Disorders of the Motor Unit Flashcards
What are some of the symptoms of motor unit disease
Muscle weakness
Myalgia = muscle ache
Fasciculations = muscle twitch
Muscle wasting
How do you differentiate myopathy and neuropathy?
Clinical features
= In neurogenic there is loss of reflex, fasciculations, sensory loss
Lab
= Biopsy: Neurogenic- Fibre type grouping. Myopathy- Necrosis & Regeneration
= Enzymes: Myopathy has much more Ck
= EMG: Duration of Potentials: Neurogenic (Giant), Myopathy (Small). Fibrillation: Neurogenic (Yes), Myopathy (No).
What are some examples of Neurogenic diseases
MND
Guillane Barre Syndrome
Peripheral Neuropathy
What are some Myopathic Diseases
Inherited: DMD, Limb Girdle muscular dystrophy
Acquired: Dermatomyositis
Patient presents with Weakness, Atrophy, Fasciculations, Suppressed Reflexes. What is the diagnosis?
Lower Motor Neurone dysfunction in MND
What are the signs for UMN Dysfunction
Spasticity, Brisk reflexes, Babinski sign, Uncontrolled emotions
What are the causes of MND
Inherited
Toxicity
Pn Misaggregation and Abnormal Precipitation
Mutation
How do you treat MND?
Antispasticity, Relieve dry mouth, Aid respiration, Anti-glutamatergic
Patient presents with motor and sensory symptoms.
Sensory: Numbness
Motor: Weakness in hands, ankles, bulbar, facial
Guillaine Barre Syndrome
What are the causes of GBS
Infection of Upper Respiratory
Cross reacting antibodies attach myelin to periph nerves
What is the treatment of GBS
Plasma exchange
Immunoglobin to surppress immune response
What are the causes of Peripheral Neuropathy
Alcohol, B12, Carcinoma, Diabetes
Patient presents with invereted champagne bottle legs and Pes Cavus, muscle weakness and loss of reflexes/sensation
Heriditary Peripheral Neuropathy
How is DMD inherited
X Linked Recessive
How do you diagnose DMD
V. High Ck
No staining with Abs to dystrophin
Visible deletion in Xp21
Mutation in dystrophin gene
What are the signs of DMD
Progressive disease
Proximal muscle wasting
Calf pseudohypertrophy
Gowers manoeuvre
How do you treat DMD
Symptomatic relief
Gene therapy - Direct muscle injection of recombinant DNA, myoblast implantation
Patient presents with violaceous rash, proximal myopathy and inflammation of muscle
Dermatomyositis
What are the causes of Dermatomyositis
Side effects of statins
Underlying malignant tumour
Autoimmune of small intramuscular bv
