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]

• Asymptomatic

  All patients should be treated and monitored as if they had central nervous system disease even if they are asymptomatic. [ncbi.nlm.nih.gov]

  We analysed 39 samples from 39 patients with Whipple's disease and 10 samples from 10 asymptomatic carriers. [mic.microbiologyresearch.org]

  However, only 10-20% of Whipple disease patients develop neurological symptoms whilst the rest of the cases remain asymptomatic. [radiopaedia.org]

  Patient remained asymptomatic from a neurologic standpoint. He clinically did well and was sent home on intravenous ceftriaxone for two weeks, followed by trimethoprim-sulfamethoxazole to continue for at least one year. [shmabstracts.com]

  First, a PCR-based study ( 69 ) demonstrated positive results for T. whipplei in CSF in 7 out of 10 (70%) neurologically asymptomatic patients at the time of diagnosis of intestinal Whipple’s disease. [oncohemakey.com]

• Anemia

  History Pernicious anemia is the most common cause of vitamin B-12 deficiency. It was first described by Thomas Addison in 1849 and the anemia associated with it is also called Addison-Biermer disease. [ispub.com]

  This will help determine if you have a low count of red blood cells and low amounts of albumin, which are both signs of anemia. Anemia is an indication that you might have Whipple’s disease. [healthline.com]

  Laboratory tests on admission revealed anemia, positive inflammatory activity tests, and normal coagulation. [karger.com]

  Other symptoms include: stomach pain weakness fatigue anemia a darkening or graying of skin exposed to the sun Endocarditis has been reported in a small number of cases. Sometimes it is the only symptom of Whipple disease. [medicalnewstoday.com]

  He had been recently diagnosed with iron deficiency anemia the month prior, but this did not improve despite iron supplementation. CT of his abdomen revealed diffuse intraabdominal, chest, and axillary lymphadenopathy. [shmabstracts.com]

• Lymphadenopathy

  Clinical manifestations are protean (joint pain, fever, weight loss, abdominal pain, lymphadenopathies), and the diagnosis is often delayed. [ncbi.nlm.nih.gov]

  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]

  In the first group, abdominal lymphadenopathy could be seen on CT scan. One-third of the examined patients had mesenteric lymphadenopathy (22 cases in the literature and 3 of our cases). [journals.lww.com]

  CT of his abdomen revealed diffuse intraabdominal, chest, and axillary lymphadenopathy. [shmabstracts.com]

• Swelling

  This is generally noticed as leg swelling and breathlessness due to accumulation of fluids as the heart becomes unable to pump the fluids throughout the body. [hxbenefit.com]

  This may show: Enlarged lymph glands Heart murmur Swelling in body tissues ( edema ) Tests to diagnose Whipple disease may include: Complete blood count ( CBC ) Polymerase chain reaction (PCR) test to check for the bacteria that cause the disease Small [mountsinai.org]

  In a majority of individuals, a lymph node swelling is caused by a benign process such as an inflammation or infection. In many cases, swollen lymph nodes are part of other signs and symptoms. [dovemed.com]

  […] fever fatigue anemia, or low red blood cell count The following signs and symptoms don’t occur as frequently but can indicate that the condition is getting worse: skin discoloration inflamed lymph nodes a chronic cough pain in the chest pericarditis, or swelling [healthline.com]

  Symptoms of endocarditis include breathlessness and swelling of the legs due to fluid buildup. The heart becomes inflamed and cannot pump fluid through the body. Whipple disease can progress to the central nervous system (CNS). [medicalnewstoday.com]

• Heart Failure

  Due to this digestive system disease, you may experience symptoms like weight reduction, fever, fatigue, regular joint pain, anemia, persistent looseness of the bowels, enlarged lymph nodes, darkening of the skin, chest pain, heart failure, pericarditis [bblc.tv]

  Other less common signs and symptoms of the disease may include enlarged lymph nodes, darkening of the skin, chronic cough, chest pain, and heart failure. [onlymyhealth.com]

  heart failure a heart murmur poor vision dementia numbness insomnia muscle weakness tics trouble walking poor memory Infection with the T. whipplei bacteria is the one and only known cause of Whipple’s. [healthline.com]

  failure Left untreated, Whipple's disease can cause serious complications, including long-lasting nutritional deficiencies, heart damage, and brain damage. [everydayhealth.com]

  The cardiac involvement of Whipple's disease includes the endocardium, myocardium, pericardium, and even the coronary arteries, it is not surprising that these patients occasionally have congestive heart failure and symptomatic pericarditis. [enotes.tripod.com]

• Heart Murmur

  This may show: Enlarged lymph glands Heart murmur Swelling in body tissues ( edema ) Tests to diagnose Whipple disease may include: Complete blood count ( CBC ) Polymerase chain reaction (PCR) test to check for the bacteria that cause the disease Small [mountsinai.org]

  heart failure a heart murmur poor vision dementia numbness insomnia muscle weakness tics trouble walking poor memory Infection with the T. whipplei bacteria is the one and only known cause of Whipple’s. [healthline.com]

