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Neurology > Neuromuscular, Neurocutaneous, and spinal cord diseases > Flashcards

Neuromuscular, Neurocutaneous, and spinal cord diseases Flashcards

1
Q

What is pathophysiology of myasthenia gravis

A

Autoantibodies against nicotinic acetylcholine receptors at NMJ junction, fatigue through day, better with rest

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2
Q

What is the most common and most common initial symptoms, and what can it be limited to in elderly ppl for myasthenia gravis

A

Extraocular eye muscles, with ptosis, diplopia, and blurred vision

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3
Q

What goes weak and what is preserved in myasthenia gravis

A

Skeletal muscles weak, sensation and reflexes preserved

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4
Q

How are limb muscles affected in myasthenia gravis

A

Proximal and Asymmetric

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5
Q

Other symptoms of myasthenia gravis besides eye symptoms

A

Dysarthria, dysphagia, and generalized weakness

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6
Q

What is myasthenia crisis and what percent of patients does it occur in?

A

An exacerbation of myasthenia gravis with respiratory muscles affected, requiring intubation15%

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7
Q

Four diagnostic modalities of myasthenia gravis

A
  1. ) Acetylcholine antibody test - specific, 20% beat it
  2. ) EMG - shows decremental conduction of motor nerves
  3. ) Edrophonium test - acetylcholine esterase inhibitor, improves symptoms but not used
  4. ) CT to see if thymoma is present - 75% have abnormal histology, 15% have actual thymoma
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8
Q

Treatment for myasthenia gravis (Five things)

A
  1. ) AChE inhibitor - symptoms only - pyridostigmine
  2. ) Thymectomy - symptoms and remission, do even when there is no thymoma
  3. ) Immunosuppressive drugs - corticosteroids second line, azathioprine and cyclosporine third line
  4. ) Plasmapharesis - removes antibodies, last resort
  5. ) IV immunoglobulins - myasthenia crisis
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9
Q

What is the forced vital capacity indication for intubation for patients with myasthenia gravis

A

Less than 15ml/kg, unless crisis, in which case do not wait

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10
Q

Where are lambert-eaton’s autoantibodies directed towards

A

Presynaptic calcium channels

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11
Q

Difference between symptoms of myasthenia gravis and lambert eaton

A

Lambert Eaton - improves with stimulation, hyporeflexia

Myasthenia Gravis - diminishes with stimulation, reflexes preserved

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12
Q

What cancer is lambert-eaton associated with

A

Small cell lung cancer

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13
Q

Gene mutation and result of duchenne’s mulscular dystrophy

A

X-linked recessive - no dystrophin, no inflammation

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14
Q

Progression of muscular weakness in duchenne’s muscular dystrophy

A

Starts proximal and symmetric in children (pelvis girdle), progresses distally

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15
Q

Two distinguishing features of duchenne’s muscular dystrophy

A
  1. ) Gower’s maneuver - Patients use hands to get up

2. ) Enlarged calf muscles - first true hypertrophy, then pseudohypertrophy as fat replaces muscle

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16
Q

Final complications of duchenne’s muscular dystrophy

A

Wheelchair bound, respiratory failure, and death in third decade

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17
Q

What diagnostic lab is distinguished in duchenne’s, and what can you use to test for duchenne’s

A

Serum creatinine phosphokinase

Definitive: DNA testing

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18
Q

What is the treatment used for duchenne’s muscular dystrophy

A

Prednisone - 5 years and older with declining motor skills

Surgery - fixes scoliosis, once wheelchair bound

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19
Q

Similaries and differences for becker’s muscular dystrophy vs. duchenne’s muscular dystrophy

A

X-linked recessive too, less common, later onset and less severe because some dystrophin present

20
Q

What two other hereditary diseases can cause muscle weakness

A

Mitochondrial disorders: Ragged red muscle fibers

Glycogen storage diseases: Mcardle’s - muscle cramping after exercise because no glycogen phosphorylase

21
Q

What three things can exacerbate myasthenia gravis

A
  1. ) Beta blockers
  2. ) Antibiotics - aminoglycosides and tetracyclines
  3. ) Antiarrhythmics - quinidine, procainamide, and lidocaine
22
Q

