Maladie de Lyme avec hépatite et corticothérapie : à propos d’une observation

Introduction

Abnormalities of liver function tests have been occasionally described in large series of Lyme disease, but only one case of hepatitis directly related to infection have been described in literature.

Case report

A 78-year-old-man, with a past medical history of polymyalgia rheumatica (PMR) who had discontinued corticosteroids two years before, presented a transient acute fever and liver cholestasis and cytolysis after an exposure to tick bites. A few days later, cervical pain occurred and corticosteroids were resumed as a PMR relapse was suspected. Hematogenous dissemination with acute meningoradiculitis and multiple erythema migrans led to conclude to a stage 2 Lyme disease.

Conclusion

Although hepatitis complicating the course of Lyme disease has been described in literature, the marked inflammation in our patient led us to investigate the possibility of a co-infection. Also, we discuss the responsibility of corticosteroids in clinical worsening of Lyme disease if they are prescribed without concomitant antibiotics.     

Lymphomes spléniques de la zone marginale et auto-immunité : à propos de six cas

Introduction

Autoimmune manifestations are common in splenic marginal zone lymphoma (SMZL) and are sometimes the presenting feature of the disease. Autoimmune cytopenia (anemia, thrombocytopenia) are the most frequently reported autoimmune conditions. However, other immunological manifestations may be associated with SMZL.

Méthodes

We report a retrospective case series of six patients with SMZL associated with autoimmunity.

Results

Auto-immune manifestations were the presenting feature of lymphoma in four cases. Auto-immune manifestations included auto-immune cytopenia in three cases (two hemolytic anemia and one pancytopenia), thyroiditis in two cases, systemic lupus and Still's disease in one case each. Antinuclear antibodies were detected with a titre of 1/250 in three cases, and with a titre of 1/32,000 in the patient with systemic lupus. Testing for DNA antibodies was negative in all cases. Two patients had a circulating lupus anticoagulant, with portal venous thrombosis following splenectomy in one case. One patient had hypogammaglobulinemia. A monoclonal gammopathy was detected in three patients. All patients had spleen enlargement. Immunophenotyping of blood peripheral lymphocyte was typical in five out of the six cases. Bone marrow was infiltrated in five out of the six cases. Diagnosis was obtained by the combination of immunophenotyping and bone marrow histopathology in five cases, and by splenic histopathology in the remaining case. Hepatitis C virus serology was negative in all patient.

Conclusion

Autoimmune disease as systemic lupus or Still's disease may be associated with SMZL before its tumoral manifestations are evident. In this mode of presentation, spleen enlargement, hypogammaglobulinemia, monoclonal gammopathy, and multiple autoimmune diseases, should alert the physician.     

La syphilis au CHU de Nantes entre 2000 et 2010 : à propos de 36 patients hospitalisés

Purpose

Syphilis infection increase has been observed since the early 2000s. The medical records of patients hospitalized for syphilis at the tertiary care hospital of Nantes between 2000 and 2010 were retrospectively reviewed.

Methods

Cases were selected on the basis of serological database of the laboratory of bacteriology and extraction from the PMIS. Syphilis cases were defined by both positive TPHA and VDRL tests.

Results

The number of positive serology testing was increased eightfold within ten years. Among the 36 patients with syphilis hospitalized cases, 97% were male, aged 17 to 75 years. Eighteen were HIV-infected patients. Among them, 94% were homosexuals and 67% had a history of sexually transmitted infections. The mean time between symptoms and diagnosis was significantly higher in non HIV-infected patients. Clinical forms of syphilis were cutaneomucous secondary syphilis with frequent systemic symptoms for 33%, neurosyphilis, including frequent uveitis for 50%, and gummatous tertiary syphilis involving bones for one patient. Secondary syphilis cases were treated with one to three doses of benzathine penicillin G. Late syphilis and cases of neurosyphilis were treated with penicillin G or ceftriaxone. Neurosensory sequelae accounted for 39% neurosyphilis cases.

