TY - JOUR
T1 - The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy
AU - Mori, Keiko
AU - Iijima, Masahiro
AU - Koike, Haruki
AU - Hattori, Naoki
AU - Tanaka, Fumiaki
AU - Watanabe, Hirohisa
AU - Katsuno, Masahisa
AU - Fujita, Asako
AU - Aiba, Ikuko
AU - Ogata, Akihiko
AU - Saito, Toyokazu
AU - Asakura, Kunihiko
AU - Yoshida, Mari
AU - Hirayama, Masaaki
AU - Sobue, Gen
N1 - Funding Information:
The authors are grateful to Dr Hayashi (Department of Oral Molecular Pathology, The University of Tokushima Graduate School of Dentistry, Japan) for providing us with alpha-fodrin vector, and information on alpha-fodrin. The authors would like to express their deep appreciation to Dr Tsutomu Yanagi (Department of Neurology, Nagoya Daini Red Cross Hospital, Japan), Dr Eiichiro Mukai (Department of Neurology, Nagoya Medical Center, Japan), Drs Kazuhiro Imamura and Yuichi Kawagashira (Department of Neurology, Okazaki City Hospital, Japan), Dr Shigehisa Mitake (Department of Neurology, Tosei General Hospital, Japan), Dr Kenji Mokuno (Department of Neurology, Toyohashi Municipal Hospital, Japan) and Dr Yukihiko Matsuoka (Department of Neurology, Higashi Nagoya Hospital, Japan) for their support in the clinical evaluation of patients and for referring the patients for this study. The authors also are grateful for the expert pathologic technical assistance given by Dr Nozomi Hishikawa and Ms Sugiko Yokoi (Department of Neurology, Nagoya University Graduate School of Medicine, Japan). This study was partly supported by the grants from the Ministry of Health, Labor, and Welfare, Japan.
PY - 2005/11
Y1 - 2005/11
N2 - We assessed the clinicopathological features of 92 patients with primary Sjögren's syndrome-associated neuropathy (76 women, 16 men, 54.7 years, age at onset). The majority of patients (93%) were diagnosed with Sjögren's syndrome after neuropathic symptoms appeared. We classified these patients into seven forms of neuropathy: sensory ataxic neuropathy (n = 36), painful sensory neuropathy without sensory ataxia (n = 18), multiple mononeuropathy (n = 11), multiple cranial neuropathy (n = 5), trigeminal neuropathy (n = 15), autonomic neuropathy (n = 3) and radiculoneuropathy (n = 4), based on the predominant neuropathic symptoms. Acute or subacute onset was seen more frequently in multiple mononeuropathy and multiple cranial neuropathy, whereas chronic progression was predominant in other forms of neuropathy. Sensory symptoms without substantial motor involvement were seen predominantly in sensory ataxic, painful sensory, trigeminal and autonomic neuropathy, although the affected sensory modalities and distribution pattern varied. In contrast, motor weakness and muscle atrophy were observed in multiple mononeuropathy, multiple cranial neuropathy and radiculoneuropathy. Autonomic symptoms were often seen in all forms of neuropathy. Abnormal pupils and orthostatic hypotension were particularly frequent in sensory ataxic, painful, trigeminal and autonomic neuropathy. Unelicited somatosensory evoked potentials and spinal cord posterior column abnormalities in MRI were observed in sensory ataxic, painful and autonomic neuropathy. Sural nerve biopsy specimens (n = 55) revealed variable degrees of axon loss. Predominantly large fibre loss was observed in sensory ataxic neuropathy, whereas predominantly small fibre loss occurred in painful sensory neuropathy. Angiitis and perivascular cell invasion were seen most frequently in multiple mononeuropathy, followed by sensory ataxic neuropathy. The autopsy findings of one patient with sensory ataxic neuropathy showed severe large sensory neuron loss paralleling to dorsal root and posterior column involvement of the spinal cord, and severe sympathetic neuron loss. Degrees of neuron loss in the dorsal and sympathetic ganglion corresponded to segmental distribution of sensory and sweating impairment. Multifocal T-cell invasion was seen in the dorsal root and sympathetic ganglion, perineurial space and vessel walls in the nerve trunks. Differential therapeutic responses for corticosteroids and IVIg were seen among the neuropathic forms. These clinicopathological observations suggest that sensory ataxic, painful and perhaps trigeminal neuropathy are related to ganglioneuronopathic process, whereas multiple mononeuropathy and multiple cranial neuropathy would be more closely associated with vasculitic process.
AB - We assessed the clinicopathological features of 92 patients with primary Sjögren's syndrome-associated neuropathy (76 women, 16 men, 54.7 years, age at onset). The majority of patients (93%) were diagnosed with Sjögren's syndrome after neuropathic symptoms appeared. We classified these patients into seven forms of neuropathy: sensory ataxic neuropathy (n = 36), painful sensory neuropathy without sensory ataxia (n = 18), multiple mononeuropathy (n = 11), multiple cranial neuropathy (n = 5), trigeminal neuropathy (n = 15), autonomic neuropathy (n = 3) and radiculoneuropathy (n = 4), based on the predominant neuropathic symptoms. Acute or subacute onset was seen more frequently in multiple mononeuropathy and multiple cranial neuropathy, whereas chronic progression was predominant in other forms of neuropathy. Sensory symptoms without substantial motor involvement were seen predominantly in sensory ataxic, painful sensory, trigeminal and autonomic neuropathy, although the affected sensory modalities and distribution pattern varied. In contrast, motor weakness and muscle atrophy were observed in multiple mononeuropathy, multiple cranial neuropathy and radiculoneuropathy. Autonomic symptoms were often seen in all forms of neuropathy. Abnormal pupils and orthostatic hypotension were particularly frequent in sensory ataxic, painful, trigeminal and autonomic neuropathy. Unelicited somatosensory evoked potentials and spinal cord posterior column abnormalities in MRI were observed in sensory ataxic, painful and autonomic neuropathy. Sural nerve biopsy specimens (n = 55) revealed variable degrees of axon loss. Predominantly large fibre loss was observed in sensory ataxic neuropathy, whereas predominantly small fibre loss occurred in painful sensory neuropathy. Angiitis and perivascular cell invasion were seen most frequently in multiple mononeuropathy, followed by sensory ataxic neuropathy. The autopsy findings of one patient with sensory ataxic neuropathy showed severe large sensory neuron loss paralleling to dorsal root and posterior column involvement of the spinal cord, and severe sympathetic neuron loss. Degrees of neuron loss in the dorsal and sympathetic ganglion corresponded to segmental distribution of sensory and sweating impairment. Multifocal T-cell invasion was seen in the dorsal root and sympathetic ganglion, perineurial space and vessel walls in the nerve trunks. Differential therapeutic responses for corticosteroids and IVIg were seen among the neuropathic forms. These clinicopathological observations suggest that sensory ataxic, painful and perhaps trigeminal neuropathy are related to ganglioneuronopathic process, whereas multiple mononeuropathy and multiple cranial neuropathy would be more closely associated with vasculitic process.
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U2 - 10.1093/brain/awh605
DO - 10.1093/brain/awh605
M3 - Article
C2 - 16049042
AN - SCOPUS:27644591230
SN - 0006-8950
VL - 128
SP - 2518
EP - 2534
JO - Brain
JF - Brain
IS - 11
ER -