Subacute inflammatory demyelinating polyneuropathy

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Overview

Subacute inflammatory demyelinating polyneuropathy (SIDP) is a rare immune-mediated peripheral nerve disorder that represents an intermediate form between acute inflammatory demyelinating polyneuropathy (AIDP, also known as Guillain-Barré syndrome) and chronic inflammatory demyelinating polyneuropathy (CIDP). It is characterized by progressive or relapsing weakness and sensory disturbances in the limbs that develop over a subacute time course, typically between 4 and 8 weeks from symptom onset. This distinguishes it from AIDP, which reaches its nadir within 4 weeks, and CIDP, which progresses beyond 8 weeks. The disease primarily affects the peripheral nervous system, where an aberrant immune response targets the myelin sheath surrounding peripheral nerves. This demyelination leads to impaired nerve signal conduction, resulting in symmetrical proximal and distal muscle weakness, reduced or absent deep tendon reflexes (hyporeflexia or areflexia), sensory loss including numbness and tingling (paresthesias), and sometimes neuropathic pain. Patients may experience difficulty walking, impaired fine motor skills, and fatigue. Cranial nerves may occasionally be involved. Electrodiagnostic studies typically reveal features of demyelination, and cerebrospinal fluid analysis often shows elevated protein with a normal cell count (albuminocytologic dissociation). Treatment for SIDP is similar to that used for CIDP and includes immunomodulatory therapies such as intravenous immunoglobulin (IVIg), plasma exchange (plasmapheresis), and corticosteroids. Many patients respond favorably to these treatments, though some may experience relapses and require long-term immunosuppressive therapy. The prognosis is generally considered better than that of CIDP, with some patients achieving full or near-complete recovery, particularly with early and appropriate treatment. Close neurological follow-up is important to monitor for progression to a chronic course.

Also known as:

Clinical phenotype terms— hover any for plain English:

Decreased motor nerve conduction velocityHP:0003431Abnormality of somatosensory evoked potentialsHP:0007377Axonal lossHP:0003447Diffuse peripheral demyelinationHP:0006881Decreased amplitude of sensory action potentialsHP:0007078Demyelinating motor neuropathyHP:0007220Symmetric peripheral demyelinationHP:0007262Peripheral demyelinationHP:0011096
Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Subacute inflammatory demyelinating polyneuropathy.

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Specialists

2 foundView all specialists →

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Common questions about Subacute inflammatory demyelinating polyneuropathy

What is Subacute inflammatory demyelinating polyneuropathy?

Subacute inflammatory demyelinating polyneuropathy (SIDP) is a rare immune-mediated peripheral nerve disorder that represents an intermediate form between acute inflammatory demyelinating polyneuropathy (AIDP, also known as Guillain-Barré syndrome) and chronic inflammatory demyelinating polyneuropathy (CIDP). It is characterized by progressive or relapsing weakness and sensory disturbances in the limbs that develop over a subacute time course, typically between 4 and 8 weeks from symptom onset. This distinguishes it from AIDP, which reaches its nadir within 4 weeks, and CIDP, which progresses

At what age does Subacute inflammatory demyelinating polyneuropathy typically begin?

Typical onset of Subacute inflammatory demyelinating polyneuropathy is adult. Age of onset can vary across affected individuals.

Which specialists treat Subacute inflammatory demyelinating polyneuropathy?

2 specialists and care centers treating Subacute inflammatory demyelinating polyneuropathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.