Neuro E2- Movement disorders

What does damage to the basal ganglia cause?

movement disorders (does NOT cause weakness)

What does damage to the substantia nigra result in?

less dopamine, imbalance between GABA & Ach (like in PD)

What is a rhythmic oscillatory movement, enhanced. by emotional stress and disappears during sleep?

tremor

What kind of tremor is present during sustained posture?

postural / action tremor

What kind of tremor is associated with/ fear or fatigue?

physiologic tremor

What kind of tremor is present during planned voluntary movement but NOT present at rest, and may be associated with a cerebellar lesion?

intentional tremor

What tremor occurs when limb is at rest?

resting tremor

What is rapid irregular muscle jerks that occur involuntarily & unpredictably, involving only one body part at a time but “skips” from 1 part to another randomly?

chorea

What is a milkmaid grasp?

hand grip relaxes intermittently (seen with chorea)

What do patients with chorea have difficulty with?

maintaining muscular contraction for strength testing, also milkmaids grasp, irregular & unsteady gait, and speech irregular in volume/temp (explosive)

What is a violent, unilateral chorea involving sudden, wild, flail like movements of proximal muscle groups, and is most often due to vascular disease in the contralateral sub thalamic nucleus?

hemiballismus

What kind of movement is this?

• slow, sinuous, writhing in characteri

• flow to different parts of body (rather than skip)

• flex, extension, pronation, supination of fingers & hands (proximal limbs)

• not present during sleep, may be caused by brain lesion

athetosis

When athetosis is held as a prolonged posture, what is this considered as?

Dystonia

What kind of movement?

• sustained muscle contractions causes twisting & repetitive movements or abnormal postures

• not present during sleep

• enhanced by voluntary activity

dystonia

What is abnormal neck and head posturing?

spasmodic torticollis (cervical dystonia)

What is a blepharospasm?

involuntary B/L contraction of peri-ocular muscles

What condition is generalized dystonia seen in?

cerebral palsy

What are treatment options for dystonia?

anticholinergics (benztropine), dopamine, Backofen, benzodiazepines, botox

What kind of movement?

• difficulty/slowness in performing voluntary movements

• MC involve repetitive loos perioral and lingual muscles

• can be caused by long term medication use (antipsychotics, DA receptor blockers, metoclopramide)

tardive dyskinesia

What kind of movement?/

• sudden, rapid, very brief twitch ike muscle contractions

• spontaneous or brought on by stimulation/initiation of movement

• generalized or focal

myoclonus

What are treatment options for myoclonus?

anticonvulsants (VPA) & benzodiazepines (clonazepam)

What kind of myoclonus involve twitches that may awaken patient from sleep, including hiccups?

physiologic / nocturnal myoclonus

What is a form of myoclonus associated with hepatic encephalopathy (liver flap)?

asterixis (negative myoclonus)

If you ask a patient to extend their wrists like their stopping traffic and it causes rhythmic hand flapping, what should you think of?

liver issues (asterixis)

What kind of movement?

• sudden, recurrent, quick, jerky, coordinated movements of smaller muscle groups; recur in a pattern

• suppressing movement can cause anxiety

• worse with stress & disappear with sleep

• associated with Tourette’s syndrome (chronic)

tics

Transient simple tics that terminate spontaneously usually occur in what population?

children

When do chronic simple tics develop?

any age, usually childhood

When do persistent simple or multiple tics develop?

before age 15, resolution by end of adolescence

If abnormal movements are present since infancy, what is the likely cause?

Cerebral palsy

If abnormal movements begin in early adult life, what is the likely cause?

benign essential tremor or huntington’s

What meds resort tardive dyskinesia or extrapyramidal sx as an ADR?

Metoclopramide, Promethazine, Haloperidol

Abnormal movements in a patient with a history of rheumatic fever is likely what condition?

syndenham’s chorea

What body part do tremors usually spare?

legs

What condition?

• unknown cause or autosomal dominant

• begins at any age

• enhanced by emotional stress, improved by small amounts of ETOH

• interferes w/ delicate manual skills, writing, drinking, speaking (one or both hands MC)

benign essential / familial tremor

What is the treatment for benign essential tremors?

propranolol or nadolol, primidone, clonazepam, carbamazepine

surgery: stereotactic thalamotomy, deep brain stimulator

What condition?

• reversible, extrapyramidal, spasmodic or sustained involuntary contractions of muscles

• arise from a drug induced alteration of dopaminergic cholinergic balance in the nigrostriatum (basal ganglia)

acute dystonic reactions

What drugs can cause acute dystonic reactions?

DA receptor antagonists: neuroleptic/antipsychotics (haldol), phenothiazine anti-emetics

cocaine, street valium, LSD, amitriptyline, lithium, anticonvulsants (phenytoin, carbamazepine)

What occurs with drug induced laryngeal dystonia?

dysphagia & stridor

What occurs with drug induced oculogyric crisis?

involuntary deviation of eyes due to contraction of extraocular muscles

What occurs with drug induced buccolingual crisis?

forced spasms of face, jaw, tongue

What occurs with drug induced opisthotonus?

forced spasm of paravertebral muscles, forcing trunk & neck into hyperextension

What is the treatment for acute dystonia?

emergency - diphenhydramine or benzotropine, discontinue offending drug, admit to ICU if airway involvement

What does failure to respond to repeated doses of an anticholinergic drug suggest?

non-drug induced dystonia

What condition?

