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- CLINICA NEUROL, 42: 216－220, 2002
- Vol.42 No.3 contents
Case Report
A patient with primary Sjö gren's syndrome featuring polyneuropathy and oculomotor paralysis and associated with asymptomatic left middle cerebral artery stenosis
Chie Yanagihara, M. D. , Kayoko Nakaji, M. D. and Yo Nishimura, M. D.
Department of Neurology, Nishi-Kobe Medical Center
We report a case of primary Sjö gren's syndrome (primary SjS) with polyneuropathy and right oculomotor paralysis associated with middle cerebral artery stenosis. A 39-year-old woman developed progressive numbness and clumsiness of the limbs. Two months later, right third cranial palsy manifested itself and she was admitted to our hospital. A cranial MRA showed left middle cerebral artery stenosis confirmed by transcranial color doppler sonography. A nerve conduction study showed a decrease in the NCV and reduced CMAP, while sural nerve biopsy showed axonal degeneration and infiltration of inflammatory cells around the small blood vessel walls. The patient complained of dry mouth and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to the diagnosis of Sjö gren's syndrome.
Reports of primary SjS with involvement of large cerebral arteries are rare. In our case, polyneuropathy and oculomotor paralysis were the manifest symptoms, but middle cerebral artery stenosis was also observed. This indicates that, even in the absence of CNS symptoms, cerebral artery involvement may be present in primary SjS.
(CLINICA NEUROL, 42: 216－220, 2002)
key words: primary Sjögren's syndrome, polyneuropathy, oculomotor paralysis, middle cerebral artery stenosis
(Received: 17-Dec-01)
