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Neurologic Manifestation of Whipple Disease

Whipples Disease


Presentation

  • A rationalisation of the 1350 boxes used throughout the book gives a simpler and clearer presentation of the various categories.[books.google.com]
  • Gerard A, Sarrot-Reynauld F, Liozon E, et al.: Neurologic presentation of Whipple disease: Report of 12 cases and review of the literature . Medicine (Baltimore) 2002, 81 :443–457.[link.springer.com]
  • Uveitis, retinitis, optic neuritis and papilloedema may be found. 80 percent of the reported cases of neuro-Whipple had associated systemic symptoms or signs but many patients are presenting without concurrent intestinal manifestation.[ncbi.nlm.nih.gov]
  • Conclusions CNS WD may present with a variety of MR imaging lesions or no lesions at all.[ajnr.org]
  • CLINICAL PRESENTATION: Most patients present with such symptoms as arthralgia, weight loss, steatorrhea, lymphadenopathy, and hyperpigmentation. Some patients develop neurological disease, usually in combination with systemic symptoms.[neuropathologyblog.blogspot.com]
Whipple Disease
  • This is a series of 12 patients with CNS Whipple’s disease, along with an analysis of 122 CNS Whipple’s disease cases reported in the literature. CrossRef Google Scholar 10.[link.springer.com]
  • Although previously considered a late manifestation of Whipple disease, neurological involvement is now frequently the initial clinical manifestation and represents the greatest risk for long-term disability.[ncbi.nlm.nih.gov]
  • Anyone can be affected by Whipple's disease, although it is more common among middle-aged Caucasian males. Whipple's disease is fatal. Signs of Whipple's Disease The signs and symptoms of Whipple's disease can vary rather widely.[disabled-world.com]
  • Whipple’s Disease. Virch. Arch. Path. Anat. 366 : 238 – 269. Feurle, G.E., Volk, B. and Waldherr, R. ( 1979 ). Cerebral Whipple’s Disease with Negative Jejunal Histology. N.E. J.M., 300 : 907 – 908.[cambridge.org]
  • How to Prevent Whipple's Disease Currently, there is no known way to prevent Whipple's disease.[tipdisease.com]
Vertical Gaze Palsy
  • gaze palsy, rhythmic myoclonus, 3) dementia with psychiatric symptoms, 4) hypothalamic manifestations”.[benthamopen.com]
  • It is characterized by smooth, continuous, slow (1-3 Hz), pendular, convergent-divergent nystagmus, concurrent contractions of the masticatory muscles, supranuclear vertical gaze palsy, and occasionally, rhythmic movements of the limbs (see video in Media[eyewiki.aao.org]
  • She still suffered from vertical gaze palsy and sixth cranial nerve paresis on the left side. MRI from November 2014 found no new lesions; the previous lesions had partially regressed.[dovepress.com]
  • Pathognomonic findings include oculomasticatory or oculofacial-skeletal myorhythmia, which generally occur with supranuclear vertical gaze palsy. 7 Cranial nerve findings (including hearing loss and visual changes) have also been described. 8 Rarely,[gastroenterologyandhepatology.net]
Oculomasticatory Myorhythmia
  • Oculomasticatory myorhythmia (OMM) is a pathognomonic manifestation of Whipple’s disease of the central nervous system.[eyewiki.aao.org]
  • Note that oculomasticatory myorhythmia (continuous rhythmic movements of the eye with mastication and convergence) is almost pathognomonic of the disease.[prognosisapp.com]
  • A progressive dementia may be seen, but the pathognomonic signs of CNS disease, when present, are oculomasticatory myorhythmia and oculofacial-skeletal myorhythmia. Antibiotics that cross the blood-brain barrier are therefore required.[enotes.tripod.com]
  • Oculomasticatory myorhythmia and oculofacial-skeletal myorhythmia occur and are said to be pathognomonic for Whipple's disease—Louis et al 34 claim that these findings have not been documented in cases other than Whipple's disease and consider them to[jnnp.bmj.com]
  • The issues that are most commonly seen in relation to the brain include: Decreased levels of consciousness Dementia Confusion Loss of memory Myorhythmia of the face and eye movement disturbances occurs in a condition known as oculomasticatory myorhythmia[hxbenefit.com]
Nystagmus
  • Gaze-Evoked Nystagmus Our patient also had gaze-evoked nystagmus. The slow phase of the nystagmus had velocity-decreasing waveform (arrows in Figure 3 ).[journal.frontiersin.org]
  • Nystagmus present Oculopalatal myoclonus – oculopalatal myoclonus refers to a condition in which palatal myoclonus is associated with skeletal limb involvement and convergent-divergent nystagmus that can persist during sleep.[eyewiki.aao.org]
  • Other common clinical signs include ophthalmoplegia, nystagmus, and myoclonia. Various cranial nerve symptoms, such as hearing loss and blurring of vision, have also been reported.[prognosisapp.com]
  • Further neurological symptoms include disorders of eye movement, for example, ophthalmoplegia and nystagmus, complex cranial-nerve manifestations and myoclonus [ 1 , 5 ].[jmedicalcasereports.biomedcentral.com]
  • Convergent and return divergent movements happen at the same speed (this does not represent nystagmus, as both phases are of the same speed) and are not accompanied by miosis or accommodation.[emedicine.medscape.com]
Pendular Nystagmus
  • It differs from other forms of pendular nystagmus in that it is smooth and continuous with a high amplitude and slow frequency.[eyewiki.aao.org]

