LEC-10 Neuromuscular Diseases Flashcards
Lambert Eaton myasthenic syndrome is typically a paraneoplastic syndrome, with ______________________ cancer being the most commonly associated malignancy.
Small cell lung cancer
How do patients with peripheral nerve disease present?
I. Numbness II. Impaired vibration perception III. Atrophy of small muscles in hands and feet IV. Ataxia V. Pain
Reduced muscle tone, depressed tendon and superficial reflexes, along with weakness that is patchy in distribution may signify a problem or disorder at the _________________.
Neuromuscular junction
______________________ is the loss of motor neurons in the anterior horn and corticospinal tracts. It is most commonly caused by a loss of the enzyme superoxide dismutase 1.
Amyotrophic Lateral Sclerosis, ALS or Lou Gehrig’s disease
ALS may be treated by administration of the drug _____________.
Riluzole
Lou Gehrig disease = riLOUzole
________________ is the autosomal recessive, most common form of an inherited motor neuron disease.
Spinal muscular atrophy
Spinal muscular atrophy that onsets in infancy is known as (Werdnig Hoffman disease/Kugelberg Welander disease).
Werdnig Hoffman disease
Spinal muscular atrophy that onsets in adolescence is known as (Werdnig Hoffman disease/Kugelberg Welander disease).
Kugelberg Welander disease
A peripheral nerve disorder in which the pattern of weakness conforms to the distribution of a single nerve is known as a(n) __________________.
Mononeuropathy
E.g. Carpal tunnel - The weakness follows the distribution of the median nerve.
A peripheral nerve disorder in which multiple nerves are affected in a seemingly random pattern, has acute onset, and is frequently painful to the patient is known as ________________.
Mononeuritis multiplex
(T/F) Sensory symptoms in a polyneuropathy first start in the feet and move proximally. Hand symptoms then appear when lower extremity symptoms reach the knees.
True
Guillain Barre syndrome is an example of a (mononeuropathy/polyneuropathy).
Polyneuropathy.
What are the three types of Myasthenia Gravis antibody to ACh receptor?
I. Binding
II. Blocking
III. Modulating
ACh receptor antibody is detected in __-__% of patients with generalized Myasthenia Gravis.
90-95%
In patients with myasthenia gravis, symptoms tend to (improve/worsen) with exercise.
Worsen
_____________________ drugs may be given to patients with myasthenia gravis.
Acetylcholinesterase inhibitor drugs
(T/F) One of the primary distinguishing features between botulism and Myasthenia Gravis is that botulism pathology affects the pupils, while Myasthenia Gravis does not.
True.
Lambert Eaton myasthenic syndrome is a (presynaptic/postsynaptic) complication, while Myasthenia Gravis is a (presynaptic/postsynaptic) complication.
Lambert Eaton myasthenic syndrome - Presynaptic
Myasthenia Gravis - Postsynaptic
Antibodies in Lambert Eaton myasthenic syndrome target ___________________. This impedes the release of vesicles containing ACh.
voltage-gated calcium channels
(Duchenne muscular dystrophy/Myotonic dystrophy) is the most common of the dystrophies.
Myotonic dystrophy
(T/F) Myotonic dystrophy causes failure of the muscle to relax after contraction, often resulting in prolonged hand contractions or hands “getting stuck”.
True.
How do patients with upper motor neuron lesions present?
Weakness or paralysis Spasticity (increased muscle tone) Increased reflexes An extensor plantar (Babinski) response Loss of superficial abdominal reflexes Little, if any, muscle atrophy
How do patients with lower motor neuron lesions present?
Weakness or paralysis (at level of anterior horn)
Wasting and fasciculations (not immediate after insult)
Hypotonia (flaccidity); immediately after insult
Normal abdominal and plantar reflexes
No increased tone
No pathological reflexes (no Babinski’s sign)
