A rationalisation of the 1350 boxes used throughout the book gives a simpler and clearer presentation of the various categories. [books.google.com]

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]

The presentation of OMM is pathognomonic for Whipple’s disease and thus can aid in quickly making a diagnosis. [eyewiki.aao.org]

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]

Either diarrhea or steatorrhea is the most common presenting complaint; however, this symptom is not invariably present. 3 Other intestinal symptoms include abdominal bloating, cramps, and anorexia. [gastroenterologyandhepatology.net]

• Whipple Disease

  Since both Whipple’s disease and sarcoidosis can cause a multitude of clinical findings, many of which overlap, it is important to perform diagnostic stains for Whipple’s disease on small bowel biopsies showing granulomas before giving a definitive diagnosis [shmabstracts.com]

  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]

  Whipple’s Disease. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on May 29, 2016.) [eyewiki.aao.org]

  The topic of CNS Whipple’s disease came up in a recent email exchange, so I thought I’d share with you a fact sheet on the disease: DESCRIPTION: First described by George Whipple in 1907, Whipple disease is a multisystem disorder that usually includes [neuropathologyblog.blogspot.com]

  The tinctorial demonstration of a glycoprotein in Whipple's disease. Proc Soc Exp Biol Med. 1949. 72:225. Paulley JW. A case of Whipple's disease (intestinal lipodystrophy). Gastroenterology. 1952. 22:128-33. [emedicine.medscape.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]

• Myoclonus

  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]

  A specific clinical triad noted in CNS-WD includes dementia, vertical ophthalmoplegia, and myoclonus. [emedicine.medscape.com]

  Segmental myoclonus in Whipple's disease Electromyogr Clin Neurophysiol 1995; 35: 113-6. ]. [benthamopen.com]

  The most common neurologic manifestations are dementia, ophthalmoplegia, hypothalamopituitary dysfunction, and myoclonus. Occulofacial-skeletal myorhythmia is particularly suggestive of Whipple’s disease. [neuropathologyblog.blogspot.com]

  Clinical presentation Clinical features are non-specific and include: progressive dementia external ophthalmoplegia myoclonus seizures ataxia hypothalamic dysfunction (e.g. sleep disorders, polydipsia, polyphagia) Radiographic features The CT scans and [radiopaedia.org]

• Oculomasticatory Myorhythmia

  Oculomasticatory Myorhythmia. Oculomasticatory Myorhythmia :: EHSL - Shirley H. Wray Neuro-Ophthalmology Collection 2002. [eyewiki.aao.org]

  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]

  Note that oculomasticatory myorhythmia (continuous rhythmic movements of the eye with mastication and convergence) is almost pathognomonic of the disease. [prognosisapp.com]

  myorhythmia [OMM] [18] and/or oculofacialskeletal myorhythmia [OFSM]). [emedicine.medscape.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]

• Nystagmus

  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]

  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]

  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]

• Cognitive Impairment

  Approximately 20%–40% of patients suffer from neurological manifestations such as encephalopathy, ophthalmoplegia, myoclonus, ataxia, upper motor neuron manifestations, cognitive impairment, and hypothalamic manifestations. 3 CNS involvement carries a [dovepress.com]

  Detailed neuropsychological examination showed significant cognitive deterioration, with predominant memory, visuomotor and executive impairment. [benthamopen.com]

  Patients with central nervous system (CNS) involvement may present in a variety of ways, including cognitive impairment, psychiatric manifestations, gaze palsies, upper motor neuron signs, and hypothalamic dysfunction. [link.springer.com]

  Over a period of several years prior to presentation, the patient experienced progressive hearing loss (predominantly affecting his left ear), and progressive cognitive impairment was noted by his family members. [gastroenterologyandhepatology.net]

  Patients may present in a variety of ways, including cognitive impairment, psychiatric manifestations, gaze palsies, upper motor neurone signs and hypothalamic dysfunction. [jmedicalcasereports.biomedcentral.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]

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

  positive rod shaped bacillus which is not acid fast. [jnnp.bmj.com]

[…] for the Initial treatment of Morbus Whipple' (SIMW)) [ 1 ]. [jmedicalcasereports.biomedcentral.com]

He was given a short-duration antibiotic treatment without further episodes. [benthamopen.com]

[…] second and third year of treatment. [dovepress.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]

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]

(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]

Though there are too few reported cases in the literature to prove a correlation, earlier recognition and treatment of CNS Whipple’s disease may lead to fewer neurologic complications and better long-term prognosis. [shmabstracts.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]

If treated properly and promptly with antibiotics, the prognosis is generally good. References 1. [prognosisapp.com]

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]

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]

Multispacer typing of Rickettsia prowazekii enabling epidemiological studies of epidemic typhus. J Clin Microbiol 43, 4708 –4712. [mic.microbiologyresearch.org]

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]

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]

Delineating these mechanisms will facilitate our understanding of the pathophysiology and heterogeneity of saccadic disorders. [journal.frontiersin.org]

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]

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]

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]

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]