PSC and MS

[Guest guest]

Dear and ;

Here's the case report on PSC and MS that I promised I would post:

_____________________

Digestive Diseases and Sciences, Vol. 48, No. 7 (July 2003), p. 1397

LETTER TO THE EDITOR

SIMULTANEOUS OCCURRENCE OF PSC AND MS IN A PATIENT

To the Editor:

Primary sclerosing cholangitis (PSC) and multiple sclerosis (MS) are

both diseases of unknown etiology in which immunologic factors are

thought to be important. We report what we believe is the first case

of PSC and MS occurring in the same individual.

A 41-year-old white man had been symptomatic with recurrent episodes

of optic neuritis for 12 years prior to his evaluation. Six months

after the initial episode of optic neuritis he had paresthesias in

the legs and hands which progressed over a month, then resolved 4

months after onset. He presented with recurrence of similar symptoms

for the preceding 6 weeks. Eight years previously, he was noted to

have elevated liver enzymes. An ERCP done at that time showed

evidence of PSC, and liver biopsy showed stage 2 fibrosis. He was

started on ursodiol 600mg twice a day, with a subsequent reduction in

enzymes.

The examination showed bilateral optic atrophy, mild difficulty with

the finger-nose test, and generalized hyperreflexia with a flexor

plantar response. The skin was normal. The liver edge was palpable 1

cm below the right costal margin and felt normal.

The AST, alkaline phosphatase, and CA19-9 tumor marker were normal.

The liver ultrasound was unremarkable except for a small hemangioma.

The cerebrospinal fluid study showed an elevated IgG index and

synthesis rate, 3 oligoclonal bands, 10 cells/ul (lymphocytes 82% and

monocytes 18%), a slightly elevated protein and abnormal glucose. A

brain MRI showed multiple T2 hyperintensities in the brain and brain

stem consistent with MS. The visual evoked potential studies were

abnormal. The neurological findings were consistent with MS.

To our knowledge, there is no previous report of a patient with PSC

and MS. There have been three reports of patients with primary

biliary cirrhosis and MS (1-3). The association between primary

sclerosing cholangitis and ulcerative colitis is well described (4).

There have been reports in the literature about the association of

ulcerative colitis with MS (5) and a concern that focal white-matter

lesions in the brain in patients with inflammatory bowel disease

(IBD) may be linked toMS(6, 7). MS and IBD are said to coexist at

about three times the expected frequency (5), and familial

aggregation of the two diseases is said to occur about nine times

more often than would be expected by chance (8).

The association of PSC and MS in this patient may have occurred by

chance. The relationship between IBD and MS on one hand, and the

striking association between PSC and ulcerative colitis on the other,

makes this report more interesting. As PSC may remain asymptomatic

for years, and as MS may elude diagnosis, the association may be

underrecognized. We would encourage others to report the coexistence

of these diseases. A confirmed association might provide insight into

the etiopathogenesis of these conditions.

JOHN B. GROSS, JR., MD

Division of Gastroenterology and Hepatology

NEERAJ KUMAR, MD

Department of Neurology

Mayo Clinic and Foundation, Rochester, Minnesota

Mayo Clinic

West 19

200 First Street SW

Rochester, MN 55905

REFERENCES

1. Taub WH, Lederman RJ, Tuthill RJ, Falk GW: Primary biliary

cirrhosis in a patient with multiple sclerosis. Am J Gastroenterol

84:415-417, 1989

2. Noseworthy JH, Ebers GC: Primary biliary cirrhosis and multiple

sclerosis. Am J Gastroenterol 84:1584-1585, 1989

3. Pontecorvo MJ, Levinson JD, Roth JA: A patient with primary

biliary cirrhosis and multiple sclerosis. Am J Med 92:433-436, 1992

4. Lee Y-M, Kaplan MM: Primary sclerosing cholangitis. N Engl J Med

332:924-933, 1995

5. Rang EH, BN, Hermon- J: Association of ulcerative

colitis with multiple sclerosis. Lancet 2:555, 1982

6. Geissler A, Andus T, Roth M,Kullmann F, Caesar I, Held P, Gross V,

Feuerbach S, Scholmerich J: Focal white-matter lesions in the brain

of patients with inflammatory bowel disease. Lancet 345:897-889, 1995

7. Agranoff D, Schon F: Are focal white matter lesions in patients

with inflammatory bowel disease linked to multiple sclerosis? Lancet

316:190-191, 1995

8. Sadovnick AD, Paty DW, Yannakoulias G: Concurrence of multiple

sclerosis and inflammatory bowel disease. N Engl J Med 321:762-763,

1989

_____________

Best regards,

Dave

(father of (20); PSC 07/03; UC 08/03)