Workup

  • In most patients, differentiating between these etiologies requires a systematic and at times protracted workup.[prognosisapp.com]
  • Nevertheless, at the present time, culture is not a suitable tool for routine diagnostic purposes in the workup of possible Whipple’s disease.[oncohemakey.com]
Gram-Positive Rods

Treatment

  • Mahnel R, Marth T: Progress, problems, and perspectives in diagnosis and treatment of Whipple’s disease . Clin Exp Med 2004, 3 :39–43. This is a review of current recommendations for diagnosis and treatment of Whipple’s disease.[link.springer.com]
  • Extensively updated to reflect advancements in the field, this fifth edition covers new imaging modalities such as pediatric neuroimaging, spinal fluid examination, neurophysiology, as well as the treatment and management of epilepsy, ADHD, infections[books.google.com]
  • Most agree that initial treatment with a combination of parenteral penicillin and streptomycin for at least 14 days is appropriate, thereafter cotrimoxazole orally 3 times a day for at least one and probably for two years.[ncbi.nlm.nih.gov]
  • Treatment Antibiotics are primary treatment for Whipple disease. For many years, Whipple disease was considered a fatal primary metabolic disorder, but current treatment greatly improves a patient's chances of survival.[medicalnewstoday.com]
  • If you relapse during treatment, your doctor may change your antibiotics. Treatment for standard cases In most cases, Whipple's disease therapy begins with 14 days of intravenous (IV) ceftriaxone (Rocephin).[tipdisease.com]

Prognosis

  • (Outcomes/Resolutions) The prognosis of Whipple’s Disease Lymphadenopathy is based on the severity of the underlying bacterial infection In general, the prognosis of Whipple’s Disease is excellent with appropriate antibiotic treatment Without treatment[dovemed.com]
  • If treated properly and promptly with antibiotics, the prognosis is generally good. References 1.[prognosisapp.com]
  • Prognosis: The prognosis for this disease so treated is excellent, and most patients are cured. Occasional patients may relapse when antibiotics are discontinued, necessitating their reinstitution.[enotes.tripod.com]
  • These include the following: Weight loss Brain damage Nutritional deficiencies Heart valve damage caused by endocarditis Relapse of symptoms caused by drug resistance Whipple Disease Prognosis Following treatment, the likely outcome or prognosis for Whipple[hxbenefit.com]

