Name that Nerve!
This nerve innervates the deltoid and teres minor muscles, making it essential for shoulder abduction beyond the first 15 degrees.
What is the axillary nerve?
An infant born after a difficult breech delivery develops an arm held in adduction and internal rotation, with the elbow extended and forearm pronated—the classic “waiter’s tip” posture. This injury involves the upper roots/trunk of this plexus.
What is an upper trunk (C5–C6) brachial plexopathy (Erb palsy)?
These two respiratory measurements are used to predict neuromuscular respiratory failure and often precede hypoxemia.
What are FVC and NIF?
(Functional vital capacity, threshold <20 mL/kg)
(Negative inspiratory force, threshold <-30 cm H2O)
This X-linked motor neuron disease, caused by a CAG trinucleotide repeat expansion in the androgen receptor gene, classically presents in adult men with proximal weakness, muscle cramps, fasciculations, gynecomastia, and hyporeflexia.
What is Kennedy's Disease/ Spinal and bulbar Muscular Atrophy
This monoclonal antibody against CD20 is used off label for multiple neuromuscular disorders as an adjunct/ steroid-sparing therapy.
What is rituximab (rituxan)?
Cost: $9000-13,000/ 1g dose
Injury to this nerve causes weakness of thumb opposition, abduction, and flexion, producing the classic "ape hand" deformity.
What is the median nerve?
This electrodiagnostic finding favors brachial plexopathy over cervical radiculopathy.
What are normal paraspinal muscles on needle EMG?
In a weak myasthenic patient, strength in this muscle group tracks diaphragm strength.
What is neck flexion?
This autosomal recessive lysosomal storage disorder is caused by deficiency of acid α-glucosidase, leading to glycogen accumulation in skeletal and cardiac muscle. Adult-onset disease often presents with limb-girdle and axial weakness, respiratory muscle involvement, and a disproportionately severe diaphragmatic weakness.
What is Pompe's Disease/ late-onset glycogen storage disease type II
This treatment for MG and GBS that comes from pooled donors carries a risk of this neurologic side effect?
What is aseptic meningitis from IVIG?
Cost of IVIG: ~$11,000-15,000 per dose (2g/kg for a 75 kg adult)
-Usually delayed reaction (6-48 hours after infusion)
-More common in patients being treated for GBS
-May be due to hypertensitivity reactions (immune complex mediated//type 3 and cell mediated//type 4) of the leptomeninges
Following prolonged lithotomy positioning, a patient has weakness of hip abduction with a positive Trendelenburg sign but normal knee extension and intact sensation over the lateral thigh.
What is the superior gluteal nerve?
After pelvic radiation, a patient develops painless progressive leg weakness. EMG demonstrates myokymic discharges, a finding classically associated with this diagnosis.
What is radiation-induced lumbosacral plexopathy?
This extremely sensitive test for myasthenia gravis measures the variation in the time interval between the two action potentials of the same motor unit.
What is single fiber EMG?
(jitter)
This inherited peripheral neuropathy classically causes slowly progressive distal weakness and atrophy, foot deformities such as pes cavus, and distal sensory loss. Nerve conduction studies can help distinguish its demyelinating and axonal subtypes.
What is Charcot Marie Tooth Disease?
This medication is the first neonatal Fc receptor antagonist approved for the treatment of gMG
What is intravenous efgartigimod alfa (Vyvgart)
Cost: $7900/ dose
This nerve is responsible for ankle dorsiflexion and toe extension. It is commonly injured at the fibular neck, producing foot drop while largely sparing plantar flexion and inversion.
What is the deep fibular (deep peroneal) nerve?
A rare syndrome that may occur in otherwise normal healthy individuals with sudden, rather abrupt, unilateral shoulder pain and weakness/sensory changes to the shoulder girdle and proximal upper limb muscles.
What is Parsonage-Turner Syndrome (idiopathic brachial plexopathy or neuralgic amyotrophy)?
This clinical exam sign for MG is seen when the upper eyelid briefly overshoots or twitches upward before dropping back down when a person moves their eyes from looking down to looking straight ahead.
What is Cogan's lid twitch?
(https://www.instagram.com/reel/DZHuxmsqEM9/?hl=en)--> link for video
Name the gene and type of mutation involved in this inherited syndrome of teenage/ adult onset proximal leg and hip girdle weakness associated with calf pseudo-hypertrophy and elevations in CK.
What is the in-frame deletion or duplication of the dystrophin gene?
(Disease= Becker's Muscular Dystrophy)
This one-time gene therapy for spinal muscular atrophy uses an adeno-associated virus serotype 9 vector to deliver a functional copy of the SMN1 gene, allowing motor neurons to produce the survival motor neuron protein.
What is Zolgensma (onasemnogene abeparvovec)?
Cost: $2.1 million for a single dose
A patient can flex the DIP joints of the ring and little fingers but cannot make the "OK" sign because of weakness of flexor pollicis longus and the index finger FDP. Sensation is completely intact.
What is the anterior interosseous nerve?
Weakness of gluteus medius, tensor fascia lata, tibialis anterior, tibialis posterior, and hamstrings with sparing of iliopsoas best localizes to this lesion.
What is a lumbosacral trunk (L4–L5) plexopathy?
The Japanese clinical guideline from 2022 classifies MG into these 6 clinical subtypes--> points for whoever can name the most :D
What are:
1.Ocular MG
2.Generalized early-onset MG
3. Generalized late-onset MG
4. Generalized thymoma-associated MG
5. Generalized MuSK antibody-positive MG
6. Generalized seronegative MG (g-SNMG): Generalized MG without detectable AChR or MuSK antibodies (patients with LRP4-positive generalized MG are also grouped here)
A young adult has slowly progressive, asymmetric proximal and distal weakness, scapular winging, and preserved sensation. CK is mildly elevated. Genetic testing identifies a D4Z4 repeat contraction on chromosome 4q35.
What is facioscapulohumeral muscular dystrophy (FSHD)?
This siRNA medication is used for the treatment of polyneuropathy in people with hereditary transthyretin-mediated amyloidosis.
What is patisiran?
Cost: $13,000 per dose, $450-670 annually