  […] involves performing a physical exam, which may reveal: Heart murmur Enlarged lymph glands Edema or swelling in the body tissues The various diagnostic tests to evaluate Whipple disease include: Small bowel biopsy CBC or complete blood count PCR or polymerase [hxbenefit.com]

  Other well-recognized physical findings in patients with Whipple disease include fever, peripheral arthritis, heart murmurs, pleural or pericardial friction rubs, and ocular abnormalities, reflecting the systemic nature of the infection. [gastroenterologyandhepatology.net]

• Heart Disease

  Association: Endorsed by the Infectious Diseases Society of America Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Cardiovascular Disease in the Young, and the Councils on Clinical Cardiology, on Rheumatic Fever, Endocarditis, and Kawasaki [link.springer.com]

  […] of the membrane surrounding the heart heart failure Neurologic symptoms of Whipple’s disease include Neurologic symptoms occur in some people diagnosed with Whipple’s disease and can mimic symptoms of almost any other neurologic condition. vision problems [tipdisease.com]

  The most common diseases complicating thrombosis are cancers, systemic inflammation, sepsis, heart disease, protein-losing states, infectious diseases, and the use of a central venous catheter. [karger.com]

• Hepatomegaly

  This can result in the accumulation of fluid in the abdominal cavity, called chylous ascites Enlarged liver (hepatomegaly) Involvement of the bone marrow, which is seen in up to 40% of the affected individuals Damage to the heart walls causing murmurs [dovemed.com]

  Exploratory laparotomy was performed in August 1988 and showed multiple retroduodenal, pancreatic, and mesenteric lymphadenopathy associated with hepatomegaly and splenomegaly. [journals.lww.com]

• Hyperpigmentation

  However, this patient's examination reveals a valuable clue which helps short-circuit the diagnostic pathway: the presence of generalized hyperpigmentation. [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]

  Other less frequently observed signs include fever, cough, chest pain, darkening of the skin (hyperpigmentation) in scars and areas exposed to sun, enlarged lymph nodes, and an enlarged spleen. [ucsdguardian.org]

  The third stage is characterized by steatorrhea, cachexia, lymphadenopathy, hyperpigmentation, and neurologic or ocular dysfunction. Currently, survival is much improved as a result of effective antibiotic treatment. [eportal.mountsinai.ca]

  Various extraintestinal manifestations include arthritis and arthralgia, lymphadenopathy, pleuritis, bronchiectasis, fever, skin hyperpigmentation, and neurologic abnormalities (personality changes and dementia). [enotes.tripod.com]

• Muscle Weakness

  The most common symptoms of Whipple's disease are: diarrhea abdominal pain weight loss malabsorption edema Neurological symptoms may include: headaches, and vision problems memory problems and dementia muscle weakness or twitching Although not covered [ddc.musc.edu]

  Facial tingling, muscle weakness, dementia, vision issues, and memory issues are a few of the neurologic symptoms you may experience because of this disease. And, have you considered: Incubation duration of lyme disease is around two weeks. [bblc.tv]

  Some people may also have some neurologic symptoms including facial numbness, vision problems, dementia, headache, memory problems, muscle weakness or twitching, and difficulty walking. [onlymyhealth.com]

  weakness tics trouble walking poor memory Infection with the T. whipplei bacteria is the one and only known cause of Whipple’s. [healthline.com]

  A wide range of symptoms may result, including: Weakness Darkened skin Fatty or bloody stools Loss of appetite Enlarged lymph nodes Vision problems Memory problems Facial numbness Muscle weakness and difficulty walking Joint stiffness Hearing problems [everydayhealth.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]

• Psychiatric Manifestation

  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]

  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]

  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]

• 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]

• 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]

• Personality Change

  Various extraintestinal manifestations include arthritis and arthralgia, lymphadenopathy, pleuritis, bronchiectasis, fever, skin hyperpigmentation, and neurologic abnormalities (personality changes and dementia). [enotes.tripod.com]

  CNS manifestations may first become apparent as a memory disorder, personality change, or dementia. Other common clinical signs include ophthalmoplegia, nystagmus, and myoclonia. [prognosisapp.com]

  Abstract Background A 39-year-old man presented with a history of several months of progressive personality changes, social withdrawal, bradykinesia, mutism, dysphagia, worsening gait, and difficulty with daily living activities. [nature.com]

  Central nervous system manifestations are present in up to 10% of patients and include headache, ataxia, deafness, muscle weakness, lethargy, sensory deficits, visual changes, progressive dementia, seizures, personality changes, and meningitis. [eportal.mountsinai.ca]

  There is no evidence for person to person transmission and it does not seem to occur in clusters. [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]

• 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]

  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]

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

  Approximately 71% of patients with CNS Whipple’s disease show signs of cognitive impairment, and nearly half of these patients also demonstrate psychiatric disturbances ( 93 ). Patients have sometimes been misdiagnosed with Alzheimer disease. [oncohemakey.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]