Etiology

  • In most patients, differentiating between these etiologies requires a systematic and at times protracted workup.[prognosisapp.com]
  • To the Editor: The clinical spectrum of Whipple disease has widely expanded since its etiologic agent, Tropheryma whipplei, was isolated in 2000 ( 1 ).[wwwnc.cdc.gov]
  • He had a history of a short-lasting arthritis of left ankle, unknown etiology recurring episodes of fever and one episode of persistent high fever and lymphadenopathy for which he was referred to an outpatient clinic; at that time, laboratory examinations[benthamopen.com]
  • Myorhythmia: Phenomenology, Etiology, and Treatment. Movement Disorders 2015;30(2):171–179. doi:10.1002/mds.26093. 7.0 7.1 Fenollar, F., Puéchal, X., Raoult, D. January 2007. "Whipple's disease".[eyewiki.aao.org]
  • Infectious etiology was suspected and PENICILLIN administered... without finding any germ! But of course it worked -empirically- for a few months.[thenakedscientists.com]

Epidemiology

  • Genotyping studies done in Europe, Africa and Asia showed high genetic diversity with no correlation between genotypes and clinical features, but contributed to a better understanding of the epidemiology of the disease.[mayoclinic.pure.elsevier.com]
  • EPIDEMIOLOGY: Interestingly, Whipple’s disease occurs about six times more commonly in men than in women. The disease tends to occur in late middle age.[neuropathologyblog.blogspot.com]
  • EPIDEMIOLOGY Classical Whipple’s disease with intestinal involvement is a rare entity.[oncohemakey.com]
  • A small epidemiologic study from western Switzerland calculated the incidence of Whipple’s disease to be approximately 0.4 per million of the population per year. 16 A similar incidence of 0.4 per million per year was estimated for Germany. 17 An epidemiologic[clinicalgate.com]
  • Genotyping in Whipple's disease may, however, be useful in epidemiological studies. Edited by: K. E. Weaver The GenBank/EMBL/DDBJ accession numbers for the HVGS marker sequences of T. whipplei are given in Table 1 T1 .[mic.microbiologyresearch.org]
Sex distribution
Age distribution

Pathophysiology

  • In addition, the genome contains a family of genes predicted to encode an unusual set of variable surface-associated proteins likely involved in pathogenesis and pathophysiology [ 3 ].[academic.oup.com]
  • Delineating these mechanisms will facilitate our understanding of the pathophysiology and heterogeneity of saccadic disorders.[journal.frontiersin.org]
  • Bacteria adhesion to a thrombogenic surface can assist in the pathophysiology and explain thromboembolic events associated with infection [ 10, 13 ]. Risk factors for venous thrombosis are well defined in medical reports.[karger.com]
  • Furthermore, the exact pathophysiology is poorly understood, though it is suspected that damage is caused by direct bacterial replication, more so than the associated host's immune response with inflammatory damage. 6 Histologic specimens of WD from the[ajnr.org]
  • Pathophysiology Of Neurological Manifestations Cyanocobalamin mediates two important enzymatic reactions in humans.[ispub.com]

Prevention

  • Whipple's disease is a rare, infectious condition that prevents the small intestine from properly absorbing nutrients.[ddc.musc.edu]
  • Currently, there’s no known way to prevent Whipple’s disease. Whipple’s disease prevents your body from properly absorbing nutrients. Because of this, it affects many different parts of the body and is associated with a variety of symptoms.[healthline.com]
  • Prevention Until now, there is no known way to prevent Whipple disease. However, practicing good hygiene, for example, regular hand-washing, can reduce the risk.[medicalnewstoday.com]
  • Much of this research is aimed at learning more about these disorders and finding ways to prevent, treat, and, ultimately, cure them. Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission.[my.clevelandclinic.org]
  • It is still not clear if mild or moderate B12 deficiency can cause dementia and whether supplementation of the diet with B12 can prevent or delay the onset of dementias like Alzheimer's disease.[ispub.com]

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