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Movement Disorders : Official Journal... Nov 2018Whipple's disease, affecting the CNS, can cause a wide variety of symptoms. Movement disorders are very prevalent, and some are pathognomonic of the disease. This... (Review)
Review
Whipple's disease, affecting the CNS, can cause a wide variety of symptoms. Movement disorders are very prevalent, and some are pathognomonic of the disease. This systematic review analyzed all published cases of movement disorders because of CNS Whipple's disease, providing detailed information on clinical and associated features. We have also attempted to address sources of confusion in the literature, particularly related to differing uses of the terminology of movement disorder. This comprehensive overview of Whipple's disease-induced movement disorders aims to aid neurologists in recognizing this very rare disorder and successfully reaching a laboratory-confirmed diagnosis in order to initiate appropriate therapy. © 2018 International Parkinson and Movement Disorder Society.
Topics: Databases, Bibliographic; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Movement Disorders; Ocular Motility Disorders; Whipple Disease
PubMed: 30338868
DOI: 10.1002/mds.27419 -
Journal of Gastrointestinal and Liver... Sep 2018Whipple's disease (WD) is known as an infrequent, systemic, chronic infection caused by the actinomycete Tropherima whipplei (T. whipplei). The disease is frequently...
Whipple's disease (WD) is known as an infrequent, systemic, chronic infection caused by the actinomycete Tropherima whipplei (T. whipplei). The disease is frequently characterized by a long prodromal and protean extra-intestinal phase, which often causes misdiagnosis and inappropriate treatments. Herein, we describe the case a 62-year-old man with a histological diagnosis of WD established when oral steroid treatment was started due to rheumatic manifestations, triggering intestinal symptoms. Systematic review of the literature was performed to include studies where WD was eventually diagnosed on duodenal biopsies. Three patients' subgroups were identified according to the clinical presentation.
Topics: Anti-Bacterial Agents; Biopsy; Duodenoscopy; Duodenum; Humans; Intestinal Mucosa; Middle Aged; Tropheryma; Whipple Disease
PubMed: 30240479
DOI: 10.15403/jgld.2014.1121.273.fra -
Practical Neurology Oct 2018A 63-year-old man presented with a 2-month history of progressive right-sided exophthalmos, painful ophthalmoplegia and fevers. As more features developed, he was... (Review)
Review
A 63-year-old man presented with a 2-month history of progressive right-sided exophthalmos, painful ophthalmoplegia and fevers. As more features developed, he was diagnosed with giant cell arteritis, then Tolosa-Hunt syndrome, and transiently responded to corticosteroids. A bland cerebrospinal fluid and highly metabolically active brain (F)-fluoro-D-glucose-positron emission tomography suggested lymphoma. Biopsy of the mass showed sulphur granules with Gram-positive filamentous bacteria with -like colonies. cavernous sinus infections are rare and indolent. They often mimic non-infective causes including other inflammatory and infiltrative conditions, vascular and neoplastic causes, particularly lymphoma. Clinicians should consider infective cavernous sinus syndromes in people with a fluctuating painful ophthalmoplegia that responds poorly to corticosteroids. The term Tolosa-Hunt syndrome is problematic and should be retired or used only with reservation.
Topics: Actinomyces; Actinomycosis; Cavernous Sinus; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Sinusitis
PubMed: 29650638
DOI: 10.1136/practneurol-2017-001844 -
The American Journal of Dermatopathology Apr 2017Inflammatory and subcutaneous nodules can arise in treated and untreated cases of Whipple disease (WD). The inflammatory immune reconstitution syndrome describes... (Review)
Review
Erythema Nodosum Leprosum-Like Lesions Are a Histopathologic Pattern in Whipple's Disease and a Sign of the Immune Reconstitution Inflammatory Syndrome: A Case Series and Review of the Literature.
Inflammatory and subcutaneous nodules can arise in treated and untreated cases of Whipple disease (WD). The inflammatory immune reconstitution syndrome describes paradoxical clinical inflammatory worsening of a preexisting condition because of a return of immune function. Clinicopathologic examination of 4 patients with WD who presented with erythema nodosum leprosum (ENL)-like lesions and the findings of a systematic review of this phenomenon revealed that ENL-like lesions occurred in predominantly middle-aged male patients who suffered from WD, mostly on the legs. Patients showed a nonvasculitic, mostly septal panniculits with neutrophils, macrophages, and lymphocytes. Numerous bacteria-laden periodic acid-Schiff + macrophages and free bacilli were detected in the dermis, as well as subcutaneous septae and adipose lobules. These lesions occurred in both untreated and treated patients as part of inflammatory immune reconstitution syndrome. In conclusion, ENL-like lesions represent a characteristic histopathologic pattern associated with WD, which can occur in different contexts whenever there is a change in the immunological status of the patient. This change can be triggered by antimicrobial treatment, immunomodulatory and immunosuppressant therapy, or occur spontaneously, rarely.
