whipple's disease confined to the central nervous system

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  • There have been previous isolated case reports of subacute and relapsing neuropathy occurring in preg- nancy, frequently during its later stages. Castaigne and colleagues [ 3 ] referred briefly to 2 patients who had relapses during pregnancy at 3 and 6 months, respec- tively. Calderon-Gonzalez and associates 121 described a patient in whom relapses of a symmetrical poly- neuropathy occurred during the second and third trimesters in three successive pregnancies, and subse- quently while taking oral contraceptives. Novak and Johnson [ll] reported a patient who had a subacute onset of peripheral neuropathy in the third trimester of her first pregnancy and in the second trimester of her second pregnancy. Jones and Berry IS] descrihed a patient who had the onset of CIDP in her first preg- nancy and relapses in the subsequent two pregnancies. Dalakas and Engel 141 studied one patient in whom the onset occurred in the later months of pregnancy. DAmbrosio and de Angelis 153 described a patient who had the onset of relapsing polyneuritis during the third trimester of her first pregnancy, a relapse late in the third trimester of the second pregnancy, and a further relapse six months after commencement of an oral contraceptive.

    Guillain-Bar& syndrome may occur in pregnancy but it does not appear to be more commcm in pregnant women than in the general population, and pregnancy does not seem to influence the course or severity of the disease [I, 5 , 10, 131.

    The mechanism of relapses of CIDP in pregnancy is uncertain. Studies of the immune system in pregnancy have been directed mainly toward the mechanism of protection of the fetus from rejection by the mother [71. It is not known how immunological responses in pregnancy could cause or exacerbate CIDP. PossibIe mechanisms may be the existence of a cross-reactivity between fetal and maternal neural antigens or an im- mune response in pregnancy that provokes an anam- nestic response to neural antigen.

    - P. A. McCombe was a National Health and Medical Research Coun- cil Postgraduate Medical Scholar and P. G. McManis was a Bushell Research Fellow. The statistical advice of Professor G. Berry, School of Public Health and Tropical Medicine, and Dr J . Simes, Royal Prince Alfred Hospital, and the skilled technical assistance of Miss P. Martin are gratefully acknowledged. -

    References 1. Ahlberg G, Ahlmark G. The Landry-Guillain-Barre syndrome

    and pregnancy. Acta Obstet Gynecol Scand 57:377-380, 19.78 2. Calderon-Gonzalez R, Gonzalez-Cantu N, Rizzi-Hernandez H:

    Recurrent polyneuropathy with pregnancy and oral contracep- tives. N Engl J Med 282:1307-1308, 1970

    3. Castaigne P, Brunet P, Nouailhat F: Enquete cilinique sur les polyradiculonCvrites inflammatoires en France. Rev Neurol (Paris) 115:849-872, 1966

    4. Dalakas MC, Engel W K Chronic relapsing (dysimmune) poly-

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    neuropathy: pathogenesis and treatment. Ann Neurol

    DAmbrosio G, de Angelis G: Syndrome de Guillain-Barre au cours de la grossesse. Rev Neurol (Paris) 141:33-36, 1985 Dyck PJ, Lais AC, Ohta M, et al: Chronic inflammatory poly- radiculoneuropathy. Mayo Clin Proc 50:62 1-637, 1975 Jacoby DR, Olding LB, Oldstone MBA: Immunological regula- tion of fetal-maternal balance. Adv Immunol35:157-208, 1984 Jones MW, Berry K Chronic relapsing polyneuritis associated with pregnancy. Ann Neucol9:413, 1981 Korn-Lubetzki I, Kahana E, Cooper G, Abramsky 0: Activity of multiple sclerosis during pregnancy and puerperium. Ann Neurol 16229-231, 1984; (reply to letter) 18:101, 1985 McFarland HR, Heller G L Guillain-Barr6 disease complex: a statement of diagnostic criteria and analysis of 100 cases. Arch Neurol 14:196-201, 1966 Novak DJ, Johnson KF: Relapsing idiopathic polyneuritis dur- ing pregnancy. Arch Neurol 28:219-223, 1977 Prineas JW, McLeod JG: Chronic relapsing polyneuritis. J Neurol Sci 27:427-458, 1976 Ravn H: The Landry-Guillain-Barre syndrome. Acta Neurol

    ~ ( suPP~) : 134- 135, 1981

    Stand 43:l-64, 1967

    Whipples Disease Confined to the Central Nervous System Melanie Adams, MD, Patricia A. Rhyner, MD,t John Day, MD,S Stephen DeArmond, MD, PhD,+ and Edward A. Smuckler, MD, PhD

    Progressive hypersomnia, memory disturbance, and vertical ophthalmoplegia developed in a 63-year-old woman. The diagnosis of Whipples disease of the cen- tral nervous system was suggested by her presentation and results of studies using magnetic resonance imag- ing. Despite a one-month course of antibiotics, active Whipples disease, localized to the central nervous sys- tem, was found at autopsy.

