Motor Neuron Disease: Difference between revisions
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=== Clinic === | === Clinic === | ||
MNDs are a group of rare | MNDs are a group of rare neuro-degenerative disorders that selectively affect motor neurons, including | ||
* Amyotrophic lateral sclerosis (ALS) | * Amyotrophic lateral sclerosis ([[ALS, Amyotrophic Lateral Sclerosis remedies|ALS]]) | ||
* Progressive bulbar palsy (PBP) | * [[Progressive bulbar palsy]] ([[Progressive bulbar palsy|PBP]]) | ||
* Pseudobulbar palsy | * [[Pseudobulbar palsy]] | ||
* Progressive muscular atrophy (PMA) | * [[Progressive muscular atrophy]] (PMA) | ||
* Primary lateral sclerosis (PLS) | * [[Primary lateral sclerosis]] (PLS) | ||
* Spinal muscular atrophy (SMA) | * Spinal muscular atrophy (SMA) (Gentic) | ||
* Monomelic amyotrophy (MMA)<ref>Ramroop H, Cruz R. Electrodiagnostic Evaluation Of Motor Neuron Disease. 2022 Nov 5. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 33085325.</ref> | * [[Monomelic amyotrophy]] (MMA)<ref>Ramroop H, Cruz R. Electrodiagnostic Evaluation Of Motor Neuron Disease. 2022 Nov 5. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 33085325.</ref> | ||
=== Signs / Symptoms === | === Signs / Symptoms === | ||
==== UMN | ==== UMN ==== | ||
* | * Spasticity, Increased muscle tone | ||
* Overactive reflexes: Hyper reflexia or Abnormal reflexes (Brisk reflexes, [[Babinski Reflex|Babinski reflex]], Hoffman's reflex) | |||
* Overactive reflexes | * Slow / Difficult. voluntary movements | ||
* | * Inability to walk or control other movements. | ||
* | |||
==== LMN | ==== LMN ==== | ||
* Muscle Wasting / Weakness / Atrophy | * Muscle Wasting / Weakness / Atrophy | ||
* Muscle | * Muscle Twitching/ Fasciculations | ||
* Hypotonia or atonia | * Hypotonia or atonia | ||
| Line 32: | Line 29: | ||
==== Common symptoms | |||
==== Bulbar ==== | |||
* [[Dysarthria]] | |||
* [[Dysphagia]] | |||
* Sialorrhea | |||
* [[Pseudobulbar affect]] | |||
* Cognitive / Behavioural changes (Problems in word fluency, Decision-making, and Memory) | |||
* Sensation (Ability to feel) is typically not affected | |||
==== Common symptoms ==== | |||
* Gait abnormalities | * Gait abnormalities | ||
* Tongue atrophy | * Tongue atrophy | ||
| Line 39: | Line 48: | ||
* Thenar muscle atrophy | * Thenar muscle atrophy | ||
* Exertion Dyspnea | |||
* Orthopnea | |||
=== Various Patterns of weakness === | === Various Patterns of weakness === | ||
| Line 64: | Line 61: | ||
# Symmetric weakness without sensory loss (e.g. PMA, PLS) | # Symmetric weakness without sensory loss (e.g. PMA, PLS) | ||
# Symmetric focal midline proximal weakness (neck, trunk, bulbar involvement; e.g. ALS, PBP, PLS) | # Symmetric focal midline proximal weakness (neck, trunk, bulbar involvement; e.g. ALS, PBP, PLS) | ||
=== Classification === | === Classification === | ||
| Line 103: | Line 101: | ||
!UMN | !UMN | ||
* | * | ||
!LMN | !LMN | ||
* | * | ||
* | * | ||
!Emotional lability | !Emotional lability | ||
!Bulbar palsy | !Bulbar palsy | ||
* | * | ||
|- | |- | ||
|CJD | |CJD | ||
Latest revision as of 22:54, 13 April 2023
Clinic
MNDs are a group of rare neuro-degenerative disorders that selectively affect motor neurons, including
- Amyotrophic lateral sclerosis (ALS)
- Progressive bulbar palsy (PBP)
- Pseudobulbar palsy
- Progressive muscular atrophy (PMA)
- Primary lateral sclerosis (PLS)
- Spinal muscular atrophy (SMA) (Gentic)
- Monomelic amyotrophy (MMA)[1]
Signs / Symptoms
UMN
- Spasticity, Increased muscle tone
- Overactive reflexes: Hyper reflexia or Abnormal reflexes (Brisk reflexes, Babinski reflex, Hoffman's reflex)
- Slow / Difficult. voluntary movements
- Inability to walk or control other movements.
LMN
- Muscle Wasting / Weakness / Atrophy
- Muscle Twitching/ Fasciculations
- Hypotonia or atonia
- Hypo reflexia
Bulbar
- Dysarthria
- Dysphagia
- Sialorrhea
- Cognitive / Behavioural changes (Problems in word fluency, Decision-making, and Memory)
- Sensation (Ability to feel) is typically not affected
Common symptoms
- Gait abnormalities
- Tongue atrophy
- Upper limb and truncal muscle atrophy
- Positive Babinski sign
- Thenar muscle atrophy
- Exertion Dyspnea
- Orthopnea
Various Patterns of weakness
- Symmetric or asymmetric
- Distal, proximal, or both
- According to Statland et al., there are three main weakness patterns: [2]
- Asymmetric distal weakness without sensory loss (e.g. ALS, PLS, PMA, MMA)
- Symmetric weakness without sensory loss (e.g. PMA, PLS)
- Symmetric focal midline proximal weakness (neck, trunk, bulbar involvement; e.g. ALS, PBP, PLS)
Classification
| Type | UMN degeneration | LMN degeneration |
|---|---|---|
| Sporadic MNDs | ||
| ALS | Yes | Yes |
| PLS | Yes | No |
| PMA | No | Yes |
| PBP | Yes | Yes, bulbar region |
| Pseudobulbar palsy | Yes, bulbar region | No |
| MMA | No | Yes |
Entities / Miasms
| Miasms | UMN
|
LMN
|
Emotional lability | Bulbar palsy
|
|---|---|---|---|---|
| CJD | +++ | +++ | ||
| PLV | + | +++ | +++ | |
| GSS | +++ | +++ | ||
| TBE | +++ | +++ | ||
| NVCJD ? | +++ | +++ ??? |
- ↑ Ramroop H, Cruz R. Electrodiagnostic Evaluation Of Motor Neuron Disease. 2022 Nov 5. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 33085325.
- ↑ Statland JM, Barohn RJ, McVey AL, Katz JS, Dimachkie MM (November 2015). "Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis". Neurologic Clinics. 33 (4): 735–748.