Whipple’s Disease
Abstract and Commentary
Commentary by Michael Rubin, MD, Professor of Clinical Neurology, New York Presbyterian Hospital-Cornell Campus, and Assistant Editor, Neurology Alert.
Synopsis: Progression of the cord lesion and lack of steroid response prompted antibiotic treatment for Whipple’s disease.
Source: Schroter A, et al. Whipple’s Disease Presenting as an Isolated Lesion of the Cervical Spinal Cord. Eur J Neurol. 2005;12;276-279.
This rare multi-system disease, predominantly affecting men with a mean age of onset of 50 years (80%), usually manifests systemically with fever, weight loss, diarrhea, abdominal pain, polyarthritis, lymphadenopathy, and hyperpigmentation. Neurologic symptoms occur in 10-20 %, but less than 5% present with or have purely neurologic disease. Slowly progressive dementia, similar to Alzheimer’s disease, though more rapid, is the usual neurologic presentation, but cerebellar ataxia, supranuclear ophthalmoplegia, seizures, myoclonus, and oculomasticatory myorhythmia, a unique pendular vergence oscillation of the eyes with concurrent masticatory muscle contractions, are also seen. Central nervous system involvement, limited to the spinal cord, has been described only twice.
In this case report, a 50-year-old man developed stocking-glove hypesthesia over 3 weeks, with normal strength and deep tendon reflexes. Cervical spine MRI revealed swelling and hyperintensity on T2 turbo spine echo, with enhancement of the dorsal aspect of the cervical cord following GAD administration. Brain MRI and tibial, median, and visual-evoked potentials were normal. Cerebrospinal fluid analysis revealed 7 cells, but was otherwise unremarkable, including negative PCR for Tropheryma whipplei. Progression of the cord lesion, and lack of steroid response, prompted antibiotic treatment for Whipple’s disease. Within 2 months, significant clinical and radiographic improvement was seen, and PCR, on a peripheral blood specimen, returned positive for T. whipplei.
Commentary
George Hoyt Whipple was a pathology instructor at Johns Hopkins University in 1907 when he published a case report in the Johns Hopkins Hospital Bulletin regarding a 36-year-old medical missionary who died following a 5-year illness consisting of recurrent arthropathy, worsening steatorrhea, weight loss, and anemia. At autopsy, Whipple reported foamy intestinal mucosal mononuclear cells, later shown to be periodic acid Schiff (PAS) positive, in which he described a “peculiar rod-shaped organism.” The bacterial origin was confirmed in 1961, and the bacillus, a gram-positive actinomycete unrelated to any known genus, was cultivated for the first time in 2000 (N Engl J Med. 2000;342;620-625).
It is a disease that raises more questions than answers. Whether the same bacillus causes all the manifestations of the disease is uncertain. Humans are the only known host, but it does not seem to be contagious. Infection may follow ingestion of the organism, but this is speculative, based on the intestinal mucosal location of the organism on jejunal biopsy. Whether direct invasion of the organism or a subsequent inflammatory response results in disease, remains to be determined. Most of us will never see a case, but one thing is certain, the topic is popular on neurology board examinations. — Michael Rubin
Progression of the cord lesion and lack of steroid response prompted antibiotic treatment for Whipple’s disease.
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