Peripheral Neuropathy in Behçet's Disease: Clinical and Neurophysiological Study

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.

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Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

Tulbă

Popescu

Manole

et al. 2021

Front. Pharmacol.

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Autonomic function assessment in Egyptian patients with Behçet's disease

Ali

Talaat

Nazer

et al. 2021

Al-Azhar Assiut Medical Journal

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Background Behçet's disease (BD) is considered a chronic multisystem disorder, where neurological manifestations are common. There is scarcity of investigations that have assessed the autonomic nervous system dysfunction in BD, with conflicting results. Objectives The current study aimed to identify presence of autonomic nervous system dysfunction using neurophysiological assessment in BD and to investigate the relationship between the indicators of autonomic function and the disease activity parameters. Patients and methods The study involved 30 patients with BD in accordance with the Universal Criteria for Behçet's Diseases and 25 controls, who were subjected to clinical evaluation and Palmar sympathetic skin response (SSR). Results Patients with BD had a significant difference in SSR (P<0.001) compared with controls. Moreover, a significant positive association was found among neuropathic pain and disease activity parameters. Conclusion The study confirms the presence of sympathetic autonomic dysfunction in BD, which related to disease activity using a simple noninvasive test such as SSR.

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Peripheral Neuropathy in Behçet's Disease: Clinical and Neurophysiological Study

Cited by 2 publications

References 12 publications

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

Autonomic function assessment in Egyptian patients with Behçet's disease

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