Case report: a case of acute autonomic and sensory neuropathy with fluctuating symptoms and literature review

doi:10.21203/rs.3.rs-4895411/v1

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Case report: a case of acute autonomic and sensory neuropathy with fluctuating symptoms and literature review

https://doi.org/10.21203/rs.3.rs-4895411/v1

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Purpose By reporting a patient with acute autonomic neuropathy (AASN) and reviewing relevant literature, we aim to improve understanding of the disease and assist in early clinical diagnosis and treatment.

Methods The clinical data and treatment of a patient with AASN who was admitted to our hospital were summarized, and the previously reported AASN cases or case series were systematically analyzed to summarize the gender, age of onset, prodromal infection, clinical manifestations, auxiliary examinations, treatment and prognosis of the patients.

Results The patient was a 50-year-old female who complained with “paresthesia and dizziness for more than 7 months”. In the early stage of the disease, the patient showed abnormal pain, dizziness, and sensory ataxia, without limb muscle weakness. Retrospective analysis of AASN case reports showed that the age of onset was 34.15 ± 17.43 years old, the male-female ratio was 3:10, and most of them had a history of prodromal infection. Previous literature reports on the treatment of AASN patients mainly included symptomatic supportive treatment and immunomodulatory treatment with hormones or intravenous immunoglobulin. Most patients gradually recover from autonomic nervous system symptoms after treatment, while sensory deficits persist.

Conclusions AASN is a rare, rapidly progressive disease involving autonomic and sensory nerves, often secondary to upper respiratory or digestive tract infections. Clinical manifestations include hypoesthesia, hyperalgesia, digestive and cardiovascular dysfunction, etc. AASN patients should be identified as early as possible and given immunomodulatory therapy and symptomatic treatment in order to improve the prognosis.

Acute autonomic and sensory neuropathy

Autonomic neuropathies

Guillain-Barre syndrome

Autoimmune disease

Diagnosis.

Acute autonomic and sensory neuropathy (AASN) is a rare neuroimmune disease first described by Colan et al. in 1980(1). The onset is similar to Guillain-Barre syndrome (GBS), and about 2/3 of patients have a history of respiratory or gastrointestinal infection before the disease. Symptoms peak within 2 weeks, and clinical symptoms include autonomic nervous system dysfunction and severe, rapidly progressive sensory loss, with motor function relatively preserved. This presents as widespread symptoms involving the skin, urinary-genital system, cardiovascular system, and gastrointestinal system. In the patients with AASN reported so far, most magnetic resonance imaging (MRI) shows abnormal high signal intensity in the posterior column of the spinal cord, and nerve conduction study (NCS) shows decrease in myelinated or unmyelinated nerve fibers. Lumbar puncture in patients with AASN can reveal the phenomenon of albuminocytologic.

However, the diagnostic criteria for this disease are not well-defined. AASN is extremely rare, and there are only a few reported cases and summaries of clinical features, with no specific laboratory diagnostic markers discovered so far. Imaging studies also lack specific features, leading to easy misdiagnosis and missed diagnosis. Here, a patient with AASN admitted to our hospital is reported, and a review of relevant literature is conducted to improve the broad recognition of this disease by clinicians.

The patient, a 50-year-old female, was admitted to our hospital due to abnormal body sensation and dizziness for over 7 months. On the day of onset, she suffered developed severe pain in the lower extremities during sleep without a clear cause, accompanied by abdominal distention, but no limb weakness or urinary dysfunction. The next day, she woke up with overall body pain and a temperature of 38.2°C, which returned to normal after using antipyretic drugs. The pain relieved after taking morphine-like analgesics. She experienced dizziness while standing and developed chest tightness, shortness of breath, nausea, and amaurosis ten days later. She also had multiple syncope episodes, which improved after she lay down. Thoracic spinal cord MRI showed suspicious abnormal signal of T7-8, considered “demyelinating disease”. She was admitted to the local hospital and developed urinary retention on the same day. Her symptoms improved and she was discharged after treatment with dexamethasone (10mg for 5 days) followed by oral prednisone (35mg per day, reduced by 10mg per week). Three days after discharge, her symptoms worsened, and she experienced general numbness and chest strangalesthesia. Two months after onset, the dizziness worsened, and she experienced dysphagia and bucking (prednisone dose of 5mg/day). She was then transferred to another hospital and received treatment with intravenous immunoglobulin (0.4g/kg, 5 days). Dysphagia and bucking improved, but other symptoms persisted, eventually she was admitted to our hospital.

She was hospitalized due to a lung abscess 4 weeks before onset, which was treated with appropriate medication for 2 weeks and recovered. She denied a history of hypertension, diabetes, heart disease, exposures to toxic substances or drugs. Her family history was unremarkable.

