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

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.

Rituximab treatment in seronegative autoimmune autonomic neuropathy and autoimmune autonomic ganglionopathy: Case-report and literature review.

BACKGROUND AND PURPOSE: Autoimmune autonomic ganglionopathy (AAG) is a rare disease with no well-established treatment. Until recently, AAG could be seropositive (50 to 60% of patients) or seronegative for ganglionic (α3-type) nicotinic acetylcholine receptor (Gα3NAChR) antibodies. In early 2018, the two forms of the disease were distinguished, separating seropositive from seronegative ones, designating this latter form "seronegative autoimmune autonomic neuropathy" (SAAN). Most described treatments are plasma exchange (PE) and intravenous immunoglobulin (IVIG). However in some cases with no or small benefit, other immunomodulatory therapies, such as rituximab have been reported. We report the case of a 24-year-old female patient successfully treated for SAAN with rituximab and steroids after IVIG and PE failure. We also provide a review of case-reports reporting rituximab treatment for both SAAN and AAG. METHODS: To identify articles reporting SAAN and AAG treatment with rituximab, we searched the PubMed database using the terms "autoimmune autonomic ganglionopathy", "autoimmune autonomic neuropathy" or "seronegative autoimmune autonomic neuropathy" and "rituximab". RESULTS: Including our patient, nine cases have been described in the literature (4 SAAN and 5 AAG). Rituximab had a significant positive effect in 2 out of 4 SAAN and all 5 AAG cases, used alone or in association with other etiologic treatments. CONCLUSION: Our study suggests rituximab (alone or in association with other treatments) could provide efficacy in both SAAN and AAG when PE and/or IVIG are not effective enough.

Recurrent autonomic and sensory neuropathy in a patient with anti-ganglionic acetylcholine receptor antibodies.

We report a case of recurrent neuropathy with predominant autonomic and sensory involvement whose serum was positive for anti-ganglionic acetylcholine receptor (anti-gAChR) antibodies, a diagnostic marker of autoimmune autonomic ganglionopathy. An 11-year-old girl complained of numbness and limb pain after gastroenteritis. Although hyperalgesia and autonomic dysfunctions, such as orthostatic intolerance and gastrointestinal dysmotility subsequently developed, these symptoms faded after a few days. Similar sensory and autonomic impairments recurred three times within 12 months after the first episode. The sensory and autonomic symptoms were rapidly ameliorated by the administration of intravenous immunoglobulin (IVIg) at the second and third relapse; however, the symptoms persisted even after the administration of IVIg at the fourth relapse. The residual symptoms disappeared after methylprednisolone pulse therapy. The patient's serum was found to be positive for anti-gAChR antibodies at the second relapse, and was negative after methylprednisolone pulse therapy. Further studies are needed to clarify the efficacy of treatment and the nosological position in the spectrum of neuropathies that are associated with autonomic and sensory impairments.

A Case of Acute Autonomic and Sensory Neuropathy Accompanied by Respiratory Failure

Acute autonomic and sensory neuropathy (AASN) is a rare neuropathy characterized by the acute onset of autonomic dysfunction and objective sensory disturbances. A 33-year-old woman experienced generalized fatigue, urinary retention, and defecation difficulty with numbness around her mouth followed by a decreased pain sensation over her face and whole body, and respiratory difficulty with aspiration pneumonia. Neurological examination revealed bilaterally dilated fixed pupils, loss of sweating and lacrimation, orthostatic hypotension, and decreased sensation of all modalities with transient mild to moderate motor weaknesses. The muscle weakness may have been responsible for her respiratory failure as her respiratory distress was resolved in conjunction with improved general muscle power. Although the pathogenetic mechanism of AASN has been generally believed to be immune-mediated, the absence of immunoglobulin responsiveness and the negative results to various autoantibody tests in our case, raises questions against its autoimmune etiology. The patient showed slow progress of overcoming her autonomic dysfunction with relatively persistent sensory deficits.