Einsiedel, H. von

Hamann, Gerhard F.

Sander, D.

Zachoval, R.

English

G.F. Hamann

R. Zachoval

H. von Einsiedel

D. Sander

Crossref

Hepatitis-C-Associated Multiple Arterial Dissection of Cerebral Vessels

Open Access LMU ( Ludwig-Maximilians-Univ. München)

180 Short ReportsEur Neurol 2003;49:180–182DOI: 10.1159/000069072Hepatitis-C-Associated Multiple ArterialDissection of Cerebral VesselsG.F. Hamann a, R. Zachoval b, H. von Einsiedel c, D. Sander dDepartments of a Neurology and b Internal Medicine II,Klinikum Grosshadern, Ludwig Maximilian University, andDepartments of c Radiology and d Neurology, TechnicalUniversity, Munich, GermanyNeurological complications in hepatitis are common, and in mostcases a polyneuropathy is described [1]. Cerebrovascular complica-tions are rare but mostly seen in association with a systemic vasculitisor a cryoglobulinemia [2–4]. We describe an unusual case of cerebro-vascular findings associated with hepatitis C.Case ReportA 48-year-old woman was admitted with right facial pain and anMRI suggestive of a right internal carotid artery (ICA) dissection. Nofurther neurological deficit was noted but a right Horner syndrome.The patient had no vascular risk factors besides hypercholesterinem-ia. The family history did not evidence any stroke. Next day, shenoticed left neck pain. Ultrasound revealed new dissections of theShort Reports 181Fig. 1. a, b Angiography of the right and left ICAshowing the dissection on both sides (black ar-rows). c Color-coded duplex sonography of theright vertebral artery, showing dissection (whitearrow). d MRI (T1-weighted, fat suppressed)showing the dissection of the right vertebral artery(white arrow) and semilunar hematoma of thevessel wall. e MRI (T1-weighted, fat suppressed)showing the dissections of both internal arteries(black arrows) and semilunar hematomas of thevessel walls.182 Short Reportsleft ICA and the right vertebral artery. Angiography proved thearterial dissections in the three major arteries supplying the brain(fig. 1). No signs of vasculitis, fibromuscular dysplasia or other vesselwall diseases were found; there was no history of neck manipulationor trauma. Possible causes like rheumatic, connective tissue, coagula-tion, inflammatory and autoimmune disorders were ruled out. Twoweeks later, an axonal polyneuropathy (distal, symmetrical) wasfound and confirmed by nerve conduction studies and electromyog-raphy. Although she had never had an icterus nor a blood transfusionand no clinical evidence of hepatitis, anti-HCV antibodies were posi-tive (ELISA). HCV RNA revealed 130,000 IU/ml; the HCV geno-type was 2a (Simmonds). The transaminases were elevated (ALT 40U/l, AST 27 U/l). Cryoglobulins were elevated with 10 g/l in serum.The liver biopsy showed lymphocytic, follicular infiltration but nonecrosis, cirrhosis or cholestasis. The patient was put on interferon·2b, 3 MU s.c. thrice weekly, and ribaverin, 800 mg/day, for 6months. ALT and AST rapidly normalized and HCV RNA becamenegative. Six months later, these promising results still hold. Thepolyneuropathy stabilized, both clinically and by nerve conductionstudies and electromyography. Using ultrasound after 18 months, allvessels were normal without signs of dissection or stenosis.DiscussionWe report the very first case of arterial dissections of brain-sup-plying vessels associated with hepatitis C. Previous reports on extra-hepatic manifestations of hepatitis C [1] focus on glomerulonephri-tis, porphyria cutanea tarda, Sjögren’s syndrome and cryoglobuli-nemia. The polyneuropathy is the most prominent neurological find-ing in hepatitis C (10–20% of all patients). Involvement of the centralnervous system is thought to be caused by cerebral vasculitis [2]. Thecombination of hepatitis C and cryoglobulinemia is responsible forvarious extrahepatic complications [1, 2]. The hepatitis-B-associatedpolyarteritis nodosa is prone to cerebrovascular complications main-ly by a central nervous system vasculitis and by dissections of theaorta [1, 3, 4] with a vasculitis of the vasa vasorum. So far, only a caseof coronary dissection associated with hepatitis C has been described[5]. Since in rare instances polyarteritis nodosa may occur in hepati-tis C, a similar pathophysiology can be hypothesized. Unfortunately,we can only report the association between the observed multiplecervical artery dissections and the hepatitis C. This association is notdefined as a causal relationship. The link between the two findings ismainly given by the tight temporal relationship. Further support forthe association might be seen in the complete recovery from the dis-section following treatment of the hepatitis C and the lack of anyfurther dissections since the hepatitis was under control. In accor-dance with previous findings, our patient had no clinical signs ofbrain infarction [6]. Patients with Horner syndrome and lack ofartery occlusion are significantly less endangered to develop ischemicsymptoms [6].Two central issues emerge from our case: when developing isch-emic symptoms, hepatitis C patients should be screened for arterialdissections, and patients with dissections should be tested for hepati-tis C when no other apparent reasons can be found.References1 Willson RA: Extrahepatic manifestations of chronic viral hepatitis. Am JGastroenterol 1997;92:4–17.2 Heckmann JG, Kayser C, Heuss D, Manger B, Blum HE, Neundörfer B:Neurological manifestations of chronic hepatitis C. J Neurol 1999;246:486–491.3 Iino T, Eguchi K, Sakai M, Ishijima M, Toriyama K: Polyarteriitis nodosawith aortic dissection: Necrotizing vasculitis of the vasa vasorum. J Rheu-matol 1992;19:1632–1636.4 Petty GW, Duffy J, Huston J: Cerebral ischemia in patients with hepatitisC virus infection and mixed cryoglobulinemia. Mayo Clin Proc 1996;71:671–678.5 Fernandez-Gutierrez B, Zamorano J, Battle E, Alfonso F, Conde A, San-chez-Harguindey L, Jover JA: Coronary dissection associated with hepati-tis C virus-related cryoglobulinaemia. Rheumatology 1999;38:1299–1301.6 Baumgartner RW, Arnold M, Baumgartner I, Mosso M, Gonner F, StuderA, Schroth G, Schuknecht B, Sturzenegger M: Carotid dissection with andwithout ischemic events: Local symptoms and cerebral artery findings.Neurology 2001;57:827–832.Prof. Gerhard Hamann, Department of NeurologyLudwig Maximilian University, Klinikum GrosshadernMarchioninistrasse 15, D–81377 München (Germany)Tel. +49 89 70 95 36 70, Fax +49 89 70 95 36 77E-Mail hamann@brain.nefo.med.uni-muenchen.de