Her blood pressure was 127/91 mmHg (supine) and 90/74 mmHg (upright). Neurological examination showed relatively slow light reflex, symmetrical loss of deep and shallow sensations below the neck, urinary dysfunction, positive Romberg sign and sensory ataxia, with inaccurate finger-to-nose and heel-knee-shin tests with eyes closed. Her muscle strength and tone in all four extremities were normal, and the reflexes and Babinski’s sign were negative. The results of the rest of her physical examination were normal.

Complete blood counts, liver function tests, blood urea nitrogen, creatinine, serum electrolytes, coagulation function tests, thyroid function, vitamin B₁₂ level, glucose metabolism, autoimmune antibodies, tumor markers, virus infection indicators were normal. The cerebrospinal fluid (CSF) analysis showed a normal cell count and the protein level was 1.02 g/L. Anti-ranvier node/paranodal antibodies, anti-ganglioside antibody, anti-paraneoplastic antibodies, anti-demyelinating antibodies and anti-autoimmune encephalitis antibodies in serum and CSF were all negative, CSF oligoclonal band (OB) was positive. Renal ultrasonography showed increased residual urine (288ml). Echocardiography showed reduced left atrial contractility. Cranial MRI suggested multiple ischemic lesions in the brain, with no enhancement. Cervical and thoracic spinal MRI showed abnormal signals in posterior columns at the C2-7 and T1-12 level, with no enhancement (Fig. 1). Ganglionic nicotinic acetylcholine receptor antibody (gAchR) in serum and CSF was negative. NCS results showed that the amplitudes of the bilateral median and ulnar sensory nerves were reduced; the amplitudes of the bilateral peroneal (fibular) sensory nerves were absent (Table 1).

Table 1

Table of patient electromyography results
Motor nerve	Stimulation site	Latency (ms)		CMAP (mV)		MNCV (m/s)
Motor nerve	Stimulation site	Left	Right	Left	Right	Left	Right
Median	Wrist-APB	3.85	3.67	7.0	7.7	--	--
Median	Elbow-Wrist	8.10	8.22	6.5	7.5	54.1	52.7
Ulnar	Wrist-ADM	3.03	2.44	8.1	8.9	--	--
	Below elbow-Wrist	5.46	4.98	8.3	8.9	57.6	55.1
	Above elbow-Below elbow	8.02	7.81	7.1	8.5	54.7	49.5
Tibial	Ankle-Abd hal	3.67	3.00	9.6	12.8	--	--
Peroneal	Ankle-EDB	3.04	3.42	3.6	3.0	--	--
Peroneal	Fib. head-Ankle	9.78	10.70	3.5	2.9	43.0	41.2
Sensory nerve	Stimulation site	Latency (ms)		SNAP (µV)		SNCV (m/s)
Sensory nerve	Stimulation site	Left	Right	Left	Right	Left	Right
Median	Dig I -Wrist	1.57	1.88	10.4(↓75%)	7.4(↓82%)	47.8	42.6
Median	Dig III -Wrist	2.03	2.22	5.9(↓70%)	11.2	54.2	47.3
Ulnar	Dig Ⅴ -Wrist	1.73	1.81	3.7(↓81%)	4.1 (↓7%)	46.2	45.2
Peroneal	Calf-Fib.head	--	--	--	--	--	--
Sural	Ankle-Mid-calf	--	--	--	--	--	--
F-wave response	Stimulation site	Latency (ms)		#F		F %
Left Median	Wrist-APB	24.3		20		100%
Left Tibial	Ankle- AH	42.0		20		100%
CMAP: compound muscle action potential; MNCV: motor nerve conduction velocity; SNAP: sensory nerve action potential; SNCV: sensory nerve conduction velocity.

Based on clinical features, examination, and laboratory testings, AASN was finally confirmed. Although the course of the disease was relatively long, intravenous methylprednisolone was administered (1000mg*5d, 500mg*3d, 240mg*3d), and then tapered to an oral dose (60mg/day). After 3 years, her autonomic nervous system function has significantly improved, dizziness has been recovered, but sensory disorders still persist (pain and zonesthesia still prominent).

We reviewed the published English literature on AASN and autonomic neuropathy. We searched the PubMed database using the keywords "AASN" or "acute autonomic sensory neuropathy". The search period is from the database inception to February 2024, and the results will be summarized. Ultimately, a total of nine articles were included in our study.

