Multiple System Atrophy

Neurology Shelf Exam and Board Review
6 Jun 202105:31

Summary

TLDRMultiple System Atrophy (MSA) is a neurodegenerative disorder characterized by glial cytoplasmic inclusions of alpha-synuclein. With an average onset at age 54, it presents with significant autonomic dysfunction, including urinary incontinence and erectile dysfunction, alongside parkinsonism and cerebellar ataxia. Two primary motor subtypes are identified: predominant parkinsonism (MSAP) and predominant cerebellar ataxia (MSAC). Diagnosis is challenging, often confirmed post-mortem, with treatment options focusing on symptom management rather than disease modification. Prognosis is generally poor, with many patients becoming wheelchair-bound within three to five years after diagnosis.

Takeaways

  • 🧠 Multiple System Atrophy (MSA) is a neurodegenerative disease characterized by glial cytoplasmic inclusions of alpha-synuclein.
  • 📅 The average age of onset for MSA is around 54 years, with the cause remaining unknown.
  • 🔍 MSA presents with significant autonomic dysfunction due to neuron loss in specific brain regions.
  • 🚻 Common autonomic symptoms include urinary issues, erectile dysfunction, and orthostatic hypotension.
  • 💪 There are two main motor subtypes of MSA: predominant parkinsonism and predominant cerebellar ataxia.
  • 🔄 Symptoms of MSA can evolve, and a patient's subtype may change as the disease progresses.
  • 🛌 REM sleep behavior disorder is a notable early symptom and may appear years before diagnosis.
  • 🧪 Diagnosis of MSA is primarily clinical, with a definitive diagnosis confirmed only through autopsy.
  • 💊 Treatment options are limited; levodopa may help early but is ineffective long-term, while physical and occupational therapies are beneficial.
  • ⏳ Prognosis for MSA patients typically includes becoming wheelchair-bound within 3-5 years and a life expectancy of 6-10 years post-diagnosis.

Q & A

  • What is the main pathological hallmark of multiple system atrophy (MSA)?

    -The main pathological hallmark of MSA is glial cytoplasmic inclusions of alpha-synuclein.

  • At what age does multiple system atrophy typically onset?

    -The mean age of onset for MSA is around 54 years old.

  • What types of neuron loss contribute to autonomic dysfunction in MSA?

    -Neuron loss in the intermediolateral cell column and ventrolateral medulla contributes to autonomic dysfunction.

  • What are some common symptoms of autonomic dysfunction in MSA?

    -Common symptoms include bladder dysfunction (retention or incontinence), erectile dysfunction, and orthostatic hypotension.

  • What distinguishes the predominant parkinsonism subtype (MSAP) of MSA?

    -The MSAP subtype features prominent symptoms like bradykinesia, rigidity, and postural instability, with a tendency for action or postural tremors.

  • What classic cerebellar symptoms are associated with the predominant cerebellar ataxia subtype (MSAC)?

    -Classic symptoms include gait or limb ataxia, scanning dysarthria, nystagmus, and dysmetria.

  • How does the progression of MSA affect a person's subtype?

    -As the disease progresses, a person's subtype can change, reflecting different symptom presentations.

  • What imaging findings may be observed in an MRI of a patient with MSA?

    -MRI may show atrophy of the putamen, pons, and middle cerebellar peduncles, with the characteristic 'hot cross bun' sign due to degeneration of transverse fibers.

  • What treatments are available for managing symptoms of MSA?

    -While there is no disease-modifying treatment, levodopa can be helpful early on, and other medications, physical therapy, and speech therapy can assist with symptom management.

  • What is the typical prognosis for patients diagnosed with multiple system atrophy?

    -Patients often become wheelchair-bound within three to five years of diagnosis, with death typically occurring six to ten years after diagnosis.

Outlines

plate

Dieser Bereich ist nur für Premium-Benutzer verfügbar. Bitte führen Sie ein Upgrade durch, um auf diesen Abschnitt zuzugreifen.

Upgrade durchführen

Mindmap

plate

Dieser Bereich ist nur für Premium-Benutzer verfügbar. Bitte führen Sie ein Upgrade durch, um auf diesen Abschnitt zuzugreifen.

Upgrade durchführen

Keywords

plate

Dieser Bereich ist nur für Premium-Benutzer verfügbar. Bitte führen Sie ein Upgrade durch, um auf diesen Abschnitt zuzugreifen.

Upgrade durchführen

Highlights

plate

Dieser Bereich ist nur für Premium-Benutzer verfügbar. Bitte führen Sie ein Upgrade durch, um auf diesen Abschnitt zuzugreifen.

Upgrade durchführen

Transcripts

plate

Dieser Bereich ist nur für Premium-Benutzer verfügbar. Bitte führen Sie ein Upgrade durch, um auf diesen Abschnitt zuzugreifen.

Upgrade durchführen
Rate This

5.0 / 5 (0 votes)

Ähnliche Tags
NeurodegenerativeHealthcareMultiple System AtrophySymptomsDiagnosisTreatmentAutonomic DysfunctionPatient CareOccupational TherapyPrognosis
Benötigen Sie eine Zusammenfassung auf Englisch?