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]

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]

Conclusions CNS WD may present with a variety of MR imaging lesions or no lesions at all. [ajnr.org]

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

  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]

  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]

  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]

  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]

  Brain Involvement in Whipple’s Disease. A Case Report. Acta Neuropath. (Beri.) 33 : 275 – 278. Knox, D.L., Bayless, T.M. and Pittman, F.E. ( 1976 ). Neurologie Disease in Patients with Treated Whipple’s Disease. Medicine 55 : 467 – 476. [cambridge.org]

• Inflammation

  […] of the jelly, present in the rear side of the eye Keratitis - inflammation of the cornea, in the front portion of the eye Retinitis - inflammation of the retina, possibly leading to blindness Retinal hemorrhages - eye disorder, where bleeding occurs [dovemed.com]

  In infectious states, inflammation is often accompanied by alteration of stasis or disturbance of the normal hemostatic balance by procoagulant and anticoagulant mechanisms characterized by biomarkers of inflammation, coagulation, and activation of monocytes [karger.com]

  The pathogenesis of cerebral infarction is not well established, but cerebral vasculitis, arterial fibrosis, thrombosis, and thickening associated with the inflammation of adjacent brain parenchyma and leptomeninges, caused by the hematogenous spread [asmscience.org]

  Mediastinal R4 lymph node biopsy showed benign features with non-necrotizing granulomatous inflammation. Stains for acid-fast bacilli, fungal organisms, and occult Hodgkin’s lymphoma were negative. [shmabstracts.com]

  The more classic signs and symptoms include periodic joint pain, either with or without inflammation, which may persist for years prior to the appearance of any other symptoms. [disabled-world.com]

• Malnutrition

  It contributed then to a weight loss of approximately 25 kg and severe malnutrition. [bjid.org.br]

  The infections caused by the bacteria damages the intestinal lining which then fails to properly absorb nutrients increasing the risk for diarrhea and malnutrition. [onlymyhealth.com]

  This damage prevents the intestinal lining from adequately absorbing nutrients, eventually leading to malnutrition. Whipple's disease is rare, and there's no consensus regarding how many people it affects. [everydayhealth.com]

  A mild malnutrition state with a weight loss of 13 kg, pallor (+/4 +), presence of lower-limb edema (+/4 +), and hypertympanic distended abdomen occurred. [karger.com]

  Eventually, those with Whipple’s lose weight and develop malnutrition. And if not treated, it is inevitably fatal. Dr. Cote figured that the pneumonia and cough that had plagued the patient over the holidays was probably part of the Whipple’s too. [well.blogs.nytimes.com]

• Pathologist

  The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. [dovemed.com]

  Whipple Disease History The condition was described initially in the year 1907 by the American physician and pathologist George Hoyt Whipple. Initially, the disease was referred to as intestinal lipodystrophy. [hxbenefit.com]

  Discussion WD, named after the American pathologist George H Whipple, who described this disease as 'intestinal lipodystrophy' in 1907, is a rare systemic infectious disorder with an estimated incidence of 0.4 per 1,000,000 a year [ 1 ]. [jmedicalcasereports.biomedcentral.com]

  For WD confirmation, the patient underwent a thorough gastroenterological evaluation including repeated duodenal biopsy; all biopsies were deep; histology was done with correct PAS staining and the specimens examined by experienced pathologists were found [dovepress.com]

  In 1907, the pathologist George H. [clinicalgate.com]

• Falling

  The amplitude to velocity relationship for segmented saccades fall along the lower margin of normative value, but the relationship of desired amplitude to velocity relationship falls below desired values. [journal.frontiersin.org]

  Hypnagogic hallucinations are vivid, often frightening, dream-like experiences that occur while dozing, falling asleep and/or while awakening. [thenakedscientists.com]

• Hypotension

  During transfer to a neurologic intensive care unit, persistent hypotension was noted, and the patient responded to phenylephrine. [gastroenterologyandhepatology.net]

  In some patients, hypotension is part of their dysautonomia. Another dysautonomic feature described in the late stages of Whipple disease is hypothermia. [emedicine.medscape.com]

  The most common signs found usually are: relative hypotension, peripheral lymphadenopathy, hyperpigmentation, low-grade fever, abdominal tenderness, peripheral edema, cardiac murmurs, pleural and pericardial friction rubs, ascites, splenomegaly and glossitis [eportal.mountsinai.ca]

