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       Whipples Polyneuropathy Handbook 

    Peripheral neuropathy in Whipples disease: a case report.

    Rusina R, Keller O, Síma R, Zámečník J.

    Source

    Department of Neurology, Thomayer Teaching Hospital, Prague, Czech Republic.

    Abstract

    Whipples disease is a chronic multisystem inflammatory disease with predominantly gastrointestinal manifestations due to Tropheryma whipplei infection. Typical neurological abnormalities include dementia, eye movement abnormalities, hypothalamic dysfunction and oculomasticatory myorhythmias. The literature on peripheral neuropathy in Whipples disease is sparse and the involvement of peripheral nerves in Whipples disease has not been documented convincingly so far. We present a case of Whipples disease presenting by axonal peripheral neuropathy without gastrointestinal involvement. The diagnosis was confirmed by a sural nerve biopsy and consequent PCR of the sample. All clinical signs disappeared progressively during the antibiotic therapy. Two years after the T. whipplei infection, the patient developed dopa-sensitive Parkinson's disease, although these two events seem to be unrelated. This case illustrates the value of peripheral nerve biopsy in cases of axonal neuropathy of unexplained origin and extends the clinical spectrum of Whipples disease to a new modality.

    J Gen Intern Med. 2008 Dec;23(12):2131-3. doi: 10.1007/s11606-008-0776-9. Epub 2008 Sep 11.
    Whipple's disease: neurological relapse presenting as headache for two years.

    Marumganti AR, Murphy TF.
    Source
    Internal Medicine Residency Program, State University of New York, University at Buffalo, Buffalo, NY, USA.
    Abstract
    Whipple's disease is a rare, chronic, multi-systemic infectious disorder caused by the bacterium, Tropheryma whipplei. Relapses are commonly associated with neurological symptoms. We report a case of a 55-year-old man who presented with symptoms of progressive headache, 13 years after apparent complete recovery from intestinal Whipple's disease. Studies showed hydrocephalus with obstruction of the aqueduct and cerebrospinal fluid findings consistent with chronic meningitis. Diagnosis of central nervous system Whipple's disease was confirmed by analysis of the cerebrospinal fluid using polymerase chain reaction. After one year of antibiotic therapy, symptoms resolved and cerebrospinal fluid pleocytosis had improved. This case illustrates that Whipple's disease should be included in the differential diagnosis of central nervous system disorders as it is a potentially treatable cause of chronic meningitis. Failure to recognize this presentation can lead to misdiagnosis or a significant delay in diagnosis and treatment.
    PMID: 18784966 [PubMed - indexed for MEDLINE] PMCID: PMC2596489

    Eur J Neurol. 2006 Jun;13(6):659-61.

    A case of cerebral Whipple's disease initially presenting with isolated focal myoclonus.

    Henning S, Tings T, Schmidt H, Tergau F, Wolf S, Scholz K, Knauth M, Buchner H, Gold R, Paulus W.

    Source

    Department of Clinical Neurophysiology, Georg-August University, Göttingen, Germany. henning@medizin.uni-goettingen.de

    Abstract

    Neurological manifestations in Whipple's disease are highly variable and tend to occur at later stages of the disease. However, isolated, focal neurological symptoms are reported to be rare. Here we describe the successful treatment of a case of cerebral Whipple's disease initially presenting solely with isolated myoclonic jerks of the left hand and forearm evolving to a segmental myoclonus at a later stage. Additionally, we present - to our knowledge - a novel treatment by administration of immunomodulatory therapy (IVIg) in addition to established antibiotics.

    Segmental myoclonus in Whipple's disease.

    Stamboulis E, Kararizou E, Manta P, Grivas I.
    Source
    Department of Neurology Medical School, University of Athens, Greece.
    Abstract
    A patient suffering from Whipple's disease, presenting with segmental myoclonus in the muscular distribution of the muscles of the right facial nerve, which is one of the first neurological findings of the disease is described. Patients suffering from segmental myoclonus and Whipple's disease are reported and the possible anatomical sites of the injury, responsible for the presence of this symptom is discussed
    .

     Eur J Neurol. 2006 Jun;13(6):659-61.
    A case of cerebral Whipple's disease initially presenting with isolated focal myoclonus.
    Henning S, Tings T, Schmidt H, Tergau F, Wolf S, Scholz K, Knauth M, Buchner H, Gold R, Paulus W.
    Source
    Department of Clinical Neurophysiology, Georg-August University, Göttingen, Germany.
    Abstract

    Neurological manifestations in Whipple's disease are highly variable and tend to occur at later stages of the disease. However, isolated, focal neurological symptoms are reported to be rare. Here we describe the successful treatment of a case of cerebralWhipple's disease initially presenting solely with isolated myoclonic jerks of the left hand and forearm evolving to a segmental myoclonus at a later stage. Additionally, we present - to our knowledge - a novel treatment by administration of immunomodulatory therapy(IVIg) in addition to established antibiotics.

     Continue to next page of Handbook of polyneuropathy



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