serious nature should encourage people to report similar cases to ours because even if we have not made identical observations to other cases. Chloroquine seems to us responsible for the clinical picture presented by our patient.