Neuropathy Hereditary Sensory And Autonomic Type 2b C2751092
An early-onset inherited sensory neuropathy affecting nerve function. Patients lack normal sensation, increasing risk of skin damage and infections.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Sudden severe orthostatic hypotension
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Insidious loss of sensation in distal limbs
(neurologic)Motor weakness in lower limbs
(neurologic)Ulcerative foot lesions
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Autonomic symptom management
Targeted pharmacotherapy for autonomic dysfunction including orthostatic hypotension, gastroparesis, and temperature regulation problems using appropriate agents such as midodrine or fludrocortisone.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Cardiovascular autonomic testing
Regular assessment of heart rate variability, blood pressure regulation, and sudomotor function to track disease progression and guide treatment adjustments.
Additional treatment options
Compression therapy and positional management
Use of compression garments and gradual position changes when rising to manage orthostatic intolerance. Adequate hydration and salt intake to support blood volume.
lifestyleGastrointestinal symptom modification
Dietary modifications including small frequent meals, adequate fiber intake, and food texture adjustments for patients with dysphagia or gastroparesis symptoms.
dietaryChronic illness counseling
Psychological support addressing the emotional impact of managing a progressive hereditary condition affecting multiple body systems and independence.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Neuropathy Hereditary Sensory And Autonomic Type 2b C2751092?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy