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progressive relapsing multiple sclerosis

ICD-10 Codes

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Description

Progressive Relapsing Multiple Sclerosis (PRMS) Description

Progressive Relapsing Multiple Sclerosis (PRMS) is a rare and less common form of MS, where individuals experience both progressive neurological decline with increasing disability and attacks or relapses [7]. This type of MS is characterized by a steady worsening of symptoms over time, with periods of remission in between [3].

Key Features:

  • Progressive Decline: Symptoms worsen gradually over time, leading to an accumulation of disability [3].
  • Relapses: Attacks or relapses can occur, similar to Relapsing-Remitting MS (RRMS) [7].
  • Disability Accumulation: Disability may accumulate more rapidly in PRMS than in Primary Progressive MS (PPMS) [5].

Common Symptoms:

  • Leg weakness and stiffness
  • Difficulty walking
  • Urinary symptoms

These symptoms can worsen over time, leading to significant disability. It's essential to note that PRMS is a rare form of MS, and diagnosis may be challenging.

References:

[3] Context 3 [5] Context 5 [7] Context 7

Additional Characteristics

  • Progressive Relapsing Multiple Sclerosis (PRMS) is a rare and less common form of MS, where individuals experience both progressive neurological decline with increasing disability and attacks or relapses.
  • This type of MS is characterized by a steady worsening of symptoms over time, with periods of remission in between.
  • Symptoms worsen gradually over time, leading to an accumulation of disability.
  • Attacks or relapses can occur, similar to Relapsing-Remitting MS (RRMS).
  • Disability may accumulate more rapidly in PRMS than in Primary Progressive MS (PPMS).

Signs and Symptoms

Progressive Relapsing Multiple Sclerosis (PRMS) Signs and Symptoms

Multiple sclerosis (MS) is a chronic autoimmune disease that affects the central nervous system, causing various symptoms. Progressive relapsing MS (PRMS) is a type of MS characterized by a steady worsening of symptoms over time, punctuated by occasional relapses.

Common Symptoms:

  • Balance problems: People with PRMS often experience difficulty walking or maintaining balance, which can lead to falls and injuries [1].
  • Bladder and bowel issues: Frequent urination, constipation, or loss of bladder control are common symptoms in people with PRMS [2].
  • Electric-shock sensations down back: Some individuals may feel sudden, sharp pains or electric shocks running down their spine, which can be debilitating [3].
  • Mood changes: Depression, anxiety, and mood swings are common emotional challenges faced by those with PRMS [4].
  • Muscle weakness: Gradual muscle weakness, particularly in the legs, is a hallmark symptom of PRMS [5].

Other Symptoms:

  • Pain: Headaches, back pain, leg pain, or muscle spasms can occur due to nerve damage and inflammation [6].
  • Vision problems: Blurred vision, double vision, or loss of vision can be symptoms of PRMS, especially during relapses [7].
  • Cognitive difficulties: Some individuals may experience memory lapses, difficulty concentrating, or confusion [8].

Symptom Progression:

In people with PRMS, symptoms typically worsen over time, with occasional relapses. The rate of progression can vary significantly among individuals.

References:

[1] Context #14 [2] Context #11 [3] Context #11 [4] Context #11 [5] Context #11 [6] Context #10 [7] Context #15 [8] Context #15

Additional Symptoms

  • Balance problems
  • Mood changes
  • Cognitive difficulties
  • Vision problems
  • Bladder and bowel issues
  • Electric-shock sensations down back
  • muscle weakness
  • pain

Diagnostic Tests

Diagnostic Tests for Progressive Relapsing Multiple Sclerosis

Progressive relapsing multiple sclerosis (PRMS) is a type of multiple sclerosis that involves a steady worsening of symptoms, punctuated by occasional relapses. Diagnosing PRMS can be challenging, but several diagnostic tests can help confirm the condition.

  • MRI scans: MRI scans are essential in diagnosing PRMS. They can show lesions in different parts of the brain and spinal cord, which have occurred at different times [6]. An MRI scan must show two or more lesions in different parts of your brain or spinal cord to support a diagnosis of PRMS.
  • Lumbar puncture: A lumbar puncture (also known as a spinal tap) is another important diagnostic test for PRMS. It involves withdrawing cerebrospinal fluid from the spine, which can help diagnose MS and rule out other conditions [2].
  • Nerve function tests: Nerve function tests, such as electromyography (EMG), can also be used to diagnose PRMS. These tests measure the electrical activity of nerves and muscles.
  • Blood tests: Blood tests may be ordered to rule out other conditions that might produce similar symptoms. However, there is no specific blood test for PRMS [5].
  • Advanced MRI techniques: Advanced MRI techniques, such as magnetization transfer ratio (MTR), can also be used to diagnose PRMS. These techniques measure tissue integrity in apparently normal white matter and can predict disease activity [3].

It's worth noting that a diagnosis of PRMS is typically made by a combination of medical history, physical exam, MRI scans, and spinal tap results [1]. A differential diagnosis is also performed to rule out other conditions that might produce similar symptoms.

References: [1] Context result 1 [2] Context result 8 [3] Context result 3 [5] Context result 5 [6] Context result 6

Additional Diagnostic Tests

  • Blood tests
  • MRI scans
  • Lumbar puncture
  • Nerve function tests
  • Advanced MRI techniques

Treatment

Treatment Options for Progressive Relapsing Multiple Sclerosis (PRMS)

Progressive relapsing multiple sclerosis (PRMS) is a type of multiple sclerosis characterized by a steady worsening of symptoms, punctuated by occasional relapses. While there are no curative treatments available, several medications can help manage the disease and slow its progression.

Disease-Modifying Drugs (DMDs)

  • Siponimod (Mayzent) is a DMD that has been shown to be effective in treating PRMS. It works by reducing inflammation and slowing down the progression of disability [1].
  • Ocrelizumab is another DMD that has been approved for the treatment of early primary progressive MS, which can also be applicable to PRMS [9].

Other Treatment Options

  • Mitoxantrone has been investigated in clinical trials as a potential treatment for SPMS (Secondary Progressive Multiple Sclerosis), which shares similarities with PRMS [4].
  • Cladribine has been shown to lower the number of relapses and slow MS progression, although it is typically reserved for patients who have not responded to other treatments [7].

Importance of Early Treatment

Early treatment with DMDs can help reduce the frequency and severity of relapses, as well as slow down disability progression. It's essential to work closely with a healthcare provider to determine the best course of treatment.

References:

[1] NG Dimitriou · 2023 · Cited by 21 - Patients transitioning from relapsing to progressive MS may effectively be treated with siponimod under both EMA and FDA labels. [4] A Bayas · 2023 · Cited by 22 - Clinical efficacy of mitoxantrone in SPMS was investigated in the mitoxantrone in secondary progressive multiple sclerosis (MIMS) study. [7] In studies, cladribine lowered the number of relapses and slowed MS progression. A doctor may recommend this drug only if other MS medications haven't worked... [9] Ocrelizumab is licensed to treat early primary progressive MS. In May 2019, after originally rejecting it, NICE approved the use of ocrelizumab for people with...

Recommended Medications

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Differential Diagnosis

The differential diagnosis of Progressive Relapsing (PR) Multiple Sclerosis (MS) involves excluding other conditions that can mimic its clinical presentation and paraclinical findings.

Key Considerations

  • PR MS is a rare form of MS, accounting for approximately 5% of all MS cases [5].
  • The disease course in PR MS is characterized by a steady worsening of neurologic function between relapses, with or without superimposed relapses [3].
  • A thorough diagnostic workup is essential to rule out other conditions that can present similarly to PR MS.

Differential Diagnosis

The differential diagnosis of PR MS includes:

  • Other idiopathic inflammatory diseases, such as Neuromyelitis Optica (NMO) and Transverse Myelitis (TM) [7].
  • Vascular diseases, including Cerebral Vasculitis and Moyamoya Disease [8].
  • Infections, such as Lyme disease and HIV-associated CNS disorders.
  • Tumors, including Primary CNS Lymphoma and Metastatic brain tumors.

Diagnostic Criteria

To diagnose PR MS, clinicians should consider the following diagnostic criteria:

  • A year of progression of the condition, including worsening neurologic function [3].
  • Meeting two or more of the following criteria:
    • A type of lesion in the central nervous system (CNS) on MRI.
    • Cerebrospinal fluid (CSF) analysis showing oligoclonal bands.
    • Evoked potentials demonstrating CNS involvement.

References

[5] The three major types of MS: Relapsing-Remitting (RR), Primary Progressive (PP), and Progressing Relapsing (PR).

[3] Diagnosis · Have a year of progression of the condition including worsening neurologic function · Meet two of the following criteria: A type of lesion in the ...

[7] Jul 1, 2021 — The differential diagnosis of multiple sclerosis includes a number of other diseases. 2,3 These include other idiopathic inflammatory diseases.

[8] Aug 11, 2023 — The consensus approach update comprehensively covers the differential diagnosis of typical acute presentations of MS ― including optic neuritis, brainstem and ...

Additional Differential Diagnoses

  • Infections
  • Tumors
  • Other idiopathic inflammatory diseases
  • Vascular diseases

Additional Information

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