• Allows one to be aware of head orientation in space
• Regulates eye position during head movement to maintain a stable image on the retina
• influences mus. tone for postural support
• influences learning and emotional developement

Tonic signals head position when there is no head movement

Detects linear acceleration and deceleration: change in velocity without change in direction (up/down, side to side, forward/back)

Like Mad Cow Dz in humans-

Bovine form- dead cow and sheep getting reprocessed and feed to the cattle

then human eat the meat

• spontaneous onset/ rapid to death
• Very rare
• Prion (protien) w/o nucleic acid replicates causing spongy brain = spongiform encephalopathy
• can also affect SC
• Its transmissible- genetic predisposition and still pass it on (w/o symptoms)

Clinical manifestations:

dementia

lapses of memory

mood changes

myoclonic activity

problem sleeping

weight loss

akinetic mutism (w/o mvm and speech)

diagnosed microscopically after death

if live biopsy is done the dz will transfer

Its highly contagious

with no cure

• Adult onset
• progressive
• motor neuron ds= ant. horn cells affected
• affects corticospinal tracts= motor tract
• Changes in the later aspect of the SC, BS, Cerebral cortex

possibly by chronic metal intoxication

male > female

> Western pacific

will affect both upper and lower motor neurons

• Also known as Lou Gehrig's DZ
• Giant pyramidal cells=primary motor cells in corticospinal tract
• will see axonal degeneration of the large myelinated fibers=
•  will have death of the peripherial motor neurons in BS and SC --> denevation and atrophy of mus fibers affected

familial form= only 10%

FALS

will have degeneration of

• Spinocerebellar
• posterior column
• Clarks column= major relay center for unconscious proprioception

Highly Selective

Sparing of occulomotor nuclei= still has eye mvm

Early collateral Sprouting= looks like pt is doing better

Clnical Manifestations:

Hyperactive DTR's

eye mvm intact, sensory intact, B&B intact

Babinski's Sign (+)= extension of great toe, other toes spread out w/ lateral plantar stim. (indicates pyramidal damage) also known as UMN damage

Hoffman's Sign (+)

UMN damage=

spasticity develops due to loss of BS control

loss of mus strenth UE>LE

• UE=extensors
• LE=flexors

LMN damage=

insidious=slow onset, w/ asymmetrical weakness starting with an extremity distally

Cervical extensors= head droop, pn

facial mus weak= eye, mouth, tongue

Dysarthria

Dysphagia

Drooling

Twitching= fasiculations

due to spontaneous contractions of mus. group fibers belonging to a single motor unit

=muscle cramps=twitching

often death within 2-5yrs secondary to:

Pneumonia

Resp. Failure

Early onset < 50 

Occurs on what side of the brain:

Expressive aphasia

Receptive aphasia

are non-neuronal cells that maintain homeostasis, form myelin, and provide support and protection for the brain's neurons

in the CNS

the earliest response to injury=astrocyte swelling

form of Glial cell = cell of the CNS

like schwann cells in PNS, they provide insulation of the axons

producing myelin sheath

Cell in the PNS that forms myelin sheath

protects axon

Consiousness happens via                      ?

At the level of the Brain Stem

• upper part controls sleep wake cycle
• Lower part controls respiration= BS damage- may end up on respirator

Hyperarousal-

- restlessness, agitation, delirium due to inhibition of the BS fxn

Hypoarousal-

Drowsiness, stupor, or coma

Any weakness in muscle

neurons that innervate mus. and originates in the Medulla

Damage to mediofrontal lobe= lack of motivation

can be physical or mental

(w/o speaking or mvm)
Abulia-lack of will or initiative

EX. Zombies

damage to pons= usually thrombosis of Basilar Artery

no mental or sensory deficit

motor limited to eyes

controls autonomic functions, and relays nerve signals between the brain and spinal cord

-respiration -chemoreceptors (O2 levels)
-cardiac center-sympathetic, parasympathetic system
-vasomotor center- baroreceptors
-reflex centers of vomiting, coughing, sneezing, and swallowing

Progressive Dementia

most common in the U.S.