We reviewed previous AASN case reports and systematic case studies, and the results are detailed in Table 2. Since Colan et al. first reported a case of AASN in 1980, together with our patient, there have been 13 patients with detailed information and a diagnosis of AASN. The onset age was 34.15 ± 17.43 years old, with a male to female ratio of 3:10. Among the 13 patients, 11 cases had a prodromic infection, with 7 cases of pulmonary or upper respiratory infection, 1 case of gastrointestinal infection, 2 case of fever, and 1 case of an elevated Epstein-Barr virus (EBV) titer in the blood(2). The interval between the preceding infection and the onset of symptoms was approximately 1–14 days. In all 13 patients, 6 patients initially presented with body pain, 4 with sensory loss, and 3 with gastrointestinal symptoms. Seven of these patients received immunosuppressive treatment (steroids or intravenous immunoglobulin), four of whom were able to walk independently. After treatment, persistent sensoryloss was reported in 8 patients (Table 3).

Table 2

Background of patients with AASN
	Sex	Age/ years	Antecedent events	Duration from antecedent event to initial symptom (days)	Duration from onset to peak (days)	Initial symptoms	High- intensity area in posterior column on MRI	CSF protein (g/l)	Sensory nerve conduction velocity (m/s) median/sural	Nerve biopsy
Colan, et al.(1)	M	9	Pueumonia	ND	1w	AP, AD, V, D	ND	0.44 (7 days later: 1.30)	NE/NE	loss of MF, ADG
Fujii, et al.(2)	F	23	EBV(+)	ND	4	P	ND	Normal	NE/NE	loss of MF and UMF
Tohg, et al.(23)	F	46	URI	2w	1w	N, AD, W	ND	1.5	MCV: 40/40	loss of MF and UMF
Kanda, et al.(4)	F	28	Fever	2w	3w	N, U	ND	ND	ND/32	loss of MF and UMF
Yasuda, et al.(5)	F	27	URI	10	10	P, D	་	ND	NE/NE	ADG
Yasuda, et al.(5)	F	44	URI	2w	ND	N, W	་	ND	NE/NE	ADG
Yasuda, et al.(5)	F	65	No	—	1w	AP	ND	ND	NE/NE	ND
Koike, et al.(3)	M	55	No	—	8	AP, AD	-	1.16	NE/NE	loss of MF and UMF
Koike, et al.(3)	F	27	Fever	6	4	N	-	1.14	60/48	loss of MF and UMF
Gales, et al.(24)	M	26	GI	3	3w	P	ND	ND	ND/ND	ND
Enokizono, et al.(25)	F	6	URI	1	5	P	ND	Albumino-cytologic dissociation	NE/NE	ND
Kim, et al.(6)	F	38	URI	3	ND	S	་	1.97	NE/NE	ND
This Case	F	50	PA	2w	2w	P	་	1.02	48/NE	ND
AASN: acute autonomic and sensory neuropathy; URI: upper respiratory infection; GI: gastrointestinal infection; PA: pulmonary abscess; ND: not determined; AP: abdominal pain; AD: abdominal distension; V: vomiting; D: diarrhoea; P: pain in the extremities or trunk; N: numbness in the extremities or trunk; W: weakness in the extremities or trunk; U: urinary disturbance; S: syncope; MF: myelinated fibre; UMF: unmyelinated fibre; AXD: axonal degeneration.

Table 3

Treatment and functional recovery of patients with AASN
	Treatment	Function recovery
Colan, et al.(1)	Antibiotic	No autonomic symptoms, pain in patchy areas over the body
Fujii, et al.(2)	—	Improvement of muscle power, severe sensory impairment and diarrhoea
Tohg, et al.(23)	GC	Improvement of muscle power, persistent autonomic and sensory symptom
Kanda, et al.(4)	ST	Improvement of OH, persistent urinary retention
Yasuda, et al.(5)	—	Pain and temperature sensation recovered, abasia
Yasuda, et al.(5)	—	Pain and temperature sensation recovered, severe ataxic gait
Yasuda, et al.(5)	—	Pain and temperature sensation recovered, severe ataxic gait
Koike, et al.(3)	IVIg	Dead
Koike, et al.(3)	IVIg	Improvement of OH 2 months after onset, able to walk 1 year after onset
Gales, et al.(24)	IVIg	No symptoms
Enokizono, et al.(25)	IVIg, GC, antiepileptic therapy	Able to walk 4 months after onset, the vomiting attacks disappeared
Kim, et al.(6)	IVIg, ST	Improvement of pain and OH
This Case	GC, IVIg	Improvement of OH, severe pain, severe ataxic gait
AASN: acute autonomic and sensory neuropathy; GC: glucocorticoid; ST: symptomatic treatment; IVIg: intravenous immunoglobulin; OH: orthostatic hypotension.