Topics: Erythema Nodosum; Humans; Immune Reconstitution Inflammatory Syndrome; Immunosuppressive Agents; Male; Middle Aged; Whipple Disease
PubMed: 28098596
DOI: 10.1097/DAD.0000000000000641 -
Journal of the Pediatric Infectious... Sep 2015Actinomycetes are Gram-positive bacteria that can be part of the normal human flora of the gastrointestinal, pulmonary, and genital tract. Infections are rare, slowly... (Review)
Review
Actinomycetes are Gram-positive bacteria that can be part of the normal human flora of the gastrointestinal, pulmonary, and genital tract. Infections are rare, slowly progressing and most commonly affect the cervicofacial region. Actinomyces israelii is the most frequently isolated species but a number of other species may cause infection. We report the first postnatally acquired case of an actinomycosis caused by A. neuii in a child. We also provide a systematic review of all published cases of A. neuii infections. In children, there is one case report of a premature infant with perinatally acquired A. neuii sepsis. In adults 21 cases have currently been reported and A. neuii infection was associated with endophthalmitis after eye surgery, foreign material-associated infection and abscess formation in the inguinal, axillary, and mammary area. Our case highlights that a A. neuii infection is also a potential differential diagnosis in children with chronic lymphadenitis.
Topics: Actinomyces; Actinomycosis; Amoxicillin; Anti-Bacterial Agents; Cervix Uteri; Chronic Disease; Clavulanic Acid; Diagnosis, Differential; Female; Humans; Infant; Lymphadenitis
PubMed: 26407440
DOI: 10.1093/jpids/piu096 -
Alimentary Pharmacology & Therapeutics Apr 2015The classical form of Whipple's disease (WD), clinically characterised by arthropathy, diarrhoea and weight loss, is rare. Recently, other more frequent forms of... (Review)
Review
BACKGROUND
The classical form of Whipple's disease (WD), clinically characterised by arthropathy, diarrhoea and weight loss, is rare. Recently, other more frequent forms of Tropheryma whipplei infection have been recognised. The clinical spectrum includes an acute, self-limiting disease in children, localised forms affecting cardiac valves or the central nervous system without intestinal symptoms, and asymptomatic carriage of T. whipplei which is found in around 4% of Europeans. Genomic analysis has shown that T. whipplei represents a host-dependent or opportunistic bacterium. It has been reported that the clinical course of T. whipplei infection may be influenced by medical immunosuppression.
AIM
To identify associations between immunomodulatory treatment and the clinical course of T. whipplei infection.
METHODS
A PubMed literature search was performed and 19 studies reporting on immunosuppression, particularly therapy with tumour necrosis factor inhibitors (TNFI) prior to the diagnosis in 41 patients with Whipple?s disease, were evaluated.
RESULTS
As arthritis may precede the diagnosis of WD by many years, a relevant percentage (up to 50% in some reports) of patients are treated with immunomodulatory drugs or with TNFI. Many publications report on a complicated Whipple?s disease course or T. whipplei endocarditis following medical immunosuppression, particularly after TNFI. Standard diagnostic tests such as periodic acid-Schiff stain used to diagnose Whipple?s disease often fail in patients who are pre-treated by TNFI.
CONCLUSIONS
In cases of doubt, Whipple?s disease should be excluded before therapy with TNFI. The fact that immunosuppressive therapy contributes to the progression of T. whipplei infection expands our pathogenetic view of this clinical entity.
Topics: Anti-Bacterial Agents; Arthritis; Diagnosis, Differential; Humans; Immunoglobulins; Immunosuppressive Agents; Lymphocyte Count; Tumor Necrosis Factor-alpha; Whipple Disease
PubMed: 25693648
DOI: 10.1111/apt.13140 -
Journal of Oral Pathology & Medicine :... Sep 2013Bone resorption inhibitor-related osteopathology of the jaw (BRIOJ) is a severe complication in patients treated with bisphosphonates or denosumab. However, the precise... (Comparative Study)
Comparative Study Review
BACKGROUND
Bone resorption inhibitor-related osteopathology of the jaw (BRIOJ) is a severe complication in patients treated with bisphosphonates or denosumab. However, the precise pathogenesis of BRIOJ is not yet fully understood. Recent studies discovered the presence of Actinomyces colonies in biopsy material from BRIOJ patients. The aim of this study was to analyze current knowledge concerning the impact of Actinomyces on the pathogenesis of this condition and to present data from our own patients.
METHODS
Data from 51 patients with histopathological diagnoses of BRIOJ were retrospectively analyzed. In addition, a systematic literature search for studies describing the presence of Actinomyces was performed.