Conclusion

This study highlights the incidence increase of syphilis cases in France, frequent poor prognosis of neurosyphilis cases, and diagnosis difficulties, particularly in non HIV-infected patients. This emphasizes the broader use of syphilis serology for compatible medical situations.     

Complications de la gastroentérite à éosinophiles : une observation et revue de la littérature

Introduction

Eosinophilic gastroenteritis is an unusual disease characterized by an eosinophilic infiltration of the gastrointestinal tract. The esophageal location of this disorder is uncommon and is usually revealed by dysphagia. Diagnosis is obtained by histology during endoscopy after exclusion of differential diagnosis. Treatment is based on systemic corticosteroids, which improve dramatically symptoms and endoscopic lesions.

Case report

We report an 88-year-old man who presented eosinophilic gastroenteritis with esophageal injury complicated by gastrointestinal haemorrhage and fistule.

Conclusion

Eosinophilic gastroenteritis may have a potentially unfavourable outcome. The treatment of complicated forms is not codified and often empirical.     

Maladie de Niemann-Pick de type B : description clinique de trois cas familiaux

Introduction

The Niemann Pick disease type B is a rare deficiency in sphingomyelinase activity, autosomal recessively inherited.

Case reports

We report three patients (two men, one woman) of the same family, who showed pulmonary and hepatosplenic lesions, usually present in the disease but also adrenal gland lesions confirmed by tomodensitometry.

Conclusion

The current treatment of Niemann Pick disease is purely symptomatic awaiting the use of enzymatic replacement therapy which has been successfully experimented in animal model.     

Un cas de syndrome de Hoigné lors d’une injection intraveineuse de ceftriaxone

Introduction

Hoigne's syndrome is characterized by the development of acute clinical manifestations which are mainly psycho-sensorial. Classically, these features immediately follow the injection of procaine penicillin G.

Case report

We report a 59-year-old man who presented with psycho-organic manifestations that occurred just after the intravenous injection of ceftriaxone; to our knowledge, this is the first case of Hoigne's syndrome reported after an injection of this antibiotic.

Conclusion

The pathophysiologic basis of this syndrome is still unknown. It is important to keep in mind its clinical characteristics, which may mimic immuno-allergic symptoms. It should be differentiated from anaphylactic manifestations because Hoigne's syndrome allows the continuation of the treatment.     

Efficacité clinique et biologique du tocilizumab au cours de la maladie de Horton : à propos de trois observations et revue de la littérature

Introduction

Treatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly.

Case reports

We report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing.

Conclusion

Tocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy.     

Méningite à éosinophiles

Introduction

Diagnosis of Angiostrongylus cantonensis meningitis is not always obvious, even in endemic areas.

Case report

We report a 51-year-old Polynesian man, farmer without past medical history, who was admitted for mild fever, headache, neurological subjective symptoms, and severe muscle pain impeding walking. The diagnosis of A. cantonensis meningitis, guided by hypereosinophilia was confirmed by lumbar puncture and positive specific serology. Lumbar puncture and administration of corticosteroids rapidly reduced headache whereas paraesthesias declined more progressively.

Conclusion

Diagnosis of A. cantonensis meningitis should be considered in patients living or returning from endemic areas with headache associated with subjective neurological symptoms and hypereosinophilia.     

Manifestations cliniques et biologiques de la primo-infection à parvovirus B19 chez l’adulte immunocompétent : étude rétrospective de 26 observations

Purpose

Parvovirus B19 causes erythema infectiosum in children, transient aplastic anemia in patients with hemoglobinopathies, pur red cell aplasia in immunocompromised persons and hydrops fetalis in pregnancy. The spectrum of clinical and biological manifestations in immunocompetent adult continues to grow up.

Methods

We report on a case series of 26 patients with primary parvovirus B19 infection in immunocompetent adults. This is a retrospective study over the period 2000 to 2010 in two departments of internal medecine. The diagnostic was clinical, serological or molecular.

