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Inflammatory Demyelination
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Cerebrospinal fluid (CSF) shows elevated levels of proteins with normal cell counts. Nerve conduction test (NCT) may show subtle changes of demyelinating pattern. Although the axons are always preserved, two variants of GBS are associated with axonal degeneration: acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN). Sensory action potential is usually normal in AMAN compared to AMSAN, which has absent sensory action potential.
AI and Autoimmunity
Published in Louis J. Catania, AI for Immunology, 2021
Guillain-Barré syndrome (GBS): GBS includes acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and pharyngeal-cervical-brachial weakness. Newly developed technologies, including metabolite analysis, peripheral nerve ultrasound, and feature selection via AI are facilitating more accurate diagnosis of axonal GBS. Nevertheless, some key issues, such as genetic susceptibilities, remain unanswered and moreover, current therapies bear limitations.30
Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (demyelinating form) accounts for about 90% of North American and European cases; 5–10% of cases constitute an axonal subtype, acute motor axonal neuropathy (AMAN).
Reversible sub-acute motor neuron syndrome after mushroom intoxication masquerading as amyotrophic lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2022
Emmeline Lagrange, Elisa de la Cruz, Florence Esselin, Jean-Paul Vernoux, Nicolas Pageot, Guillaume Taieb, William Camu
The initial diagnosis of ALS was clinically and electromyographically documented with rapidly evolving upper and lower motor neuron signs together with generalized severe and active denervation. However, some atypical features may be underlined. First, the onset was sub-acute and initially preceded by nausea, vomiting and vertigo, which could suggest a recent episode of intoxication. Even though some ALS cases have been occasionally described with apparently acute or sub-acute respiratory or bulbar symptoms, this is not usual (7). Secondly, there was mild sensory nerve involvement on ENMG, even if clinically, there was no sensory complaint nor signs of sensory involvement. Sensory nerve involvement on ENMG has already been described in some ALS cases, but, added to the sub-acute onset, it is an additional point to suggest that this “ALS case” is unusual (8). Acute of sub-acute motor deficits may also suggest a diagnosis of acute motor axonal neuropathy. However, in the present case, upper motor neuron signs as well as normal motor neurography at ENMG did not support this diagnosis. Thirdly, the patient presented with dyspnea but spirometry as well as chest CT-scan were normal. Such dyspnea may be attributed to the vocal cord paresis initially found and the dyspnea was considered of laryngeal origin, making it likely that this symptom may not solely be attributed to the motor neuron involvement.
A case of Guillain-Barré syndrome related to COVID-19 infection
Published in International Journal of Neuroscience, 2023
Ülkü Türk Börü, Cansu Köseoğlu Toksoy, Cem Bölük, Hayri Demirbaş, Ahmet Çağdaş Yılmaz
In a case published shortly before our case, on autonomic dysfunction preceding acute motor axonal neuropathy, we can see that identical to our case, there is pure motor involvement and that motor weakness develops after autonomic involvement. However, in their case, it meets the criteria for electrophysiology. It has been evaluated as AMAN associated with COVID-19 [13].
Mechanical filtration of the cerebrospinal fluid: procedures, systems, and applications
Published in Expert Review of Medical Devices, 2023
The most frequent APRM is Guillain–Barré syndrome (GBS), where sensory and motor forms can be differentiated. Acute motor axonal neuropathy usually presents with rapidly developing paralysis. The progressive paralysis may reach ventilatory muscles, leading to respiratory failure requiring tracheal intubation and artificial ventilation. Eventually, there may be total paralysis of all voluntary muscles of the body, including the muscles innervated by the cranial nerves. The associated bulbar weakness and autonomic instability reinforce the need for control of the airway and ventilation. Early and accurate detection is critical to achieve prompt initiation of therapies as this is the main factor determining outcomes [28]. While immunotherapies (particularly iv polyclonal immunoglobulins) are the most commonly used therapies, plasma exchange (PE) is a highly efficient technique to rapidly remove circulating autoantibodies and other humoral factors from the vascular compartment [28]. Liquorpheresis was proposed as an experimental therapy as it eliminates ‘blocking factors’ from the CSF, as was shown by infusing CSF from patients treated with CSF filtration in the sciatic nerve of rats. CSF pre-therapy induced a more severe impairment of nerve conduction than CSF after-therapy [29]. In humans, short series of cases and a clinical trial were published between 1990 and 2005, showing that CSF filtration was generally safe and partially effective, yet no further studies were reported thereafter [30–33]. In the clinical trial, although the number of subjects was small (37 patients with acute GBS randomized to receive either CSF filtration or PE), the authors found that CSF filtration was at least as effective as PE [33]. Neuropathies in the context of systemic autoimmune diseases largely share the autoimmune pathophysiology of autoimmune neurological neuropathies. A patient with systemic lupus erythematosus complicated with limb paresis due to acute demyelinating neuropathy was treated with conventional therapy with intravenous immunoglobulins and immunoadsorption complemented by pulse methylprednisolone and cyclophosphamide, with no improvement. The institution of CSF filtration was reported to result in a rapid improvement of the paresis [34].