Disorders of Motor System

Hypokinesia or Bradykinesia
aka Motor Slowness

The glabellar reflex. When you tap on a baby's glabellar (the area between the eyes) the baby will blink every time. As an adult the frontal cortex overrides this reflex. This override is lost in Parkinson's patients.

Restore balance between dopaminergic and cholinergic influences on GABA.

Dopamine inhibits the release of GABA

Acetylcholine increases the release of GABA

There is a disturbance in the balance of acetylcholine and dopamine for influencing the release of GABA.

The dopamine secreting cells in the substantia nigra degenerate and there is too much acetylcholine relative to dopamine which leads to too much GABA.

Tremor - Pill Rolling
Hypokinesia
Abnormal Gait
Rigidity/Increased Tone
Blepharoclonus
Myerson's Sign
Depression
Decreased sense of smell
Increased sweating

1. Block with anticholinergic drugs
2. Enhance dopaminergic transmission

1. Increase dopamine levels
2. Stimulate dopamine receptors
(dopamine agonists)
3. Decrease dopamine metabolism

Levodopa
Carbidopa
Sinemet (levodopa with carbidopa)
Amantadine
Stalevo (levodopa, carbidopa, entacapone)

A combo of levodopa with carbidopa that decreases the nausea caused by taking levodopa by itself.

An antiviral drug that was originally used to fight flu but was found to decrease Parkinson's symptoms. This is because it increases synthesis, release, or reuptake of dopamine.

Bromocriptine among others that we dont' need to memorize.

These are used in conjunction with levodopa, once levodopa stops working so will these.

MAOI-typeB (MAO type B breaks down dopamine)
COMT inhibitors (COMT breaks down levodopa and COMT inhibitors should be taken when levodopa is taken)

Because it is a combo of levodopa, carbidopa, and entacapone.

Levodopa by itself increases dopamine levels in the CNS but you get sick.

Levodopa with carbidopa increases dopamine without sickness, but COMT is still there to break down levodopa.

Entacapone inhibits COMT breaking down levodopa.

Anticholinergics

1. Deep brain stimulation
2. Thalamotomy
3. Pallidotomy

Implanting a pacemaker in the thalamus to control tremor

Bromocriptine among others that we dont' need to memorize.

These are used in conjunction with levodopa, once levodopa stops working so will these.

MAOI-typeB (MAO type B breaks down dopamine)
COMT inhibitors (COMT breaks down levodopa and COMT inhibitors should be taken when levodopa is taken)

Because it is a combo of levodopa, carbidopa, and entacapone.

Levodopa by itself increases dopamine levels in the CNS but you get sick.

Levodopa with carbidopa increases dopamine without sickness, but COMT is still there to break down levodopa.

Entacapone inhibits COMT breaking down levodopa.

Anticholinergics

1. Deep brain stimulation
2. Thalamotomy
3. Pallidotomy

Implanting a pacemaker in the thalamus to control tremor

Surgically putting a lesion in the thalamic nuclei

Surgically destroying part of the thalamus (golbus pallidus)

Implantation of human RPE cells which produce levodopa.

Autosomal dominant

In adulthood. 4-5th decade.

Cholinergic and GABA neurons are detroyed. This leads to excess dopamine levels relative to acetylcholine and low GABA (which is an inhibitory neurotransmitter)

Chorea: movement disturbance - hyperkinesia

Dementia: begins with irritability and later becomes obvious dementia.

Family Hx
Genetic testing

No cure
Tx muscle spasm (haloperidol)
Decrease dopamine (tetrabenazine)

It's autoimmune. Autoantibodies destroy the acetylcholine receptors in the post synaptic muscle membrane.

SLE
Hyperthyroidism

About 20 years old: thymus hyperplasia
50-60 years old: thymus tumor

The eyes have very small motor units compared to other skeletal muscles. One neuron innervates fewer muscle cells.

Facial muscle fatigue (esp late in day)
Ptosis
Difficulty chewing & swallowing
Limb weakness (brushing hair for example)
Respiratory weakness

Tensilon test (short acting acetylcholinesterase inhibitor IV or IM)
Lab test for thyroid dysfunction
Chest CT for thymoma
Hx and physical demonstration of fatigue
Ice test when ptosis

1. Anticholinesterase agents
2. Treat any underlying thyroid problem
3. Suppress antibody formation (corticosteroids/immunosuppressant)
4. Thymectomy

A group of disorders characterized by recurrent seizures. Recurrent is the key word.

Transient disturbance of cerebral function caused by abnormal neuronal discharge.

Benign febrile convulsions of childhood.

Yes, the child is at risk for a recurrence in the next 2 years (also with a fever - and that can't be prevented with antipyretics)

No the child is not at significant risk of developing epilepsy.

Idiopathic Seizure

Yes.

When it occurs after the first week after the trauma.

When it occurs after the first week after the stroke.