Results

There was a female predominance (sex-ratio 3.33/1). Median patient age at diagnostic was 38.8 years (range: 18–68). The predominant symptoms were fever (65%), peripheral and symmetrical polyarthralgia (62%) and skin rash (58%). Two patients had neurological manifestations (sixth cranial nerve palsy, distal paresthesia) and one patient had myocarditis. Abnormal laboratory values included increased acute phase reactants (73%), thrombocytopenia (43%), lymphopenia (38%) and elevated liver enzymes (37%). Antinuclear (19%), anti-DNA (28%) and anti-phospholipids antibodies (14%), and hypocomplementemia (32%) were observed. False reaction with anti-CMV and anti-EBV IgM positivity was documented in 27% of cases. Two patients had persistent parvovirus B19 infection.

Conclusion

The diversity of the clinical manifestations of parvovirus B19 infection may be misleading for the clinician. However, the diagnosis should be suspected in immunocompetent adults to limit the risk of transmission to the patients who could develop a severe infection such as pregnant women or immunocompromised patients.     

Infection disséminée au virus varicelle-zona avec gastrite hémorragique compliquant une leucémie lymphoïde chronique : une observation et revue de la littérature

Introduction

The reactivation of varicella-zoster virus occurs in immunocompromised patients, especially in cases of hematological malignancy. Disseminated reactivation could involve digestive tract with life-threatening condition.

Case report

A 76-year-old woman, with a history of chronic lymphocytic leukemia, presented with left hypochondrium pain, and a vesicular rash with hemorrhagic shock that revealed an hemorrhagic gastritis due to varicella-zoster virus. The literature review identified 28 additional cases of gastrointestinal mucosal damage during reactivation of varicella-zoster virus. Mortality is 40%. We report here the first case in the course of low-grade lymphoid malignancy.

Conclusion

Acute gastrointestinal symptoms in immunocompromised patients should evoke a varicella-zoster virus reactivation with gastrointestinal involvement. This clinical manifestation, although rare, should not be ignored because of its severity.     

Atteintes ORL de la maladie de Fabry : à propos de 25 observations

Purpose

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disorder due to deficient activity of the enzyme alpha-galactosidase A. Males and females exhibit severe organ involvement. The high incidence of otological symptoms was recently reported.

Patients and methods

Monocentric and retrospective study of twenty-five patients with FD (13 families; seven males and 18 females). The patients underwent audiological assessment before initiation and during enzyme replacement therapy. We also analysed neurologic heart and kidney status.

Results

Twenty patients (80%; 13 females and seven males) complained of otologic symptoms. Audiological evaluation showed a sensorineural hearing loss in 17 patients, bilateral in 16 out of them. Vestibular examination showed a functional impairment in two patients (one female, one male). Correlations were found between hearing loss and either kidney disease (73,3%), neurological complications (100%) and cardiomyopathy (80%). Fourteen patients (56%; seven females, seven males) received enzyme replacement therapy. Improvement or stabilization of the audiological evaluation was reported in seven patients, whereas worsening was observed in three patients.

Conclusion

This study confirms the high frequency of audiological involvements in females and males with FD. Our analysis suggests that the frequency of hearing loss is increased in the presence of renal or neurologic involvement or cardiomyopathy. There is no clinically significant efficacy of enzyme replacement therapy on hearing function. Although the pathophysiology remains unknown, a vascular mechanism responsible of the inner ear involvement seems to be privileged.     

Syndrome d’encéphalopathie postérieure réversible induit par un décongestionnant nasal contenant de la pseudo-éphédrine

Introduction

Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome.

Case report

We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug.

Conclusion

The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders.     

Complications infectieuses induites par le mésusage de la buprénorphine haut dosage (Subutex) : analyse rétrospective de 42 observations

Purpose

Misuse of high-dose buprenorphine (HDB), mainly by injection, is responsible of frequent infectious adverse events.

Methods

This is a retrospective study of infectious complications occurring in patients using HDB by injection. Forty-two cases were identified (29 men and ten women) and the data were collected between March 1999 and December 2008.

Results

The infectious complications included cutaneous infections (27 cases), endocarditis (nine cases), osteoarticular infections (four spondylodiscitis and one sacroiliitis), and a vascular embolism with decrease in visual acuity.