    Adams M, Rhyner PA, Day J, DeArmond S, Smuckler EA: Whipples disease confined

    to the central nervous system. Ann Neurol 21:104-108, 1987

    Whipples disease is a rare multisystem disorder pre- senting most often with symptoms of gastrointestinal malabsorption, fevers, arthralgias, and lymphadenopa-

    From the Departments of Pathology, t Radiology, and $Neurology, School of Medicine, University of California, San Francisco, San Francisco, CA 94143. Received Feb 18, 1986, and in revised form June 9, 1986. Accepted for publication June 9, 1986. Address reprint requests to Dr Adams, Department of Pathology, HSW-501, School of Medicine, University of California, San Fran- cisco, CA 94143.

    104

  • thy. Demonstration of Whipples bacilli, the bacil- liform bodies visible by electron microscopy, estab- lishes the diagnosis E2). It is suggested that these are microorganisms, based on their morphological features and antibiotic sensitivity, but serological definition and culture of the bacilli have not yet been accomplished. The mechanism of dissemination of the bacillus is also unclear. The central nervous system (CNS), liver, spleen, and heart are vulnerable. While it has been thought that the gastrointestinal tract is primarily in- volved, it has been reported that Whipples disease may present solely with CNS involvement IS, 7-91. A case of fulminant primary CNS Whipples disease is reported in which the diagnosis was made on clinical presentation and the magnetic resonance image.

    Case Report A 63-year-old woman was admitted to the University of California, San Francisco, for evaluation of hypersomnia, memory disturbance, and ataxia. She had been in excellent health until approximately 6 months prior to admission, when she noted increased appetite and somnolence. Review of systems revealed the recent development of mild blurred vision and bilateral shoulder pain. She had no gastrointestinal complaints or history of fever. Two months prior to admis- sion, the patient was evaluated with two computerized axial tomography (CT) scans of the head, which were described as normal. She was admitted to the hospital because of progres- sion of her symptoms.

    Physical examination was within normal limits, with the exception of the neurological findings. She was awake and alert, but disoriented to time and unable to name the hospi- tal. Long-term memory was impaired. Pupils were equal, round, and reactive to light. Extraocular movements were abnormal, with limitation of volitional upward gaze but pre- served ability to track upwardly, suggesting a supranuclear gaze palsy. She had limb and trunk ataxia and bilateral Babin- ski signs. There was no evidence of peripheral neuropathy.

    Laboratory values on admission revealed hyponatremia with a sodium level of 119 mEqL and chloride content of 85 mEq/L. Cerebrospinal fluid showed 7 neutrophils, with other values within normal limits. No macrophages were seen.

    In the hospital, she had increasingly abnormal eye move- ments, marked hypersomnia, and disorientation. Paralysis of upward gaze developed, with episodic limitation of left eye abduction. Her pupillary reflexes remained normal. Dolls eyes and caloric reflexes were absent. An electroencepha- logram showed diffuse slowing. Follow-up CT scan (General Electric 8800) revealed contrast enhancement in the region of the hypothalamus. The magnetic resonance images were obtained on a 0.35-Tesla (Diasonics MT15) system. TZ- weighted images (TR, 1.5 sec; TE, 56 msec) showed abnor- mal high-signal intensity in the hypothalamus, uncus, and medial temporal lobes (Fig 1). A jejunal biopsy was per- formed that showed onIy nonspecific Iymphangiectasia with few macrophages, which did not contain periodic acid-Schiff (PAS)-positive bacilli; a diagnosis of gastrointestinal Whip- ples disease could not be made.

    It was decided to treat empirically for primary CNS Whip-

    Fig 1 . Direct coronal T,weighted magnetic resonance image {TR, 1.5 sec; TE, 56 msec), showing abnormal higb-signal in- tensity about the hypothalamus, uncus, and medial temporal lobes.

    ples disease. She was initially treated with high-dose peni- cillin without improvement, then with trimethoprim- sulfamethoxazole, which was discontinued because of granulocytopenia. With return of her white cell count to normal, rifampin and chloramphenicol were started followed by marked clinical improvement. Her sleep pattern nor- malized as did her extraocular movements, although there were continued transient episodes of mild ocular paresis and ataxia. The hyponatremia reversed with demeclocycline treatment, and the patient was discharged one month after admission. Four days after discharge she developed hiccup- ping and pain in the back of the head. Laboratory studies revealed a serum sodium concentration of 153 mglL and he- matocrit of 50%. Demeclocycline was discontinued. The fol- lowing morning she could not be roused from sleep and died en route to the hospital.

    Gross findings at autopsy showed the viscera were normal. Histological examination revealed only a healed granuloma in the apex of the left upper lobe of the lung. Examination of multiple sectio

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