1. Hypoglycemia - when plasma glucose falls below 20-30 mg/dL

2. Uremia

1. Overdose with stimulants, insulin, or lidocaine

2.Withdrawal from EtOH or sedatives

The whole brain

1. Tonic-Clonic
2. Absence (petite mal)
3. Other (tonic, clonic, or myoclonic)

Focal areas, usually the temporal lobe

1. Simple partial seizures
2. Complex partial seizures

Unconsciousness and tonic contraction of limb muscles

Extension of extremities (and arching back called "opisthotonus")

Not breathing because of respiratory muscle contraction

Jaw clamped because of contraction of masticatory muscles.

Alternate muscle contractions and relaxations

Symmetric limb jerking

Breathing (irregular)

Urinary incontinence (due to sphincter relaxation)

10-30 seconds

30-60 seconds

30 minutes

Postictal CONFUSION (reorientation
may take up to 30 minutes)

Hemiparesis during postictal (Todd's paralysis) - normal.

Possible transient Babinski (extension of toes rather than curl with dull instrument running up inside of foot underneath toes)

Stroke.
Hemiparesis.

Status epilepticus

Permanent brain damage.

Begin in childhood and end by 20s

Brief loss of consciousness without loss of postural tone.

5-10 seconds

Full orientation when seizure ceases.

Hyperventilation

A generalized MYOCLONIC seizure

It occurs and stays in one side of the brain.

Temporal

Motor, sensory, or autonomic phenomena depending on the corical area involved.

Consciousness throughout!!!

Clonic movements (if affecting motor cortex) that spread across the homunculus (will probably see facial and hand spasms)

Jacksonian march

It occurs in one side of the brain and spreads to the other.

Both temporal lobes OR
Medial frontal lobe

Motor activity in mouth, face, neck

Consciousness is impaired but not lost.

They do not remember the seizure.

Simple partial seizure

Simple partial seizure.

It is thought that maybe complex partial does, but people who have these seizures don't remember anything about them.

Hx of recurrent seizures
Abnormal EEG - spikes
CT or MRI for focal seizures or if onset after age 25 to rule out brain tumor.

Like it sounds, a fake seizure. Sometimes it might be a conscious effort for attention. Sometimes it might be an unconscious emotional problem surfacing.

Warning
No tonic phase
All clonic phase where patient doesn't
hurt him or herself.
No loss of consciousness (shouting)
No postictal confusion
No EEG abnormalities

In general, we want to quiet the discharge.

1. Alter ionic transport
2. Enhance GABA activity (increase
inhibition)
3. Decrease glutamate activity
(decrease excitation)

1. Phenytoin (most common)
2. Carbamazepine

(also Topiramate)

1. Phenobarbitol
2. Carbamazepine

Alters ion transport - works on sodium channels.

Same as phenytoin. Alters sodium channels.

carbamazepine

Topiramate

It increases GABA by interfering with its breakdown.

Also used for migraines.

Increases myopia by 3-4D

Increases risk of AC glaucoma because of shift in lens. You want to give a cycloplegic (atropine) to move lens back.

1. Ethosuximide
2. Valproic Acid

Affects calcium channels.

Causes rashes.

1. Tiagabine
2. Topiramate
3. Vigabatrin

Blocks GABA uptake causing enhanced inhibitory activity.

Blocks Na+ channels and increases GABA activity.

Myopia ACG

Inhibits GABA transaminase resulting in more GABA in the brain.

Permanent peripheral field loss
Can continue to affect central cision
Can alter color vision
SEE THIS PATIENT FREQUENTLY!

One

Possibly two, but try just one first. If not with 2 then you probably won't get it under control with more.

Blood, Bone, Liver, Repiratory, Skin, Ocular

nystagmus and diplopia

1. Excision of epileptogenic zone
2. Corpus callosotomy
3. Hemispherectomy
4. Left vagus nerve stimulation

More than 75% are seizure free after 5 years.

Temporal lobe epilepsy, patients with auras. With aura it is easier to identify where seizures are occurring.

Surgical disconnection of the two hemispheres.

Doesn't seem to affect most patients at all.

Removal of one whole hemisphere of the brain.

Results in hemiplegia, hemisensory loss, hemianopia.

A stimulator is implanted in the chest and is programmed to give 30 second stimulation every 5 minutes.

During an aura the patient waves a wand over the stimulator which helps prevent seizure

Partial seizures.

Control of seizures, but not elimination of seizures.

Every couple weeks for poorly controlled

Every 3-12 months for well controlled

If seizure free after 2-5 years on meds

Slowly withdraw from meds over 6 weeks.

If seizures recur, start at previously effective level.

Sudden, unilateral facial paralysis

Upper respiratory infection

Pre-diabetic state. (Seen 2x as frequently with pre-diabetics)

CN VII
Acute inflammation
Compression by temporal bone

With a complete screening neurologic exam

Look for signs of Zoster or Simplex
Look for Lyme Disease
Syphilis

7-10 days
Full recovery at 6 months.

Prevent nerve degeneration

Steroid (controversial)
Acyclovir
A combo
Or nothing at all

Surgically altering musculature so that the patient has some kind of movement of the mouth.

Use if reinnervation is not possible.