AASN is an acute progressive autonomic and sensory nerve system disease that often secondary to upper respiratory or gastrointestinal infections. The incidence rate of males and females is about 1:2(3). Sensory nerve involvement manifests as hypoesthesia, hyperalgesia, and sensory ataxia in severe cases. Autonomic dysfunction causes gastrointestinal functional disorders, which can lead to abdominal pain, abdominal distension, diarrhea, constipation, and adynamic ileus. Impaired cardiovascular function may cause arrhythmia, orthostatic hypotension, and even syncope. In addition, there may be various manifestations such as abnormal glandular secretion and bladder weakness. The patient in this case presented with general sensory abnormalities and orthostatic hypotension, as well as urinary retention. The Electromyography showed damage to sensory fibers, and MRI imaging showed abnormal signals in the posterior and lateral columns of the spinal cord.

Immune imbalances led by viral infections are likely common causes of AASN(4). There have been reports of EBV infections in previous cases, suggesting that EBV infection may be closely related to the onset of the disease(2). Unlike motor nerves, autonomic and sensory nerves are exposed outside the blood-nerve barrier, making them more vulnerable to attack by antibodies and activated T cells in the blood after viral infection, becoming the primary targets of AASN(5). AASN mainly destroys the dorsal nerve root, resulting in asymmetric, segmental sensory symptoms, accompanied by degeneration of the posterior column of the spinal cord and surrounding nerve trunks. In the early stage of the disease, small neurons are primarily affected, presenting with reduced superficial sensory and autonomic nerve dysfunction. As the disease progresses, larger nerve cells in the ganglions are damaged, leading to sensory ataxia. At this time, MRI may show abnormal signals in the posterior column(3). Kim et al.(6) found that enhanced MRI of AASN patients showed the enhancement in the dorsal nerve roots and posterior column of the spinal cord, suggesting that inflammatory response disrupts the blood-nerve barrier. Consistent with previous studies, the axial T2-weighted cervical-thoracic MRI of our patient showed a high signal intensity in the posterior column of the spianl cord.

Autonomic neuropathy is caused by various reasons, and can be divided into acute or subacute and chronic types based on the onset of the disease(7). Acute or subacute autonomic neuropathy includes autoimmune autonomic ganglionopathy (AAG), paraneoplastic autonomic neuropathy, GBS, and others. AAG is an immune-mediated autonomic neuropathy characterized by orthostatic hypotension, anhidrosis, and severe gastrointestinal motor dysfunction(8). Serum gAChR antibody is a specific diagnostic indicator of AAG, with the antibody titer positively correlated with the severity of the disease(8, 9). Paraneoplastic autonomic neuropathy is secondary to malignant tumors, particularly small cell lung cancer. Antibodies such as anti-Hu, PCA-2, CRMP-5, VGKC, and P/Q type calcium channel antibodies can highly suggest the presence of this disease(10). Sjögren's syndrome is another autoimmune disease characterized by dry eyes and mouth, with autonomic nerve dysfunction symptoms such as arrhythmia, orthostatic hypotension, etc(11). Antibodies against SSA and SSB can highly suggest the presence of this disease(12). GBS is an immune-mediated nerve root neuropathy, mainly characterized by motor and sensory nerve damage. However, it can also affect the autonomic nervous system, resulting in arrhythmia, blood pressure fluctuations, gastrointestinal motor dysfunction, and urinary retention, indicating a poor prognosis(13). In addition, chronic autonomic neuropathy also includes diabetic autonomic neuropathy, amyloidosis, hereditary sensory autonomic neuropathy, and others.

The relationship between AASN and GBS is not clear. Due to prodromic infection and CSF albuminocytologic in AASN patients, some researchers suggest that AASN is a special type of GBS. However, AASN patients only exhibit symptoms of sensory and autonomic nerve involvement, with relatively preserved motor function, so other researchers believe that AASN is an independent autonomic neuropathy. Approximately 6–16% of GBS patients experience symptom relief after treatment, then to worsen again, known as "treatment-related fluctuations" (GBS-TRF)(14). Ruets et al.(15) found 82% of patients with GBS-TRF experienced symptom fluctuations for the first time within 8 weeks of GBS onset, and only 27% showed a second fluctuation, with fewer cases experienced three or more symptom fluctuations. GBS-TRF was more likely to occur in patients with prolonged disease course, longer time to peak symptoms, higher baseline disability scores, and neuropathic pain(16, 17). Our patient also exhibited symptom fluctuations. She received corticosteroid treatment within 3 weeks of onset of symptoms, and experienced partial improvement. After discharge, symptoms worsened. The symptoms gradually worsened during the process of drugs reduction and discontinuation. The patient was subsequently given intravenous immunoglobulin treatment more than two months after the onset of the disease, and the symptoms gradually improved. The symptom fluctuations cannot be ruled out as being related to treatment, there is currently a lack of research on the worsening of symptoms in AASN patients after treatment relief. Our case suggest that the early treatment may block the immune process, but early interruption of treatment will lead to recurrent disease course. Further research is needed to explore the mechanisms and risk factors for symptom fluctuations in AASN patients.