slow decline of memory= begins in the Hippocampus

senile plaque and neurofibrillary tangles

>70 y/o

Genetics increased risk APO E-4

Apolipoprotien E Genotype 4= abnormal amyloid processing= slow removal

UMN's and corticobulbar fibers controlling speech, mastication and swallowing are affected

may manifest inappropriate laughing, irritability, anger, tearfullness

• cranial nerve nuclei involvement
• weakness in mus of swallowing, chewing, facial gestures
• fasiculations of the tongue are usually prominent
• Dif. w/ respiration may occur before limbs
• dyarthria and exageration of the expression of emotions

• neuronal loss in the cortex
• hypersensitive DTR's with spasticity= suggesting corticospinal tract involvment
• no mus. atrophy
• no fasiculations
• rare form of ALS

progressive loss of motor neurons in the anterior horn of the SC

often begins in the Cervical area

progressive weakness, wasting, fasciculations

decline of intellectual funx

 Hallmark is significant memory loss

cortical atrophy of the frontal and temporal, with sparing of posterior 2/3

loss of suppressed or inhibited behaviors

no plaques or tangles in the neurons

looks like AD and PD dz

has PD unresponsiveness to meds

 Lewy Bodies that are found in PD

 neurofibrillary tangles & senile plaques with granulovascular degeneration similar to AD

cortibobasal Ganglionic Degen- asymmetrical gait, speed apraxia, alien hand synd., rigidity, myoclonus, cortical sensory loss

extracellular amyloid(insoluble fibrous material)  accumulation= plaques

plaques= inflammatory response

inflammation causes Tau to break down that holds microtubles together causing Tangles

ACh receptors are then decreased

normal funx is to maintain fibroblast and cell funx

normally this dissolves and is reabsorbed by the brain tis.

Extracellular deposits of amyloid in the gray matter of the brain

cerebral cortex is the 1st area to show pathological changes

Damage is very selective= pyramidal cells w/ hippocampal projections

Entorhinal Cortex is damaged=important memory center, forms main input to the hippo.

Become indifferent, impulsive, irritable, withdrawn, anxious

inability to learn new information= not helped by clues

Visiospatial deficits= hinders activities, work, cooking, driving

Cant do simple math

impaired judgement and making changes

These people have

increased wake time

eating ds

changed sexual habits

and eventually all learning is lost= walking, talking, eating

Tx -

Aricept=slows down the process of ds

Dx -

R/O other medication reactions R/O other dememtias CT/MRI= looks at brain size (shrinks)

involves degeneration of the dopamine-producing cells in the substantia nigra

in basal ganglia

primarily involves damage to the striatum

in basal ganglia

these are associated with what structure in the brain: ?

Huntingtons dz

Parkinson dz

Dystonia

result from damage restricted to the subthalamic nucleus

is characterized by violent and uncontrollable flinging movements of the arms and legs

role in controlling eye movements

controls motivation

release of neurotransmitters like:

ACh

Dopamine

degenerative of CNS

Progressive

Chronic DZ

50-79 yr. olds

>whites

IDIOPATHIC 70-78% of pt's

Drug induced, BG tumor, Metabolic Ds

Secondary too...

displays the common symptoms of:

 tremor, hypokinesia, rigidity, and postural instability

known as disorders of multiple system degeneration, are a group of neurodegenerative

tremor, rigidity, akinesia/bradykinesia, postural instability) with additional features that distinguish them from simple idiopathic

• maximal when the limb is at rest and disappearing with voluntary movement and sleep
• cogwheel rigidity" when the limb is passively moved

Gait and posture disturbances such as decreased arm swing

 a forward-flexed posture and the use of small steps when walking;

speech and swallowing disturbances; 

 other symptoms such as a mask-like face expression or a small handwriting

links to

genes; familial

viruses

toxic exposure - MPTP -(opiates) heroin, sedatives

enviromental factors

Formal education= Dr.s vs. low risk for miners

low risk= construction, smokers, intense exercise (availability of dopamine)

Ds dysfunx of subcortical gray matter in the Basal Ganglia

Decrease of dopamine (needed for normal BG funx) stores in substantia nigra=depigmentation

Lewy bodies are present=abnormal aggregate of protien, masses seen under microscope

Hallmark= Degeneration of the nucleaus (substantia nigra)

loses inability to produce dopamine

--> to increased inhibition of input to the thalamus

--> thalamus cant send excitatory input back to the frontal cortex

--> less activity in the cortex= slowed mvm

< dopamine = > ACh

= causes generalized activation of skeletal mus. and fusimotor sys --> increased inhibition of the gamma motor neuron and increased alpha motor neurons

Rigidity, Bradykinesia, Postural instability, Tremor

Early signs of            is loss of reciprocal arm swing with ambulation.

