Neuromuscular Disorders Flashcards
How is the diagnosis of MG made?
Positive antibodies (80% have them)
Tensilon Test
Repeated stimulation
What type of gait might you observe is a patient with duchenne’s muscle weakness?
Trendellenberg’s
What is the classical constellation of signs typical of myopathies in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?
Fasciculations: Absent
Muscle wasting: No
Weakness: Proximal > Distal
Reflexes: Normal
Sensation: Normal
What are some treatment options for MG?
Acetylcholinesterase antagonists
Plasma exchange
IV Ig
Immunosuppresion - Steroids or azathioprine
Thymectomy
In which type of NM disease are fasciculation common?
Anterior horn cell disease
(Motor neuron)
Which diseases would you attribute a ptosis to?
MG
Ocular nerve palsy
Thyroid eye disease
Which muscles are spared in motor neuron disease?
Ocular
In terms of location of symptoms, how do myopathy and neuropathy differ?
Myopathy - proximal
Neuropathy - distal
What is the classical constellation of signs typical of neuropathy in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?
Fasciculations: Infrequent
Muscle wasting: Late
Weakness: Distal>Proximal Localised
Reflexes: Reduced or absent early
Sensation: Glove and stocking
What is the tensilon test?
IV injection of short acting acetylcholinesterase antagonist
How does motor neuron disease progress?
Generally local muscle wasting followed by more diffuse muscular involvement
What are the most common causes of neuropathies?
DM
Alcohol/Drugs
Paraneoplastic
Hypothyroidism
What is the classical constellation of signs typical of motor neuron disease in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?
Fasciculations: Present
Muscle wasting: Early
Weakness: Diffuse, mixed picture
Reflexes: Increases or normal
Sensation: Normal
What is the best test for myopathies?
CK levels
What is the most common motor neuron disease?
Amyotrophic lateral sclerosis
What is the classical constellation of signs typical of neuromuscular junction pathologies in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?
Fasciculations: Absent
Muscle wasting: No
Weakness: Proximal>Distal, ptosis and eye muscles, fatiguable
Reflexes: Normal
Sensation: Normal
When is CK elevated?
Muscular damage
What is the key finding on physical examination of a MG patient?
Fatiguable muscle weakness
What symptoms do patients with neuromuscular disease present with?
Weakness
Gait clumsiness
Sensation loss
Diplopia
Fasciculations
Cramps
What is a strabismus?
Abnormal position of the eye eg lazy eye
What type of dementia is associated with motor neuron disease?
Fronto-temporal
What are the 4 types of neuromuscular disorders? Briefly describe them
Motor neuron disease - pathology of uppper or lower motor neurons
Myopathy - Pathology of muscle
Neuropathy - pathology of peripheral nerves
Disorders or neuromuscular junction
What are the pre and post synaptic disorders of the neuromuscular junction?
Pre - MG
MuSK Myasthenia
Post - Lambert Eaton myasthenic syndrome
- Botulism