What are the genetics of neurofibromatosis type 1

A

Autosomal dominant

23
Q

What are the clinical features of neurofibromatosis type 1

A
  1. ) Cafe au lait spots - pigmented spots
  2. ) Neurofibromas - benign tumors of nerve sheath - treat by surgery
  3. ) CNS tumors - gliomas, meningiomas
  4. ) Axillary or inguinal freckling
  5. ) Lisch nodules - iris hamartomas (dendritic melanocytes in iris)
  6. ) Bony lesions
24
Q

What are the complications of neurofibromatosis type 1

A
  1. ) Scoliosis
  2. ) Pheochromocytoma
  3. ) optic nerve glioma
  4. ) Renal artery stenosis
  5. ) Bone erosion
25
Q

What are the clinical features of neurofibromatosis type 2

A
  1. ) Bilateral acoustic neuromas - classic
  2. ) Multiple meningiomas
  3. ) Cafe au lait spots
  4. ) Neurofibromas
  5. ) Cataracts
26
Q

What are the clinical features of tuberous sclerosis

A
  1. ) Cognitive impairment
  2. ) Epilepsy
  3. ) Skin lesions (angiofibromas - papules with fibrous tissue, adenoma sebaceum)
27
Q

What are the complications of tuberous sclerosis

A
  1. ) Retinal hamartoma - can lead to retinal detachment
  2. ) Renal angiomyolipoma - could hemorrhage
  3. ) Rhabdomyoma
28
Q

What is Sturge-Weber syndrome classic visible features

A

Facial vascular nevi (port wine stain), epilepsy, mental retardation

29
Q

What should you treat in sturge weber syndrome

A

Epilepsy

30
Q

What is the see pathologic feature of sturge-weber syndrome

A

Capillary angiomatoses of pia mater - knots of capillaries

31
Q

What are important features of von hippel-lindau disease

A

Cavernous hemangiomas of brain/brainstem, renal angiomas, and cysts in multiple organs

32
Q

What are two complications of von hippel lindau disease

A
  1. ) Renal cell carcinoma

2. )Pheochromocytoma (Think about close proximity of these)

33
Q

What is the pathophysiology of syringomyelia

A

Central cavitation of cervical cord, affecting anterior commissure (pain and temperature) at C8-T1

34
Q

What is the nerve progression of syringomyelia

A
  1. ) Pain/temperature in upper extremities (C8-T1)
  2. ) muscle weakness and atrophy (LMN’s)
  3. ) Hypothalamus tract - sympathetic - horners syndrome
35
Q

What is preserved in syringomyelia

A

Touch

36
Q

What imaging modality should you use to diagnose syringomyelia?

A

MRI, consult with neurosurgery

37
Q

What causes syringomyelia

A

Arnold chiari syndrome or trauma (including infection)

38
Q

What are causes of brown-sequard syndrome

A

Trauma, crush injury, tumors, abscesses

39
Q

What are the clinical features of brown-sequard syndrome?

A 
Contralateral loss of pain and temperature
Ipsilateral hemiparesis (corticospinal)
Ipsilateral loss of position/vibration (dorsal columns)
40
Q

What is the pathophysiology of horners syndrome?

A

Cervical sympathetic nerves

  1. ) Preganglionic - more workup required because central
  2. ) Postganglionic - past superior cervical ganglion
41
Q

What are the clinical features of horners syndrome

A

Ptosis, miosis, anhidrosis (all ipsilateral)

Levator palpebrae still intact

42
Q

Five causes of horners syndrome

A
  1. ) Idiopathic (most cases)
  2. ) Pancoast tumor
  3. ) Internal carotid dissection
  4. ) Brainstem stroke
  5. ) Neck trauma
43
Q

What is the pathophysiology of poliomyelitis

A

Caused by poliovirus; anterior horn cells - LMNs affected

44
Q

What is affected and what is spared in poliomyelitis

A

Assymetric muscle weakness (legs more), normal sensation

45
Q

What two cranial nerves are affected by poliomyelitis

A

CN 9 and 10 - cardiovascular and respiratory problems

46
Q

Treatment or vaccination for poliomyelitis

A

Vaccination

Neurology (9 decks)

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