-increased response to mus. stretch

-inability to passively move part

-Increased resistance throughtout range

-SPINAL REFLEXES ARE NORMAL

types of what?

Cog-wheel- Jerky resitance to PROM, mus. tense & relax

Lead-pipe- sustained resistance to PROM

Causing much energy expenditure

70-80% depletion of dopamine occurs prior to any clinical signs of dz

onset usually insidious

--> movement ds= becomes reliant on cortical control- the pt learns strategies to improve their mobility

Inability to initiate mvm

problem is in the preparation

Paucity of natural and automatic mvm's

Decreased Blink

lack of expression

exacerbates with emotional stress, fatigue, and reduced when relaxed

stems from a combined lesion in 2 pathways

Basal Ganglia

Cerebellar

head and trunk tremor

noted when the pt is tryin to maintain an upright posture against gravity

jaw, tongue, FA, Lower limbs

worsens over time

Sudden cessation of movement in the middle of an action

-usually during walking, or could be speech, arm mvm, blinking

Could be triggered by surface you are walking on or change in enviroment

flexed posture - kyphotic spine

postural instability- abnormal posture response

loss of postural reflexes

decreased rotation

Cannot "catch themselves" when pertubered (retropulsion)

Narrow BOS

Festination (anteropulsive) starts to walk but cannot control speed

slow

shuffle

decreased step length

ANS dysfunction

due to loss of neurons in sympathetic ganglia

Sweating

Increased salivation

incontinence

ortho, HTN

Meds given to PD pt's

usually lasts from one to five years on carbidopa/levodopa (Sinemet).

PT: keep these pt's active

Gait and Balance (to music)

Decrease rigidity-

-rotation activity

-slow rocking

Involuntary Basal Ganglia Dz

in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures

Generalized or Focal

At end range

Primary or secondary

onsent can be infantile to Adult

Focal Most Common Form- single area

-onset 30-50 y/o

-musicians, keyboard operators (hand dystonia)

Segmental- spreads > than one area adjacent parts

Generalized- wider spread

often exacerbated when the person performs active voluntary mvm's

6x more common than other well-known neuromuscular ds such as HD, ALS, muscular dystrophy

"Writers Cramps"

Descending pathways involving reciprocal inhibition of motor neurons

--> defective retrieval of specific motor programs in response to sensory stimuli results in co-contraction of both agonist and antag. mus around a jt

-symptoms typically dramatically improve with low-dose administration of levodopa (L-dopa)

-precursor of the neurotransmitter dopamine

will see signs as if PD pt

• A dopa-responsive dystonia of early–childhood/adolescent-onset with progressive difficulty in walking and, in some, spasticity
• relative mobility in morning, with disability later in day and after exercise

SCM gets hypertrophied

 involuntary

Painful interfers w/ fxn

contractions can last a long time w/o remission

= OA, hypertrophy SCM, Cervical fx

• Best predictor is= Age of Onset
• if starts in childhood and affects multiple family members= progressively gets worse
• Secondary in childhood due to CP or trauma= will remain about the same
• Focal will sometimes transfer to segmental but not to generalized

Hereditary

Progressive

Choreic mvm= brief, purposeless, involuntary, random

More than jut a mvm ds

Destroys area of the brain= emotion, intellect, movement

early and middle stages= indirect pathway is effected

Mvm generator aspects of the basal ganglia are continuously active

Indirect patways affected= neurons from the striatum to the S.N. are depleting

--> Gaba, ACTH, and Metenkaphlin are reduced (opp. of PD)

--> Dopamine and norepinephrine are increased

Late Stage= looks like PD

damage to the direct pathway

Loss of the direct inhibitory substance causing inhibition of th thalamocortical output

resultin in rigidity and Bradykinesia

All caudate nucleus projection neurons are affected (lost)

