Disorders of Anterior Horn/Peripheral Nerves & NMJ Flashcards
Signs/symptoms of upper motor neuron disorders
- spastic tone
- hyperactive tendon reflexes
- pathologic reflexes (Babinski)
- emotional lability (inappropriate laughing and crying)
Signs/symptoms of lower motor neuron disorders
- muscle atrophy
- fasciculation
- diminished tone (flaccidity)
- reduced or absent reflexes
Signs/symptoms of non-motor (sensory or autonomic) peripheral nerve disorders
- sensory
- numbness
- pain
- altered sensation
- autonomic
- bowel, bladder disturbance
- altered sweating, HR, BP
Conditions w/rapidly developing muscle weakness
- NMJ disorders
- myasthenia gravis
- botulism
- organophosphate poisoning
- acute demyelination
- Guillain Barre
- electrolyte disturbance
- toxic myopathies
Electrodiagnostic studies in peripheral nerve disorders
- nerve conduction studies
- demyelinating ==> slow NCS
- vs. axonal neuropathy ==> decreased amplitude NCS
- needle electromyography (EMG)
- neuropathic (anterior horn/peripheral nerve) ==> loss of whole motor unit
- vs. myopathic ==> drop out of biers w/in motor unit
Anterior Horn cell disorders (examples)
Amyotrophic lateral sclerosis*
Spinal muscular atrophy
Poliomyelitis and West Nile virus
Radiculopathies & plexopathies examples
Cervical (neck) and lumbosacral (back) nerve root compression (common)
Brachial and lumbosacral plexopathies
Peripheral neuropathies (mononeuropathy examples)
Carpal tunnel syndrome - entrapment of median nerve at wrist
Ulnar nerve palsy –entrapment at elbow
Peroneal palsy – entrapment at the head of the fibula
Peripheral neuropathies (polyneuropathy examples)
Hereditary – Charcot Marie Tooth disease*
Systemic disease – diabetes*, immune disorders etc
Vitamin deficiency – B12 deficiency, etc.
Exogenous toxins – alcohol, chemotherapy, etc.
NMJ disorders
Myasthenia gravis*
Botulism
Organophosphate poisoning
Myopathies examples
Muscular dystrophies – Duchenne/Becker*
Myotonic disorders
Inflammatory myopathies – polymyosits, dermatomyositis
Metabolic myopathies – glycogen storage, lipid myopathy
Endocrine/toxic myopathies
ALS signs/symptoms
- progressive weakness & wasting
- coexisting: spasticity & hyperreflexia
- asymmetric limb weakness + fasciculations
- foot drop or hand deformity possible
- speech may be slurred/spastic
- diaphragm weakness ==> decreased breathing capacity + impaired swalling ==> aspiration pneumo or respiratory insufficiency
ALS pathology
- anterior horn cell disorder
- brainstem + lower motor neuron degeneration ==> weakness/wasting
- UMN degeneration ==> hyperreflexia/spasticity
*
Sx management of difficulty speaking, swallowing, breathing
- alternative communication devices
- feeding tube
- positive pressure ventilation
- mask
- tracheostomy
Charcot Marie Tooth Disease types
- CMT Type 1:
- slow nerve conduction velocities
- pathological evidence of hypertrophic demyelinating neuropathy
- CMT Type 2:
- normal nerve conduction velocity
- axonal degeneration