Intracerebral Whipple's Disease Diagnosed by Stereotactic Biopsy: A Case Report and Review of the Literature

Intracerebral Whipple's Disease Diagnosed by Stereotactic Biopsy: A Case Report and Review of the... AbstractOBJECTIVE AND IMPORTANCE:This case demonstrates the rare occurrence of intracerebral Whipple's disease in a patient lacking classic systemic manifestations of the disease. Because of the nonspecific presentation and the typically deep-seated location of cerebral lesions in these patients, definitive diagnosis is frequently problematic. We present the first reported use of stereotaxy-guided brain biopsy to confirm the diagnosis of isolated intracranial Whipple's disease.CLINICAL PRESENTATION:The patient was a 36-year-old man who presented with a 4-month history of progressive lethargy, hypersomnia, behavioral changes, and weight gain. The results of the physical examination were remarkable only for findings of hypogonadism. Subsequent laboratory evaluation confirmed the diagnosis of hypogonadotrophic hypogonadism, with low levels of testosterone, luteinizing hormone, cortisol, and prolactin.INTERVENTION:A magnetic resonance image of the brain demonstrated hyperintense lesions on T2-weighted images in the regions of the right fornix, hypothalamus, and putamen that subsequently enhanced with intravenously administered contrast medium. A biopsy was then obtained from the right putaminal lesion under stereotactic guidance. Histopathological analysis of the tissue revealed findings consistent with intracerebral Whipple's disease that were subsequently confirmed using electronmicroscopy.CONCLUSION:Intracerebral Whipple's disease should be included in the differential diagnosis of patients presenting with progressive dementia and cognitive decline. In these patients, lesions have typically been observed in the hypothalamus, cingulate gyrus, basal ganglia, insular cortex, and cerebellum. As evidenced by our case, stereotaxy affords clinicians the attractive option of a minimally invasive technique by which to obtain tissue from such deep-seated areas. A review of this rare neurosurgical entity is presented. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurosurgery Oxford University Press

Intracerebral Whipple's Disease Diagnosed by Stereotactic Biopsy: A Case Report and Review of the Literature

Intracerebral Whipple's Disease Diagnosed by Stereotactic Biopsy: A Case Report and Review of the Literature

Whipple's Disease Diagnosed by Stereotactic Biopsy 203 Tropheryma whippelii. Central n erv ou s Intracerebral Whipple's Disease system (CNS) involvem ent is well d e ­ scribed in system ic W h ip p le's patients Diagnosed by Stereotactic Biopsy: A Case and occurs in 10 to 20% of cases (8). On rare occasions, neurological m an ifesta ­ Report and Review of the Literature tions may be the first and only herald of the disease process. The nonspecific symptomatology combined with the rar­ ity of this entity often leads to difficulties Ehud Mendel, M .D., Larry T. Khoo, M .D ., John L. Go, M .D., and delays in the management of these David Hinton, M .D., Chi-Shing Zee, M .D ., patients. We present a rare case of isolated Michael L.J. Apuzzo, M .D . intracerebral Whipple's disease that ulti­ mately required stereotaxy-guided brain Departments of Neurological Surgery (EM, LTK, MLJA), Radiology (JLG, C-SZ), and Pathology biopsy before definitive diagnosis and (DH), University of Southern California School of Medicine, Los Angeles, California treatment could be rendered. OBJECTIVE AND IM P O R T A N C E : This case demonstrates the rare o ccu r­ CASE REPORT rence of intracerebral W hipple's disease in a patient lacking classic A 36-year-old right-handed Greek man systemic manifestations of the disease. Because of the nonspecific pre­ presented with a 4-month history of gen­ sentation and the typically deep-seated location of cerebral lesions in eralized lethargy, behavioral changes, these patients, definitive diagnosis is frequently problematic. We present and brief sporadic periods of hypersom ­ the first reported use of stereotaxy-guided brain biopsy to confirm the nia. These were described as episodes of diagnosis of isolated intracranial Whipple's disease. spontaneous sleep onset with speech ar­ CLINICAL PRESENTATION: The patient was a 36-year-old man who presented rest and unarousability. His family...
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Publisher
Congress of Neurological Surgeons
Copyright
© Published by Oxford University Press.
ISSN
0148-396X
eISSN
1524-4040
D.O.I.
10.1097/00006123-199901000-00123
Publisher site
See Article on Publisher Site

Abstract

AbstractOBJECTIVE AND IMPORTANCE:This case demonstrates the rare occurrence of intracerebral Whipple's disease in a patient lacking classic systemic manifestations of the disease. Because of the nonspecific presentation and the typically deep-seated location of cerebral lesions in these patients, definitive diagnosis is frequently problematic. We present the first reported use of stereotaxy-guided brain biopsy to confirm the diagnosis of isolated intracranial Whipple's disease.CLINICAL PRESENTATION:The patient was a 36-year-old man who presented with a 4-month history of progressive lethargy, hypersomnia, behavioral changes, and weight gain. The results of the physical examination were remarkable only for findings of hypogonadism. Subsequent laboratory evaluation confirmed the diagnosis of hypogonadotrophic hypogonadism, with low levels of testosterone, luteinizing hormone, cortisol, and prolactin.INTERVENTION:A magnetic resonance image of the brain demonstrated hyperintense lesions on T2-weighted images in the regions of the right fornix, hypothalamus, and putamen that subsequently enhanced with intravenously administered contrast medium. A biopsy was then obtained from the right putaminal lesion under stereotactic guidance. Histopathological analysis of the tissue revealed findings consistent with intracerebral Whipple's disease that were subsequently confirmed using electronmicroscopy.CONCLUSION:Intracerebral Whipple's disease should be included in the differential diagnosis of patients presenting with progressive dementia and cognitive decline. In these patients, lesions have typically been observed in the hypothalamus, cingulate gyrus, basal ganglia, insular cortex, and cerebellum. As evidenced by our case, stereotaxy affords clinicians the attractive option of a minimally invasive technique by which to obtain tissue from such deep-seated areas. A review of this rare neurosurgical entity is presented.

Journal

NeurosurgeryOxford University Press

Published: Jan 1, 1999

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