• adult (elderly) onset neurodegenerative disorder of extrapyramidal system

• motor sx usually unilateral

• the only neurodegenerative disease that is treatable long-term (but no cure)

parkinson’s disease (PD)

Who is Parkinsons more common in?

onset at 60, M > F

What are the 4 cardinal signs of parkinson’s?

Tremor- resting, “pill rolling”

Rigidity- difficulty moving stiff limbs, inc muscle tone hypertonia

Akinesia- difficulty initiating movement, bradykinesia

Postural instability- flexed stooped posture

What is primary parkinson’s disease?

lost dopaminergic cells in nigrostriatal system causing imbalance b/t dopamine & Ach in corpus striatum

5 stages of disability

What following symptoms are seen with what condition?

• difficulty w/ initiation of walking

• shuffling gait

• monotonous, stuttering, poorly enunciated dysarthria

• Dementia & depression common

• lead pipe rigidity (continuous inc muscle resistant felt on passive movement)

• cogwheel rigidity (ratchet-like fluctuate of muscle resistance felt on passive movement)

• ocular- dec blink reflex, dry eyes, apraxia, etc

Parkinsons

What is the name one the scale used to stage PD?

Hoehn & Yahr scale of disability

What is the first line & most effective treatment for PD?

dopaminergics: l-dopa/carbidopa (sinemet)

What are possible SEs of Ldopa/carbidopa (Sinemet)?

N/V, hypotension, arrhythmias

What drugs can be used in addition to levodopa/carbidopa in PD?

COMT inhibitors: Entacapone, Tolcapone

DA agonists: Pramipexole, Ropinirole, Bromocriptine

MAO-B inhibitors: Selegiline, Rasagiline

What SE is seen with Bromocriptine (Parlodel)?

fibrosis of lung, heart, & kidney

What should be avoided with MAO-B inhibitors?

tyramine rich foods like aged cheese or wines (hypertensive effect)

What is the second line treatment for PD?

anticholinergics: Benzotropine (Congentin), Amantadine (Symetrel)

*helo more with tremor & rigidity than bradykinesia

What classic adverse effect is seen with Amantadine (Symmetrel)?

livedo reticularis- vasculitis causing red-blue fishnet mottling

What is important to do with PD patients?

keep as physically active as possible

What condition?

• pathologically enlarged ventricular size w/ normal opening pressure on LP

• reversible w/ ventriculoperitoneal (VP) shunt - important to dx

• mimics PD- get CT to differentiate

Normal pressure hydrocephalus (NPH)

What is the classic triad of NPH?

dementia. gait disturbance, urinary incontinence

What condition?

• genetic, chronic, degenerative d/o of CNS

  • chromosome 4- caudate nucleus atrophy, dec in GABA & sub P, inc dopamine

• 3 manifestations: psychiatric sx, progressive cognitive deterioration, chorea (may manifest last)

Huntington’s disease

What population do you see Huntingon’s disease?

insidious onset after age 30 (lifespan 15 years after), M=F

Early or late ssx of Huntingtons?

• anxiety

• emotional lability

• impaired problem solving

• depression

• hypotonia

• abnormal eye movements

early

Early or late ssx of Huntingtons?

• chorea

• dementia

• dysphagia, dysarthria

• incontinence

• gait disturbance, postural instability

• rigidity, clonus

late

What is the treatment for Huntington’s?

genetic counseling, no known cure, symptomatic tx only

dyskinesia: haldol, tetrabenazine

behavior: clozapine

chorea: clonazepam

rigidity: baclofen

depression: fluoxetine

How would Huntington’s disease appear on imaging?

atrophy of cerebral cortex & caudate nucleus

What condition?

• acute or insidious onset 2-3 mos after rheumatic fever (GAS infx)

• usually subsides in 4-6 mos

• abnormal choreiform movements mistaken for fidgetiness, behavior changes (irritable, obsessive-compulsive, emotional labile)

Sydenham’s chorea

What is the tx for Sydenham’s chorea?

eliminate strep infx; consider PCN IM/PO daily until age 20

What condition?

• neurological movement disorder associated w/ a sleep complaint

  • not a true disorder of basal ganglia

• “creeping and crawling” usually worse at night

• unknown cause; idiopathic or secondary to medications, iron def, lesions, PD, pregnancy, or uremia

Restless leg syndrome (RLS)

What is the diagnostic criteria for RLS?

irresistible urge to move legs ± uncomfortable sensations

urge to move is worse during periods of inactivity, evening or night, & relieved by movement,

What is the treatment for RLS?

non pharm: stretching mental alerting activities, avoid aggravating factors

pharm: Pramipexole, Gabapentin, Benzodiazepines (Clonazepam), opioids