Carotid dissection with and without ischemic events: Local symptoms and cerebral artery findings. Neurology 2001;57:827–832.

Cerebral ischemia in patients with hepatitis C virus infection and mixed cryoglobulinemia. Mayo Clin Proc 1996;71:

Extrahepatic manifestations of chronic viral hepatitis.

JA: Coronary dissection associated with hepatitis C virus-related cryoglobulinaemia. Rheumatology

Toriyama K: Polyarteriitis nodosa with aortic dissection: Necrotizing vasculitis of the vasa vasorum.

Hepatitis-C-associated multiple arterial dissection of cerebral vessels

Universität München: Elektronischen Publikationen

180 Short Reports
Eur Neurol 2003;49:180–182
DOI: 10.1159/000069072
Hepatitis-C-Associated Multiple Arterial
Dissection of Cerebral Vessels
G.F. Hamann a, R. Zachoval b, H. von Einsiedel c, D. Sander d
Departments of a Neurology and b Internal Medicine II,
Klinikum Grosshadern, Ludwig Maximilian University, and
Departments of c Radiology and d Neurology, Technical
University, Munich, Germany
Neurological complications in hepatitis are common, and in most
cases a polyneuropathy is described [1]. Cerebrovascular complica-
tions are rare but mostly seen in association with a systemic vasculitis
or a cryoglobulinemia [2–4]. We describe an unusual case of cerebro-
vascular findings associated with hepatitis C.
Case Report
A 48-year-old woman was admitted with right facial pain and an
MRI suggestive of a right internal carotid artery (ICA) dissection. No
further neurological deficit was noted but a right Horner syndrome.
The patient had no vascular risk factors besides hypercholesterinem-
ia. The family history did not evidence any stroke. Next day, she
noticed left neck pain. Ultrasound revealed new dissections of the
Short Reports 181
Fig. 1. a, b Angiography of the right and left ICA
showing the dissection on both sides (black ar-
rows). c Color-coded duplex sonography of the
right vertebral artery, showing dissection (white
arrow). d MRI (T1-weighted, fat suppressed)
showing the dissection of the right vertebral artery
(white arrow) and semilunar hematoma of the
vessel wall. e MRI (T1-weighted, fat suppressed)
showing the dissections of both internal arteries
(black arrows) and semilunar hematomas of the
vessel walls.
182 Short Reports
left ICA and the right vertebral artery. Angiography proved the
arterial dissections in the three major arteries supplying the brain
(fig. 1). No signs of vasculitis, fibromuscular dysplasia or other vessel
wall diseases were found; there was no history of neck manipulation
or trauma. Possible causes like rheumatic, connective tissue, coagula-
tion, inflammatory and autoimmune disorders were ruled out. Two
weeks later, an axonal polyneuropathy (distal, symmetrical) was
found and confirmed by nerve conduction studies and electromyog-
raphy. Although she had never had an icterus nor a blood transfusion
and no clinical evidence of hepatitis, anti-HCV antibodies were posi-
tive (ELISA). HCV RNA revealed 130,000 IU/ml; the HCV geno-
type was 2a (Simmonds). The transaminases were elevated (ALT 40
U/l, AST 27 U/l). Cryoglobulins were elevated with 10 g/l in serum.
The liver biopsy showed lymphocytic, follicular infiltration but no
necrosis, cirrhosis or cholestasis. The patient was put on interferon
·2b, 3 MU s.c. thrice weekly, and ribaverin, 800 mg/day, for 6
months. ALT and AST rapidly normalized and HCV RNA became
negative. Six months later, these promising results still hold. The
polyneuropathy stabilized, both clinically and by nerve conduction
studies and electromyography. Using ultrasound after 18 months, all
