Sensory neuropathy as the initial manifestation of primary biliary cirrhosis.

Illa, Isabel; Graus, Francesc; Ferrer, Isidre; Enríquez, Jaime

doi:10.1136/jnnp.52.11.1307

Cited by 15 publications

(5 citation statements)

References 5 publications

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“…In patients with primary biliary cirrhosis, a distinct type of sensory neuropathy has been reported. 39,40 In agreement with these reports, three of the five patients with primary biliary cirrhosis in our study had abnormalities only detectable by QST. Only one patient was symptomatic with paresthesias and was defined to have a neuropathy.…”

Section: Discussionsupporting

confidence: 92%

Autonomic and peripheral (sensorimotor) neuropathy in chronic liver disease: A clinical and electrophysiologic study

Chaudhry¹,

Corse²,

O'Brian³

et al. 1999

Hepatology

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Peripheral neuropathy has been reported in association with chronic liver disease. However, the precise incidence, severity and characteristics of neuropathy, and the relationship of neuropathy to different etiologies of liver disease have not been defined. In this study, 58 patients with advanced liver disease were evaluated in detail for the presence of neuropathy. Peripheral (sensorimotor) neuropathy was found in 71% and autonomic neuropathy was found in 48% of the patients. Although the majority of patients were asymptomatic, neurological examination showed distal sensory loss to pain, or vibration or distal loss of reflexes in 17 patients (29%). Sensory neuropathy was seen more commonly than motor axonal polyneuropathy on nerve conduction studies. Quantitative sensory testing was frequently abnormal (62%) and cooling thresholds were more affected than vibration thresholds. Overall, the pattern of neuropathy in patients with liver disease conformed to the pattern expected in ''dying back'' or length-dependent neuropathy. The neuropathy was most severe in patients with advanced hepatic decompensation. Comparison of causes of liver disease showed no significant differences in the severity of neuropathy among the different etiologies. In conclusion, axonal sensory-motor polyneuropathy and autonomic neuropathy are commonly seen in patients with end-stage liver disease of different causes. (HEPATOLOGY 1999;29:1698-1703

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Section: Discussionsupporting

confidence: 92%

Autonomic and peripheral (sensorimotor) neuropathy in chronic liver disease: A clinical and electrophysiologic study

Chaudhry¹,

Corse²,

O'Brian³

et al. 1999

Hepatology

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“…24,39,77,84,145 The neuropathy described with hepatic disease was generally mild or subclinical, with both sensory and motor deficits, 24,39,77,84,145 although in primary biliary cirrhosis a sensory neuropathy has been reported. 25,70 In a prospective series of 58 patients with end-stage liver disease, peripheral neuropathy was present in 71% of patients. 26 Examination showed distal sensory and reflex loss consistent with a lengthdependent neuropathy.…”

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confidence: 99%

Neuromuscular diseases and disorders of the alimentary system

2002

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This review outlines the relationship and interaction between neuromuscular diseases and disorders of the alimentary system. Neuromuscular manifestations of gastrointestinal and hepatobiliary diseases are first considered. Such diseases may cause neuromuscular disorders by leading to nutritional deficiency or by more direct mechanisms. The pathogenesis, clinical features, and treatment of these various neuromuscular manifestations are discussed. The impact of disorders of nerve, neuromuscular transmission, and muscle on the alimentary system is then reviewed. The main sequelae are impaired deglutition and gastrointestinal dysmotility. The management of these complications is considered.

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“… 29 PBC is associated with axonal degenerative neuropathy with sensory predominance. 30 - 32 Nevertheless, a small number of PBC cases positive for antimitochondrial antibodies and complicated by CIDP have also been reported. 33 The expression of MHC and B7 family of costimulatory molecules is observed in the peripheral nerve myelinated fibers of patients with CIDP and in the bile duct epithelial cells of patients with PBC.…”

Section: Discussionmentioning

confidence: 99%

Autoantibodies Against Dihydrolipoamide S-Acetyltransferase in Immune-Mediated Neuropathies

Fukami,

Iijima,

Koike

et al. 2024

Neurol Neuroimmunol Neuroinflamm

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Background and Objectives This study aimed to identify disease-related autoantibodies in the serum of patients with immune-mediated neuropathies including chronic inflammatory demyelinating polyneuropathy (CIDP) and to investigate the clinical characteristics of patients with these antibodies. Methods Proteins extracted from mouse brain tissue were used to react with sera from patients with CIDP by western blotting (WB) to determine the presence of common bands. Positive bands were then identified by mass spectrometry and confirmed for reactivity with patient sera using enzyme-linked immunosorbent assay (ELISA) and WB. Reactivity was further confirmed by cell-based and tissue-based indirect immunofluorescence assays. The clinical characteristics of patients with candidate autoantibody-positive CIDP were analyzed, and their association with other neurologic diseases was also investigated. Results Screening of 78 CIDP patient sera by WB revealed a positive band around 60–70 kDa identified as dihydrolipoamide S-acetyltransferase (DLAT) by immunoprecipitation and mass spectrometry. Serum immunoglobulin G (IgG) and IgM antibodies' reactivity to recombinant DLAT was confirmed using ELISA and WB. A relatively high reactivity was observed in 29 of 160 (18%) patients with CIDP, followed by patients with sensory neuropathy (6/58, 10%) and patients with MS (2/47, 4%), but not in patients with Guillain-Barré syndrome (0/27), patients with hereditary neuropathy (0/40), and healthy controls (0/26). Both the cell-based and tissue-based assays confirmed reactivity in 26 of 33 patients with CIDP. Comparing the clinical characteristics of patients with CIDP with anti-DLAT antibodies (n = 29) with those of negative cases (n = 131), a higher percentage of patients had comorbid sensory ataxia (69% vs 37%), cranial nerve disorders (24% vs 9%), and malignancy (20% vs 5%). A high DLAT expression was observed in human autopsy dorsal root ganglia, confirming the reactivity of patient serum with mouse dorsal root ganglion cells. Discussion Reactivity to DLAT was confirmed in patient sera, mainly in patients with CIDP. DLAT is highly expressed in the dorsal root ganglion cells, and anti-DLAT antibody may serve as a biomarker for sensory-dominant neuropathies.

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Sensory neuropathy as the initial manifestation of primary biliary cirrhosis.

Cited by 15 publications

References 5 publications

Autonomic and peripheral (sensorimotor) neuropathy in chronic liver disease: A clinical and electrophysiologic study

Autonomic and peripheral (sensorimotor) neuropathy in chronic liver disease: A clinical and electrophysiologic study

Neuromuscular diseases and disorders of the alimentary system

Autoantibodies Against Dihydrolipoamide S-Acetyltransferase in Immune-Mediated Neuropathies

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