Neuromuscular Flashcards
hereditary Inclusion Body Myopathy (IBM).
Hereditary IBM is most commonly autosomal recessive, affects DISTAL lower extremity muscles first, and spares the quadriceps even in advanced disease (fingers difficulty pinching, buttoning)
Interestingly, not responsive to steroids.
IBM clinically presents with asymmetric, slowly progressive, painless weakness in proximal and distal muscles. It is associated with a polyneuropathy. It is not responsive to pharmacologic intervention, such as steroids and immunosuppressive agents. Treatment choices primarily involve appropriate rehabilitation interventions, which include provision of assistive device
Myotonic dystrophy
Myotonic Dystrophy (DM) typically follows an autosomal dominant inheritance pattern and is associated with multiple psychological disorders, including obsessive compulsive disorder, depression, and avoidant personality disorder. Testicular atrophy and cardiac abnormalities are common in DM.
Cataracts are common in myotonic muscular dystrophy (MMD). The heart is also frequently involved, particularly the electrical conduction system. MMD patients rarely get significant scoliosis. Excessive fatigue and daytime sleepiness, not insomnia, are common in both MMD1 and MMD2. Blood pressure is typically low in MMD, particularly MMD1.
Myotonic muscular dystrophy (MMD) is characterized clinically by progressive, predomi-nantly distal, muscle weakness and myotonia. Associated findings include frontal bald-ness, gonadal atrophy, cataracts, and cardiac dysrhythmias. Most persons affected with MMD will maintain the ability to ambulate throughout their lives, although gait patterns will be abnormal. However, fine motor function in the hands may be severely impaired because of difficulty with muscle relaxation (myotonia) and weakness.
Axontemesis vs Neurapraxia , how many days afteR?
Wallerian degeneration takes 9 days to occur typically, and neurapraxia should be distinguishable from axonotmesis after this time. While compound muscle action potential can still be elicited distal to the lesion in a neurapraxic injury, it will not be present if axonotmesis has occurred due to Wallerian degeneration.
CSF in AIDP and CIDP
elevated protein; also In both AIDP and CIDP, both conduction slowing and conduction block are typical on electrodiagnostic testing.
Miller Fisher Syndrome
areflexia, external ophthalmoparesis, and ataxia
Anti-GQ1b antibodies are present in about 95% of patients with MFS.
motor conduction velocities and amplitudes may be nearly normal, there is usually a marked reduction in sensory nerve conduction amplitudes (more so in the upper limbs as compared with the lower limbs).
Anti-GM1 antibodies are typically associated with
Multifocal Motor Neuropathy
Multifocal motor neuropathy vs ALS
Both ALS and MMN present with asymmetric, progressive weakness in the absence of sensory loss. On initial evaluation, it may be difficult to distinguish the two based on clinical presentation. Anti-GM1 antibodies are present in MMN, but not in ALS. The combination of both Upper and Lower Motor Neuron findings is seen in ALS, where MMN usually involves only Lower Motor Neuron findings on physical examination. Conduction block on nerve conduction studies is a hallmark of MMN due to its demyelinating pathology. In contrast, ALS involves motor neuron degeneration, and conduction block should not be present. Unlike ALS, MMN is responsive to IVIG.
FYI : if ALS runs in family then inheritance pattern is Autosomal DOMINANT
Which of the following is most useful in determining the prognosis of patients with Guillain-Barré syndrome (GBS)?
In GBS, some factors have been shown to have prognostic value, including CMAP amplitudes of < 20% of the lower limit of normal on nerve conduction studies, the magnitude of increase in serum IgG levels 2 weeks after IVIG treatment, nerve root enhancement on MRI, and elevated CSF levels of axonal neurofilament (NfH).
Spinal cord injury in HIV
Vacuolar myelopathy is the most common cause of spinal cord dysfunction in HIV. It occurs in 11-22% of AIDS cases. Presentation involves paraparesis, ataxia, posterior column sensory loss, spasticity, and neurogenic bowel and bladder.
Pompe’s disease
Pompe’s disease is an ACID MALTASE DEFICIENCY. The resultant accumulation of glycogen leads to irreversible damage of skeletal, respiratory, and cardiac muscle tissue resulting in permanent disability and death.
Most common type of MS
relapsing remitting
parkinsons plus syndromes
Multiple system atrophy (Shy-Drager syndrome)
Supranuclear palsy
Olivopontocerebellar atrophy
Striatonigral degeneration
All the symptoms involved suggest cerebellar impairment, including the ataxia and dysarthria. Multiple system atrophy has more autonomic involvement, including orthostatic hypotension, thermoregulatory dysfunction, and impotence. Choice (D) is harder to differentiate from primary Parkinson’s, but is noted to have dystonia, with general absence of tremor. Choice (A) is associated with Parkinson’s along with vertical gaze palsy.
poor prognostic factor MS
Ataxia and tremor have a poorer prognosis for MS patients. All other choices are good prognostic factors.
Optic neuritis is inflammation of the optic nerve. It may cause sudden, reduced vision in the affected eye and is considered a good prognostic factor when presenting at onset in MS patients.
Ergot deriv
Non ergot deriv
bromocriptine
Non-Ergot derivatives include ropinirole (Requip) and pramipexole (Mirapex).
A patient presents to the emergency department with loss of muscle strength in his right upper and lower extremities. He has loss of proprioception and position sense on his right side and has left-side tongue deviation. What does he most likely have?
medial medullary syndrome
