Academic literature on the topic 'Acute motor and sensory axonal neuropathy (AMSAN)'

Abstract Peripheral neuropathy describes the clinical syndrome of weakness, sensory loss, diminished tendon reflexes, or some combination of these findings caused by lesions of peripheral nerves. It may symmetrically involve the distal extremities, usually lower extremity greater than upper extremity (distal sensory, motor, or sensorimotor polyneuropathy), or may involve one or more named nerves (mononeuritis or mononeuritis multiplex). Peripheral neuropathy can also involve nerve roots (polyradiculopathy). From a pathophysiologic perspective, peripheral neuropathies are commonly divided into axonal and demyelinating types. Axonal neuropathy primarily destroys the axon, with secondary loss of the myelin sheath. Nerve conduction velocities are commonly normal with axonal neuropathy, but the sensory or motor amplitudes are low. Demyelinating neuropathy is associated with loss of the myelin sheath but preservation of the axon. Nerve conduction velocities are slowed, but the amplitudes are normal with this type of neuropathy. From a clinical standpoint, four main types of peripheral neuropathy occur among HIV-infected persons: (1) distal sensory polyneuropathy (DSPN), (2) acute and chronic inflammatory demyelinating polyneuropathy (AIDP and CIDP), (3) mononeuritis or mononeuritis multiplex (MM), and (4) lumbosacral polyradiculopathy (LSPR). With HIV-infected patients, the type of neuropathy depends in part on the degree of their immunodeficiency. Patients with AIDP and CIDP more often have early stage HIV disease. Distal sensory neuropathy, the most common form of neuropathy, generally develops in patients with more advanced, or late stage HIV disease. Similarly, LSPR predominantly occurs in patients who have progressed to AIDS. Mononeuritis or limited MM can develop in early stage HIV disease; extensive neuritis is seen in late stage HIV disease and may resemble CIDP.

Abstract This chapter describes the pathologic findings in patients with peripheral neuropathy, with a focus on the nerve biopsy. The most current clinical indications for nerve biopsy and the advanced techniques used to evaluate peripheral nerve biopsies are reviewed. Normal peripheral nerve is compared to general reactions of peripheral nerve to injury (primary axonal degeneration, primary segmental demyelination, and paranodopathy). Specific neuropathies are divided into acquired peripheral neuropathy and inherited disorders. The classification of vasculitic neuropathies has been recently updated and is compared to immune-mediated neuropathies, both acute Guillain–Barré syndrome and more chronic immune-mediated peripheral neuropathies. Neuropathies occurring secondary to infections, hematologic diseases, and neoplasms are reviewed. Metabolic disease (e.g., diabetic) and toxic exposures are also common causes of neuropathy. Advances in molecular genetics have greatly enhanced the understanding of hereditary neuropathies, including the hereditary motor and sensory neuropathies, sensory and autonomic neuropathies, and familial amyloid polyneuropathies.

A 60-year-old man sought care for painless, symmetric, ascending weakness and sensory loss affecting the lower, greater than upper, extremities, progressing over 3 weeks with associated orthostatism. He was diffusely areflexic and had symmetric weakness, distal greater than proximal, with normal bulbar strength. Muscle atrophy was not appreciated, and fasciculations were absent. Sensory examination revealed pan-sensory loss at the feet and hands. His gait was unsteady with prominent steppage. He was unable to climb stairs, kneel, or arise without assistance. Pertinent medical and social history included a spinal fusion at C5-T1, a 10-pack-year smoking history, and congestive heart failure, New York Heart Association class III with an intracardiac defibrillator for ventricular fibrillation, and taking carvedilol and furosemide. Needle electromyography and nerve conduction studies showed a severe axonal sensory-motor polyneuropathy with proximal involvement, suggesting polyradicular colocalization. Cerebrospinal fluid obtained during unremarkable spinal myelography, for exclusion of spinal compression, showed normal and abnormal findings: total nucleated cells, 3/µL; glucose, 87 mg/dL; and protein, 326 mg/dL. Sural nerve biopsy showed marked active axonal injury without significant inflammatory infiltrates. Expanded autoimmune neuroimmunologic testing by indirect immunofluorescence staining identified the classic pattern for antineuronal nuclear antibody type 1 –immunoglobulin G. Chest radiography and computed tomography showed a consolidation and volume loss in the left lower lobe without identifiable mass. The patient was diagnosed with paraneoplastic axonal sensory-motor polyneuropathy in the setting of antineuronal nuclear antibody type 1-immunoglobulin G positivity and likely small cell lung carcinoma. Acute motor axonal neuropathy was thought to be the diagnosis, and the patient was treated with plasma exchange. He continued to worsen and was transferred to the intensive care unit with new shortness of breath. Escalated therapy with intravenous immunoglobulin did not help. He had development of urinary retention, bulbar weakness, confusion, and flail limbs in all extremities. On identification of antineuronal nuclear antibody type 1-immunoglobulin G, he was treated with intravenous methylprednisolone, but his condition worsened. The patient and his family opted for comfort measures. His defibrillator was turned off, and he died 20 days after first coming to the hospital. At autopsy, small cell lung carcinoma of the left lung was identified without bronchial mass or metastasis. Neural tissues had diffuse microglial activation, with scattered microglial nodules and prominent perivascular chronic lymphocytic infiltrates. Antineuronal nuclear antibody type 1-immunoglobulin G autoimmunity was first reported in 1965. Patients had sensory neuropathy with nonmetastatic cancer and dorsal ganglia degeneration at autopsy. Neuropathy is the most common neurologic presentation, but the neurologic phenotypes have expanded since the original descriptions to include cerebellar, cognitive, and spinal cord involvement.

Background: GBS is an acute inflammatory polyneuropathy resulting from an immune response after infection. Characterized as an ascetic, progressive, selflimiting flaccid tetraparesis. It has several phenotypic presentations, which one is AMAN. The treatment’s based on use of intravenous immunoglobulin (IGIV) and plasmapheresis (PLEX). Methods: A literature review of the PubMed and UpToDate databases using descriptors “GBS” and “AMAN” between 2014-2020. Objectives: Report a case of GBS, addressing AMAN variant; a literature review with therapeutic and diagnostic possibilities. Case report: DTS,

Guillain-Barré Syndrome (GBS) is a rare disease. Its classic presentation is an acute tetraparesis and absence or diminished tendon reflex. Clinical variants such as Pharyngeal-cervical-brachial (PCB) and Bickerstaff brainstem encephalitis (BBE) can occur. We report a case of PCB and BBE overlap, with electrophysiological pattern of acute motor sensory axonal neuropathy. A 36-year-old man with recent dengue virus infection, was evaluated in the emergency department, complaining of double vision and eyelid drop for one week. Neurological examination showed palpebral ptosis, dysarthria, cervical