Pance: Neurology 3

Front 1

Myasthenia Gravis description

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blockage of neuromuscular transmission at acetylcholine receptors

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Myasthenia Gravis c/b

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antibodies form against acetylcholine nicotinic postsynaptic receptors at the neuromuscular junction of skeletal muscles

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Myasthenia Gravis onset

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Occurs at any age; MC: young ♀

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Myasthenia Gravis Risk Factors

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Assoc w/ Thymic tumor; Thyrotoxicosis; Rheumatoid Arthritis (RA); SLE

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Myasthenia Gravis early presentation

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Early: ptosis, diplopia, muscle fatigability after exercise

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Myasthenia Gravis LATE presentation

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dysarthria, dysphagia, proximal limb weakness; quadriparesis/quadriplegia; respiratory distress

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Ocular Myasthenia Gravis Risk Factor

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ocular MG: concomitant HYPERTHYROIDISM is most frequent; OCCULAR myasthenia gravis involves only EXTRAOCULAR muscles

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Ocular Myasthenia Gravis testing

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ice pack test (ie, placing ice over the lid): for assessing improvement in ptosis and diplopia in ocular MG > rationale = cooling might improve neuromuscular transmission / alternate theory = rest might be cause of improvement > BOTH the ICE test and the REST test are SENSITIVE and SPECIFIC in ocular MG

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Myasthenia Gravis labs/testing

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EDROPHONIUM or NEOSTIGMINE administration will cause improvement of symptoms; electrophysiology testing, serum levels of acetylcholine receptor antibodies

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Myasthenia Gravis imaging

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plain CXR: AP and Lateral views: may identify a thymoma as an anterior mediastinal mass; Chest CT: obtained to identify or r/o thymoma or thymic enlargement in ALL cases of MG

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Myasthenia Gravis dx

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anti–acetylcholine receptor (AChR) antibody (Ab) test is reliable for diagnosing autoimmune myasthenia gravis (MG); highly specific (as high as 100%)

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Myasthenia Gravis tx

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pyridostigmine (Mestinon) or neostigmine corticosteroids are useful for long-term tx; PLASMAPHEREISI: relieves symptoms; tx assoc dz if present

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Bell's palsy description

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Unilateral facial muscle weakness w/out evidence of neurologic disease or apparent cause

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Bell's palsy c/b

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precise pathophysiology of Bell palsy remains an area of debate; popular theory proposes that edema and ischemia result in compression of the facial nerve within this bony canal

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Bell's palsy Risk Factors

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Herpes zoster, Lyme dz, Cancer, DM, Sarcoid, Trauma, Cholesteatoma, Viral infection

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Bell's palsy onset

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young women aged 10-19 years; third trimester pregnancy = 3.3 x ↑risk; Peak = 20 and 40 years & elderly 70-80 years

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Bell's palsy presentation

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acute onset (few hours), UNIlateral, +/- pain in/behind ipsilateral ear prior to facial weakness; +/- taste impairment, lacrimation impairment; hyperacusis (over-sensitivity to certain frequency and volume ranges of sound)

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Bell's palsy labs

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r/o assoc dz: Herpes zoster, Lyme dz, Cancer, DM, Sarcoid, Trauma, Cholesteatoma, Viral infection labs: RPR(VDRL), HIV, CBC, ESR, Thyroid function test, serum glucose, CSF

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Bell's palsy imaging

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CT if there are other associated physical findings or if the paresis is progressive and unremitting; MRI: useful as a means of excluding other pathologies

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Bell's palsy dx

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Clinical H&P: If the clinical findings are doubtful or if paralysis lasts longer than 6-8 weeks > r/o assoc dz

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Bell's palsy goals of tx

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goals of treatment are to improve facial nerve (seventh cranial nerve) function and reduce neuronal damage

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Bell's palsy tx

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most resolve spontaneously: Oral prednisone w/ acyclovir started soon after onset, improves complete recovery
Guillain-Barre Syndrome description - demyelinating neuropathy with ascending weakness

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Guillain-Barre Syndrome c/b

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Idiopathic acute or subacute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes; following infection, immunization or surgical procedure; associated w/ campylobacter jejuni enteritis

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Guillain-Barre Syndrome onset

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all age groups; male-to-female ratio of 1.5:1; no racial preponderance exists

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Guillain-Barre Syndrome risk factors

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vaccines (GBS, influenza A&B), Epstein-Barr virus, CMV, frequent penicillin and antimotility drug use

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Guillain-Barre Syndrome presentation

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Symmetrical lower extremity weakness, DTR decreased or absent; ↓sensation; AUTONOMIC DYSFUNCTION: tachycardia, ↓B/P, sweating, impaired pulmonary function, ↓sphincter tone, paralytic ileus; respiratory paralysis

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Guillain-Barre Syndrome labs/dx

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electrophysiology (slowing of the motor and sensory NCV; CSF: ↑proteins & normal cell count

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Guillain-Barre Syndrome tx

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hospitalization (cardio/resp support); plasmapheresis (improves prognosis, morbidity & mortality); IV immunoglobulin (preferred instead of plasmapheresis if cardiovascular instability or in children)

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Diabetic Peripheral Neuropathy description

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Mixed polyneuropathy in majority of cases (70%) and the remainder are sensory; can affect any peripheral nerve including Cranial Nerves (CN) / most common complication of diabetes mellitus (DM)

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Diabetic Peripheral Neuropathy c/b

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vascular insufficiency or nerve infarct associated w/ hyperglycemia

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Diabetic Peripheral Neuropathy SENSORY presentation

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Negative or positive, diffuse or focal; insidious onset and showing a stocking-and-glove distribution in the distal extremities

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Diabetic Peripheral Neuropathy MOTOR presentation

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Distal, proximal, or more focal weakness; +/- occurring with sensory neuropathy (sensorimotor neuropathy)

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Diabetic Peripheral Neuropathy AUTONOMIC presentation

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postural hypotension, cardiac arrhythmias, impaired thermoregulatory sweating, bowel/bladder/sexual/gastric dysfunction

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Diabetic Peripheral Neuropathy PE

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Peripheral neuro exam: Gross light touch & pinprick; vibratory sense; DTR; strength and muscle atrophy; dorsal pedal and posterior tibial pulses; skin assessment; Tinel testing; cranial nerve testing

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Diabetic Peripheral Neuropathy labs

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Fasting plasma glucose and hemoglobin A1c; Serial Nerve Conduction testing (progression & dx); OTHER labs (r/o other causes)

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Diabetic Peripheral Neuropathy imaging

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MRI of the cervical, thoracic, and/or lumbar regions to exclude other cause for sx mimicking diabetic neuropathy

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Diabetic Peripheral Neuropathy tx

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tight control of serum glucose

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Diabetic Peripheral Neuropathy Rx for stabbing pain

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phenytoin (Dilantin); mexiletine (Mexitil); carbamazepine (Tegretol)

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Diabetic Peripheral Neuropathy Rx for deep aching pain

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amitriptyline (Elavil); fluphenazine (prolixin)

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Diabetic Peripheral Neuropathy tx for hypotension

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NaCL; TED stockings; Rx: fludrocortisones; midodrine (Proamatine)