RESULTS
Actinomyces was present in 86% of our cases and 63.3% of 371 cases presented in the literature. All of our patients and 85% of patients described in the literature had a clearly defined local focus in association with osteopathology. A clear picture of whether Actinomyces colonizes the previously necrotic bone or contributes to inflammation causing subsequent bone necrosis is lacking in the literature.
CONCLUSION
The pathogenesis of BRIOJ remains unknown; however, there seems to be a role for Actinomyces, and possibly other pathogens, in the development of osteopathology of the jaws, which is not exclusive to bisphosphonate therapy. This study supports the hypothesis that an infectious component is of utmost importance for the pathogenesis of BRIOJ.
Topics: Actinomyces; Actinomycosis; Adult; Aged; Aged, 80 and over; Alendronate; Antibodies, Monoclonal, Humanized; Biopsy; Bisphosphonate-Associated Osteonecrosis of the Jaw; Bone Density Conservation Agents; Denosumab; Diphosphonates; Female; Humans; Imidazoles; Male; Middle Aged; RANK Ligand; Retrospective Studies; Tooth Extraction; Zoledronic Acid
PubMed: 23369166
DOI: 10.1111/jop.12038 -
Journal of the Neurological Sciences Sep 2011Whipple's disease (WD) is a rare multisystemic infectious disease that can involve a variety of organs namely the gastrointestinal tract, lymphatic system, heart and... (Review)
Review
INTRODUCTION
Whipple's disease (WD) is a rare multisystemic infectious disease that can involve a variety of organs namely the gastrointestinal tract, lymphatic system, heart and nervous system. Myorhythmia is a hallmark of WD. Isolated CNS involvement is very rare.
CASE
We present a 50 year-old African-American woman with rapid cognitive decline, visual hallucinations, insomnia, dysarthria, and gait unsteadiness. She subsequently developed pendular nystagmus and gaze paresis. Serial brain MRI scans showed T2 hyperintense lesions in the left striatum and right parahippocampal gyrus. FDG-PET scan showed marked increase of glucose uptake in the left putamen. Serum and CSF PCR for Tropheryma whipplei was negative. Stereotactic biopsy of the lesion and tissue PCR was consistent with WD.
REVIEW OF LITERATURE
A systematic review identified 24 cases of isolated intracranial presentation of WD since 1975. Cases with systemic and extracranial manifestations were excluded.
DISCUSSION
In patients with rapidly progressive cognitive decline with negative workup for common etiologies, there should be a high index of suspicion for WD. Diagnosis of WD remains a challenge as traditional methods commonly fail to culture T. whipplei. PET scans can help in identifying areas of inflammation that can be biopsied. Our case proves that a negative serum and CSF PCR should not exclude CNS WD and a brain biopsy of the lesion with PCR assay should be performed when possible.
Topics: Anti-Bacterial Agents; Brain Diseases; Cognition Disorders; Female; Humans; Middle Aged; Whipple Disease
PubMed: 21696776
DOI: 10.1016/j.jns.2011.05.029 -
Pain Mar 2008This systematic literature review aims to assess the prognostic value of psychological factors in the development of late whiplash syndrome (LWS). We included... (Review)
Review
This systematic literature review aims to assess the prognostic value of psychological factors in the development of late whiplash syndrome (LWS). We included prospective cohort studies that provided a baseline measure of at least one psychological variable and used outcome measures relating to LWS (i.e. pain or disability persisting 6 months post injury). A search of electronic databases (Pubmed, Medline, Cinahl, Embase and Psychinfo) up to August 2006 was done using a predetermined search strategy. Methodological quality was assessed independently by two assessors. Data extraction were carried out using a standardised data extraction form. Twenty-five articles representing data from 17 cohorts were included. Fourteen articles were rated as low quality with 11 rated as adequate quality. Meta-analysis was not undertaken due to the heterogeneity of prognostic factors, outcome measures and methods used. Results were tabulated and predefined criterion applied to rate the overall strength of evidence for associations between psychological factors and LWS. Data on 21 possible psychological risk factors were included. The majority of findings were inconclusive. Limited evidence was found to support an association between lower self-efficacy and greater post-traumatic stress with the development of LWS. No association was found between the development of LWS and personality traits, general psychological distress, wellbeing, social support, life control and psychosocial work factors. The lack of conclusive findings and poor methodological quality of the studies reviewed highlights the need for better quality research. Self-efficacy and post-traumatic distress may be associated with the development of LWS but this needs further investigation.
Topics: Community Health Planning; Disability Evaluation; Humans; Prognosis; Prospective Studies; Psychology; Risk Factors; Severity of Illness Index; Statistics as Topic; Whipple Disease
PubMed: 17570588
DOI: 10.1016/j.pain.2007.04.035