Mvm's can initially be covered by incorporating purposeful activity

looks like exaggerated normal restlessness

usually UE and face

Magnified with mental concentration

Unable to perform RAM

Gait- Ataxic= dance like

wide BOS

Staggering

urinary incontinence

Sleep ds

disturbed eye mvm's

dysphagia, dysarthria

EVENTUAL CACHEXIA= ILL looking

more severe course for early onset

death 15-20 years after onset

Infection will cuse early death

Suicide

A primary demyelinating ds

Hallmark

Sclerotic plaques found throughout the CNS

Scarring

chronic

Autoimmune illness

a primary demyelinating ds

Lesion=slow and block transmision resulting in weakness and sensory deficits

90%- 16-60 y/o

Females 2.5x> Males

Virus is likely the trigger

-immune sys mistakes protien of myelin protien for a virus and targets it for destruction

Deficiency of the myelin production cells= oligodendrocytes

Astrocyticgliosis --> scarring

2 possible culprits

1. Herpes virus 6

2. bacterium chlamydia pneumoniae

Slow, writhing fluctuating muc. tone

part of CP

rapid, jerky

dance like

will see changes in the body but not a progressive dz

just growth that causes uneveness in the body

treatment for mus relaxants

Dorsal rhizotomy- selective cutting of nerve roots (decrease spasticity)

Baclofen- decreases pn. prevents increased in deformity which improves ease of care

more diffuse brain infection

global

prevents the infection from entering the brain or the CNS

Organisms get in the brain and or CSF when the body has a break down of endothelial BV's in the brain and cerebral BV's

pt is supine = then flex hip and knee

= pn, strong involuntary resistance w/ attempts to passively extend

High grade tumor with the worst prognosis- remove they come back (eventually pt dies)

each has a dif orientation from the others

oriented on 3 planes of mvm

Flexion/Ext= pitch

Lateral Flexion= roll

Rotation= yaw

Each canal is filled with endolymph, a viscous liquid, which moved when there is head mvm

when the endolymph moves, the hair cell receptors are bent causing?

endolymphatic hyrops

recurrent severe vertigo with nausea, disequillibrium, fluctuating hearing, and tinnitus due to increased pressure exerted by endolymph

Brain damage due to the reaction to the trauma

Brain swelling

decreased cerebral perfusion

normal is 5-10mm Hg

Defer TX if >20mm Hg

severe can cause herniation

inability to form new memory

decreased mem. decreased word/name finding

Levels of Cognitive function

What scale?

I- No response;

II- Generalized response

III-Localized response

IV-confused agitated

V- Confused inappropriate

VI- confused-appropriate

VII- Automatic-appropriate

VIII- Purposeful-appropriate

4 states =outcome

conscious state of a person

vegetative

severe disability

moderate disability

good recovery

is diffuse

and only affecting one type of tissue in many areas

is diffuse

affects different type of tissues in many places

neuron cell death

necrosis

apoptosis

type of glial cell

support and nourish- 02 and nutrients

removes debris

seal off damage brain tis.

are nerve cells

carry info from one brain region to another

Consciousness happens via

Ascending reticular activating system

in the ?

Mediofrontal lobe damage

lack of motivation

physical or mental

right lobe

responible for orientation of body to space

common problem is hemineglect

Struggles with interpersonal relationships and socialization

Suggests phyciatric involvement

Hallmark

Scarring

has usually a coexisting autoimmune ds such as

HASHIMOTO's thyroiditis, psoriasis, inflammatory Bowel mvm, and RA

Four subtypes:

• Relapsing-remitting:
• Secondary progressive
• Primary Progressive
• Progressive relapsing

has periods of neurologic dysfxn

lasting days to months followed by fall or partial recovery

there is a stable course between relapses

Most common pattern

Steady decline in neurologic fnx from the outset with episodes of minimal recovery

Gradual, progressive weakness of mus

progressive dz from the onset with clear exacerbations

rarest form

chromosome 6 could be a genetic determinant

vit d could be a protection

Optic Neuritis is often the first manifestation

Dorsal column symptoms include Paresthesias and Hypoesthesia, loss of vibration, 2-pt discrim.

tingling/ prickling

diminished sensitivity

complaints are feeling of swelling,

wetness, the body part affected is tightly wrapped

Heat increases weakness

spasticity is an extremly common problem

also called Tic Douloureux

shocklike pn in the face

momentary electricity- like sensation evoked by neck flexion or cough