vessels were normal without signs of dissection or stenosis.
Discussion
We report the very first case of arterial dissections of brain-sup-
plying vessels associated with hepatitis C. Previous reports on extra-
hepatic manifestations of hepatitis C [1] focus on glomerulonephri-
tis, porphyria cutanea tarda, Sjögren’s syndrome and cryoglobuli-
nemia. The polyneuropathy is the most prominent neurological find-
ing in hepatitis C (10–20% of all patients). Involvement of the central
nervous system is thought to be caused by cerebral vasculitis [2]. The
combination of hepatitis C and cryoglobulinemia is responsible for
various extrahepatic complications [1, 2]. The hepatitis-B-associated
polyarteritis nodosa is prone to cerebrovascular complications main-
ly by a central nervous system vasculitis and by dissections of the
aorta [1, 3, 4] with a vasculitis of the vasa vasorum. So far, only a case
of coronary dissection associated with hepatitis C has been described
[5]. Since in rare instances polyarteritis nodosa may occur in hepati-
tis C, a similar pathophysiology can be hypothesized. Unfortunately,
we can only report the association between the observed multiple
cervical artery dissections and the hepatitis C. This association is not
defined as a causal relationship. The link between the two findings is
mainly given by the tight temporal relationship. Further support for
the association might be seen in the complete recovery from the dis-
section following treatment of the hepatitis C and the lack of any
further dissections since the hepatitis was under control. In accor-
dance with previous findings, our patient had no clinical signs of
brain infarction [6]. Patients with Horner syndrome and lack of
artery occlusion are significantly less endangered to develop ischemic
symptoms [6].
Two central issues emerge from our case: when developing isch-
emic symptoms, hepatitis C patients should be screened for arterial
dissections, and patients with dissections should be tested for hepati-
tis C when no other apparent reasons can be found.
References
1 Willson RA: Extrahepatic manifestations of chronic viral hepatitis. Am J
Gastroenterol 1997;92:4–17.
2 Heckmann JG, Kayser C, Heuss D, Manger B, Blum HE, Neundörfer B:
Neurological manifestations of chronic hepatitis C. J Neurol 1999;246:
486–491.
3 Iino T, Eguchi K, Sakai M, Ishijima M, Toriyama K: Polyarteriitis nodosa
with aortic dissection: Necrotizing vasculitis of the vasa vasorum. J Rheu-
matol 1992;19:1632–1636.
4 Petty GW, Duffy J, Huston J: Cerebral ischemia in patients with hepatitis
C virus infection and mixed cryoglobulinemia. Mayo Clin Proc 1996;71:
671–678.
5 Fernandez-Gutierrez B, Zamorano J, Battle E, Alfonso F, Conde A, San-
chez-Harguindey L, Jover JA: Coronary dissection associated with hepati-
tis C virus-related cryoglobulinaemia. Rheumatology 1999;38:1299–1301.
6 Baumgartner RW, Arnold M, Baumgartner I, Mosso M, Gonner F, Studer
A, Schroth G, Schuknecht B, Sturzenegger M: Carotid dissection with and
without ischemic events: Local symptoms and cerebral artery findings.
Neurology 2001;57:827–832.
Prof. Gerhard Hamann, Department of Neurology
Ludwig Maximilian University, Klinikum Grosshadern
Marchioninistrasse 15, D–81377 München (Germany)
Tel. +49 89 70 95 36 70, Fax +49 89 70 95 36 77
E-Mail hamann@brain.nefo.med.uni-muenchen.de


https://epub.ub.uni-muenchen.de/16806/1/10_1159_000069072.pdf

Hepatitis-C-associated multiple arterial dissection of cerebral vessels

Abstract

Similar works

Full text

Available Versions

Crossref

Open Access LMU ( Ludwig-Maximilians-Univ. München)

Universität München: Elektronischen Publikationen