  Upon admission to the hospital, she was severely dehydrated with hyperosmolarity, metabolic acidosis, and hypotension. After rehydration and correction of the acid–base dysbalance, she regained consciousness. [dovepress.com]

• Liver Dysfunction

  Later, liver dysfunction and renal insufficiency developed. Liver and kidney biopsies disclosed granulomatous hepatitis and nephritis. Because of the morphologic and clinical findings, the diagnosis of Boeck's disease (sarcoidosis) was made. [thenakedscientists.com]

• Arthralgia

  At admission, he had no active gastrointestinal tract symptoms but his history included single or recurrent episodes of arthralgia, fever and lymphadenopathy. [benthamopen.com]

  Although generally considered a gastrointestinal disease, Whipple's disease often begins as an arthralgia or arthritis. 3. The main symptom tetrad is arthralgia, weight loss, diarrhea with abdominal pain, and pigmentation. 4. [prognosisapp.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]

  Features common to these conditions and to Whipple disease include long-standing, unexplained arthralgia and deterioration of the patient’s condition after treatment with immunosuppressive drugs ( 2 ). [wwwnc.cdc.gov]

  The classic clinical features comprise diarrhea, weight loss, arthralgia, and abdominal pain. 1 Neurologic signs and symptoms can also be observed. 2 The diagnosis of CNS involvement is difficult in the setting of systemic WD, and is even more difficult [cp.neurology.org]

• Dermatitis

  He had dermatitis ocher in the lower limbs, a palpable lymph node in the right anterior cervical region, and swelling in the left thigh. [karger.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]

• Prolapse

  A transesophageal echocardiogram revealed a vegetation on the anterior leaflet of the mitral valve, which prolapsed into the left ventricle. [gastroenterologyandhepatology.net]

• Anger

  Episodes may be triggered by sudden emotional reactions such as laughter, anger, surprise, or fear, and may last from a few seconds to several minutes. The person remains conscious throughout the episode. [thenakedscientists.com]

• 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]

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

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

• Ataxia

  Neurological manifestations include dementia (56 percent), abnormalities of eye movements (33p. cent), involuntary movements (28 percent), seizures, hypothalamic dysfunction, myelopathy, ataxia and psychiatric manifestations. [ncbi.nlm.nih.gov]

  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]

  Cerebellar ataxia and central nervous system whipple disease. Arch Neurol. 2005;62:618-20. Rossi T, Haghighipour R, Haghighi M, et al. Cerebral Whipple’s disease as a cause of reversible dementia. Clin Neurol Neurosurg. 2005;107:258-61. [rarediseases.org]

  Results Subject 1 A 54-year-old man presented with hypersomnia, weight gain, lower extremity weakness, dysarthria, confusion, and gait ataxia. [ajnr.org]

  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]

• 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]

  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]

• Confusion

  Possible neurological signs of the disease are visual impairment, lack of control over eye movement, memory loss, confusion, and difficulty walking. [ucsdguardian.org]

  Neurological issues that can develop from Whipple’s Disease include dementia, memory loss, confusion, and decreased consciousness. [disability-benefits-help.org]

  Enlarged lymph nodes Skin darkening (hyperpigmentation) in areas exposed to the sun and in scars Chest pain Enlarged spleen Neurological signs and symptoms may include: Difficulty walking Visual impairment, including lack of control of eye movements Confusion [drugs.com]

  Neurological symptoms — dementia, memory loss, confusion — are more common in those with severe abdominal disease. [well.blogs.nytimes.com]

  Granulomatous disease including sarcoid and more chronic CNS infections such as tuberculosis, particularly if atypical bacteria are implicated, and AIDS is associated can cause diagnostic confusion. [jnnp.bmj.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]

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]

[…] 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]

After treatment most symptoms of the disease usually disappear in the initial couple of months. However, the relapse is common, making it very important to closely monitor the treatment. [onlymyhealth.com]

Is there any treatment? The standard treatment for Whipple's disease is a prolonged course of antibiotics (up to two years), including penicillin and cefriaxone or doxycycline with hydroxychloroquine. [my.clevelandclinic.org]

(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]

Hashimoto’s encephalopathy: epidemiologic data and pathogenetic considerations. J Neurol Sci 2004; 217(2):165–168. CrossRef PubMed Google Scholar Ferracci F, Moretto G, Candeago RM et al. [link.springer.com]

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]

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]