Currently, there are no specific treatments for AASN. Clinically, immunomodulatory therapy and symptomatic treatment can be performed. Corticosteroids or intravenous immunoglobulin may slow the progression of the disease and improve the patients' symptoms to some extent(18). Symptomatic treatment for AASN patients can alleviate their pain, improve outcomes, and help maintain their quality of life. Parasympathetic stimulants such as carbachol and methacholine can alleviate pupillary, intestinal and bladder dysfunction. Mineralocorticoids, droxidopa, and midodrine can be used to treat orthostatic hypotension(19, 20). For patients with supine hypertension, short-acting antihypertensive drugs such as angiotensin II receptor blockers (losartan) and calcium channel blockers (nifedipine) can help control blood pressure(21). Patients with tachycardia may be treated with β-receptor antagonists after exclusion of cardiac disease. With treatment, AASN patients experience some improvement in autonomic nerve symptoms, but sensory symptoms tend to recover less favorably, and the presence of sensory ataxia indicates a poor prognosis(3). Furthermore, substantial neuronal damage is indicated by high neuron-specific enolase (NSE) levels in the CSF, which is associated with a poor prognosis(22).

Early identification and prompt treatment of AASN are important for improving disease outcomes, survival rates, and quality of life. AASN should be highly suspected in patients who present with symptoms of autonomic dysfunction and the presence of abnormal pain or limb numbness with relatively preserved motor function. In order to determine whether the disease has a history of prior infections and its onset features, a complete medical history should be obtained. Additionally, an objective neurological examination and autonomic nervous function assessment should be carried out. Spinal MRI, lumbar puncture, NCS, and sural nerve biopsy can help increase the diagnosis rate and distinguish AASN from other autonomic neuropathies. It should be emphasized that timely and effective treatment before deep sensory involvement can effectively delay the progression of the disease and improve prognosis. Corticosteroids or intravenous immunoglobulin may be effective treatments for AASN. During the treatment process, giving varying doses of treatment according to the severity of the patient's condition can bring better treatment outcomes to patients and avoid irreversible damage to the sensory and autonomic nerves caused by symptom fluctuations of the disease.

AASN: acute autonomic sensory neuropathy; GBS: Guillain-Barre syndrome; MRI: magnetic resonance imaging; CSF: cerebrospinal fluid; OB: oligoclonal band; gAchR: ganglionic nicotinic acetylcholine receptor antibody; NCS: nerve conduction study; EBV: Epstein-Barr virus; AAG: autoimmune autonomic neuropathy; GBS-TRF: treatment-related fluctuations; CMAP: compound muscle action potential; MNCV: motor nerve conduction velocity; SNAP: sensory nerve action potential; SNCV: sensory nerve conduction velocity; URI: upper respiratory infection; GI: gastrointestinal infection; PA: pulmonary abscess; ND: not determined; AP: abdominal pain; AD: abdominal distension; V: vomiting; D: diarrhoea; P: pain in the extremities or trunk; N: numbness in the extremities or trunk; W: weakness in the extremities or trunk; U: urinary disturbance; S: syncope; MF: myelinated fibre; UMF: unmyelinated fibre; ADG: axonal degeneration; GC: glucocorticoid; ST: symptomatic treatment; IVIg: intravenous immunoglobulin; OH: orthostatic hypotension.

Ethics statement

Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.

Conflict of Interest

The authors declare that they have no conflict of interest.

Author Contributions

Ming-Xuan Yang: Conceptualization, Formal analysis, Investigation, Writing - Original draft preparation; Jian-Li Wang, Xiao-Feng Wu: Investigation; Mei-Ni Zhang, Jun-Hong Guo: Supervision; Hua-Xing Meng: Conceptualization, Resources, Supervision, Project administration, Funding acquisition.

Funding

This work was supported by the Shanxi Province Basic Research Program [202203021222377].

Acknowledgments

Our authors deeply appreciate the editors and reviewers for their help with the manuscript.

Data Availability Statement

The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.

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Case report: a case of acute autonomic and sensory neuropathy with fluctuating symptoms and literature review

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