Differential Recovery in Cardiac and Vasomotor Sympathetic Functional Markers in a Patient with Acute Autonomic Sensory and Motor Neuropathy

Ohyama, Ken; Yasui, Kohichiroh; Hasegawa, Yasuhiro; Morozumi, Saori; Koike, Haruki; Sobue, Gen

doi:10.2169/internalmedicine.52.8787

Cited by 5 publications

(2 citation statements)

References 27 publications

(37 reference statements)

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“…The significant drop in blood pressure upon standing, low or undetectable plasma norepinephrine levels, preserved heart rate response to standing and reduced blood pressure recovery during phase IV of the Valsalva maneuver indicate damage to postganglionic sympathetic adrenergic fibers innervating the blood vessels, with relative preservation of the sympathetic innervation to the heart, which has been previously reported. 61 Pathological studies show massive loss of neurons in sympathetic and parasympathetic ganglia and sural nerve, 7,22 which underlies the severity of the symptoms. In some cases, preganglionic fibers are also affected.…”

Section: Autonomic Function Testingmentioning

confidence: 99%

Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia

Gutiérrez

Palma

2020

Semin Neurol

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Acute-onset and severe sensory and autonomic deficits with no motor dysfunction, typically preceded by a febrile illness, with poor recovery, and often fatal outcome are the hallmark features of acute sensory and autonomic neuronopathy (ASANN). Pathologically and electrophysiologically, ASANN is characterized by an extensive ganglionopathy affecting sensory and autonomic ganglia with preservation of motor neurons. Consequently, patients, usually children or young adult, develop acute-onset profound widespread loss of all sensory modalities resulting in automutilations, as well as autonomic failure causing neurogenic orthostatic hypotension, neurogenic underactive bladder, and gastroparesis and constipation. The diagnosis is clinical with support of nerve conduction studies and autonomic testing, as well as spinal cord magnetic resonance imaging showing characteristic posterior cord hyperintensities. Although the presumed etiology is immune-mediated, further studies are required to clarify the physiopathology of the disease. We here performed a systematic review of the epidemiology, pathophysiology, diagnosis, and management of ASANN, with three representative cases that recently presented at our clinic. All three patients had the typical clinical manifestations of ASANN but in different combinations, illustrating the variable phenotype of the disorder. Immunosuppression is seldom effective. Management options are limited to supportive and symptomatic care with the goal of minimizing complications and preventing death.

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Section: Autonomic Function Testingmentioning

confidence: 99%

Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia

Gutiérrez

Palma

2020

Semin Neurol

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“…AASN causes various degrees of autonomic and sensory function impairments. In some patients with AASN, mild or slight motor weakness can be combined with autonomic and sensory dysfunction [ 3 ]. In addition, there are no definite diagnostic criteria to confirm AASN.…”

Section: Introductionmentioning

confidence: 99%

Gadolinium enhancement in cervical dorsal roots in a patient with acute autonomic and sensory neuropathy: a case report

et al. 2023

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Background We report an enhancement of the dorsal roots on gadolinium-enhanced cervical magnetic resonance imaging (MRI) in a patient with acute autonomic and sensory neuropathy (AASN). Case presentation A 38-year-old woman visited our university hospital for dizziness and fainting while rising from sitting or lying down and a tingling sensation in the whole body, including her limbs, torso, and abdomen, which was sustained for 15 days. The patient had hyperalgesia in nearly her entire body and slight motor weakness in her bilateral upper and lower limbs. Autonomic dysfunction was confirmed using autonomic testing. Furthermore, the nerve conduction study showed an absence of sensory nerve action potentials in all evaluated peripheral nerves. Cervical MRI was performed 18 days after dysautonomia onset. In the axial T1-gadolinum-enhanced MRIs, enhancement in cervical ventral and dorsal nerve roots and the posterior column of the spinal cord were observed, and the axial T2-weighted MRI showed high signal intensity in the posterior column of the cervical spinal cord. Considering the clinical, electrophysiological and imaging findings, the patient was diagnosed with AASN. A total dose of 90 g (2 g/kg) of intravenous immunoglobulin was administered over 5 days. At the follow-up at 4 years after AASN symptom onset, the hyperalgesia and orthostatic hypotension symptoms improved. However, her systolic blood pressure intermittently decreased to < 80 mmHg. Conclusion Gadolinium-enhanced MRI may facilitate the accurate and prompt diagnosis of AASN.

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Diagnosis and therapeutic options for peripheral vasculitic neuropathy

Blaes

2015

Therapeutic Advances in Musculoskeletal

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Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types.

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Differential Recovery in Cardiac and Vasomotor Sympathetic Functional Markers in a Patient with Acute Autonomic Sensory and Motor Neuropathy

Cited by 5 publications

References 27 publications

Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia

Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia

Gadolinium enhancement in cervical dorsal roots in a patient with acute autonomic and sensory neuropathy: a case report

Diagnosis and therapeutic options for peripheral vasculitic neuropathy

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