Conclusion

The results of HDB maintenance treatment must be improved, both from the point of view of substitution and to limit its misuse by intravenous route injection. Health professionals have to play an important role in drug addict patients’ education and supervision, to prevent buprenorphine injection and related infectious complications.     

Thrombophlébite cérébrale : une complication rare de l’infection aiguë à cytomégalovirus

Introduction

Acute cytomegalovirus (CMV) infection increases the risk of vascular thrombosis but reports of cerebral venous thrombosis are rare.

Case report

We report a 36-year-old woman who presented with a cerebral venous thrombosis and acute CMV infection heralded by a cytolytic hepatitis. Heterozygous factor V Leiden mutation was also identified. The patient was treated with anticoagulation for 1 year with favourable outcome.

Conclusion

Serologic tests for CMV infection should be performed in case of cerebral venous thrombosis with liver cytolysis or flu-like symptoms. CMV infection often triggers thrombosis in combination with other inherited or genetic predisposing risk factors that should always be searched.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

Lichen plan buccal érosif associé à un syndrome de Good

Introduction

Good syndrome is characterized by thymoma, hypogammaglobulinemia, low number of peripheral B cells, and variably, peripheral CD4 T cell lymphopenia and inverted CD4/CD8 T cell ratio, associated with infections and autoimmune diseases.

Case report

We report an 85-year-old woman who presented with oral erosive lichen planus and thymic tumor. Improvement of oral erosive lichen planus was observed after resection of a benign thymoma, but was ineffective on the immunodeficiency syndrome. Only 11 patients with Good syndrome and lichen planus have been previously reported and are reviewed.

Conclusion

The association of oral erosive lichen planus with Good syndrome is rare, but does not seem to be fortuitous.     

Maladie de Kikuchi-Fujimoto : une présentation atypique

Introduction

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) is a rare clinical entity characterized by the association of enlarged lymph nodes in the posterior cervical region and fever. The disease is more frequent in young women.

Case report

We report a 41-year-old African patient who presented with atypical features of Kikuchi's disease including cutaneous lupus, haemophagocytosis, and lymphocytic meningitis. The ethnic origin and the clinical presentation were initially suggestive of tuberculous meningitis. However, microbiological analyses remained negative, histological findings were suggestive of Kikuchi's disease and HHV6 DNA integration was documented in our patient.

Conclusion

Kikuchi's disease should be suspected in an African patient when lymphocytic meningitis is associated with enlarged cervical lymph nodes, hemophagocytosis and HHV6 DNA integration.     

Encéphalopathie de Gayet-Wernicke après sleeve gastrectomie pour obésité morbide

Introduction

Bariatric restrictive interventions, as sleeve gastrectomy or gastric banding can cause metabolic complications, especially when vomiting is present, such as thiamine deficiency that can lead to Wernicke's encephalopathy.

Case report

A 31-year-old man with a 47 kg/m² body mass index presented with Wernicke's encephalopathy, with ophtalmoplegia, nystagmus, ataxia and confusion, followed by a Korsakoff syndrome, occurring two months after a sleeve gastrectomy. MRI showed hyperintense signals on T2 and FLAIR image in both thalamus, periaqueducal area and mamillary bodies.

Conclusion

A close clinical and biological monitoring is required in the first year after surgery, especially if vomiting occurs. Early diagnostic and treatment are needed to avoid severe sequelae.     

Myélopathie à Schistosoma mansoni

Introduction

Neurological complications of schistosomiasis remain exceptional even in hyperendemic area.

Case report

We report a 26-year-old Senegalese man, without past medical history, who was admitted for spastic paraplegia, acute retention of urine, and pain in low back and lower limbs. The final diagnosis was spinal cord schistosomiasis. Diagnosis was based on the endemic context, MRI medullar conus imaging, schistosoma serology in cerebrospinal fluid and blood, and the absence of other cause of myelopathy. Treatment was based on praziquantel, corticosteroids and physiotherapy. The outcome was favorable after a 2-year follow-up.

Conclusion

Schistosomiasis should be included in the differential diagnosis of myelopathy in patients living actually, or even traveled in the past, in endemic tropical areas.