Neuro - Presentations

MG patho

Antibodies attack ACh receptors

Decrease in ACh receptor sites at the NMJ

This prevents ACh molecules from attaching and stimulating muscle contraction

Clinical features are weakness and fatigue of muscles that occur and increase during repeated use and is better following rest and sleep

MG

Most common first complaint for MG

Diplopia and ptosis (because eyelid muscle contract to make us blink sm, so often fatigue quickly)

Other sxs associated with MG

Snarling expression

Speech - sound nasally

Hard to swallow

Respiratory crisis

in 85% of patients with MG, weakness becomes genealized, affecting limb muscles, limb weakness is often _____

proximal and assymetric

Despite muscle weakness in MG, ______ are preserved and ____ is norma

DTR

Sensation

Anticholinesterase Test

Dx for MG

Give drugs to inhibit anticholinesterase and muscle strength improves if they have MG

Erdophonium test

Dx for MG

If improvement occurs --> Positive test

This test is good for MG pts with clinical findings suggestive of MG with negative aB, negative electrodiagnostic & icepack test

Edrophonium test

Electric/Nerve stimulation test

stop meds before test

electric shock delivered to nerves & AP recorded

Normal = AP does not change

MG = amplitude decreases by 10-15%

In the serum of 80% of MG patients

Antiacetylcholine Receptor antibodies

Ice pack test

for MG

Ice pack on ptosis - improvement suggest MG

Treatment for MG

Pyridostigamine (anticholinesterase inhibitor)**

Thymectomy - to remove thyoma or for treatment

Immunosuppresive drugs (many)

MG Crisis

Pts need vent support in ICU 2/2 diaphragm and intercostal weekness

MG - tx for immediate improvement for severe weakness

Plasmapheresis

____ is involved in approx 75% of MG patients

thymus

MuSK associated MG

Rare subtype of MG

Acute onset affecting facial bulbar muscles

CN 9,10,11,12 effected

Early respiratory crisis are frequent

Most common form of progressive motor disease that affects cells in the brain and spinal cord

ALS

ALS - where is muscle atrophy seen when dx

Clinical exam & biopsy

What are the main dysfunctions of ALS

LMN dysfunction = assymetric weakeness, usually distal first

UMN dysfunction = hyperactivity of DTRs, spacitiy, + babinkski

Muscle biopsy shows small, angular fibers consistent with neurogenic atrophy

ALS

EMG shows sharps, fasciulations, and/or fibrillatiom

ALS

Treatment of ALS

-Rilutek (inhibits glutamate release and antagonizes its receptor)

No real treatment for underlying cause

X linked recessive disorder of progressive weakness

DMD

Less severe form of X linke recessive MD that resukts for allelic defects in the same gene that is responsible for DMD

Becker Muscular Dystrophy

Normal mile stones until the child begins to walk

DMD

When do symptoms become apparent in DMD

2-5yo

Proximal or distal weakness MC in DMD

Proximal

Common posture in DMD

Gower sign

Lordotic posture

Psuedohypertrophy, toe walking, waddling gait, frequent falls

DMD

What lab is typically elevated in DMD

Serum Creatinine

Muscle biopsy shows muscle fibers of varying size as well as small groups of necrotic and regenerating fibers

DMD

Treatment of DMD

Steroids

PT

goal is to minimize progression and mantain mobiloity

Prognosis of DMD

Death in 20s

BMD maybe 40s

Most cases involve spasticity of limbs, hyperreflexia, presistant primitive reflex and poor head and trunk control

CP

Multiple milestones not met as per pediatrician

CP

Dx the full extent of CP with

MRI

CP tx

OT, PT, Speech

Treat symptoms

acute, polyradiculopathy that attacks and degenerates the peripheral nerves of the body

GB

Most cases of GB usu occur from

Acure respiratory or infectious processes

MC in US = GI infxn 2/2 Campylobacter**

What type of paralysis in GB

Rapid, ascending areflexic motor paralysis

Ascending symmetric areflexic motor paralysiks with assoaciated tinging and dysehtesia

GB

DTRs in GB?

Lost

Motor or sensory greater in GB?

Motor

LP shows elevated protein in

GB

LP in GB

Elevated protein

What do nerve conduction studies show in GB

Demylination

Treatment of GB

Plasma exchange

'IVIG

Muscle biopsy shows ragged red fibers c/w mitochondrial abnormalities

Myopathy

Drug induced myopathy is MCC by

Statins & Glucocorticoids

Co use of statins with ____ and ____ increase risk of drug induced myopathy

fibrates and cylosporine

Group if disorders that present with symmetrical and proximal weakness that worsens over several weeks to months

Polymyositis

Polymyositis - what is commonly involved

heart, lung, joint

increased risk for cancer

Genetic problems in the mitochondria and the production of ATP. Buildup of lactic acid from this causes muscle fatigue and damaged muscle and nerve tissue

Mitochondrial myopath y

Mitochondrial myopathy - weakness most prominent in

the muscles that control the eye movemnet and eyelids

Disorder of the extrapyramidal system that predom effects the dopamine cells

Parkimsoms

Parkinsons is the loss of _____ in the ____ and the presence of _____

loss of pigmeneted dopaminergic neurons in the substantia nigra

pressence of lewy bodies

Loss of neurons in parkinsoms is predominetly in the

Ventral lateral substantia nigra

Approx ____% of the dopaminergic neruons are lost before motor signs of parkinsons emerge

60-80%

4 cardinal features of parkinson

Tremor at rest

Rigidity

Bradykinesia

Gait distruvance with postural instability

Usu presenting complaint of parkinsons

Tremor

Pill rolling is seen with

Parkinosn

Types of rigidity in parkinsons

Lead-pipe type or cogwheel with tremor

Initial treatment for Parkinson's

Dopamine agonist - levodopa

___ can be added to levodopa in Parkinsons to lower the dose and SE

Carbidopa

Look over parkinsons drugs*

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