pediatric neuro: movement disorders

Movement disorders are impaired ability to control body movement, not _____

weakness

6 types of movement disorders?

• Chorea

• Ballismus

• Dystonia

• Myoclonus

• Tics

• Tremor

CBDMTT

Things to ask when seeing a movement disorder patient

How long has it been present?

What does the movement look like?

Is it rhythmic, jerky, ‘dancelike’? Show me.

Can it be suppressed?

What medications is the patient taking?

Family history of similar movements?

Case: Parents bring their 3-year-old daughter for evaluation of ADHD. Beginning about two months ago she has been unable to keep still, and they show you this phone video. Her naturopath recommended a regimen of 12 herbs and supplements, but she spits them out. She has stopped talking over the last 10 days or so.

dx?

Chorea

_______ – rapid movement of face/limbs incorporated into voluntary movement to hide it.

Chorea

Constant restlessness with movements flowing from side to side and limb to limb is characteristic of __________.

Chorea

High-amplitude, violent flinging of a limb is called __________.

Ballismus

An extreme form of chorea involving violent flinging movements is __________.

Ballismus

Sustained muscle contractions that may be focal, segmental, hemi, or generalized describes __________.

Dystonia

Involuntary rapid muscle jerks that are usually nonrhythmic define __________.

Myoclonus

Rapid muscle jerks that may be spontaneous, on action, or reflex occur in __________.

Myoclonus

Stereotyped movements or utterances that are shock-like, purposeless, and suppressible briefly indicate __________.

Tics

Dyskinetic movements that come and go are called __________.

Paroxysmal dyskinesias

Brief, irregular movements that are not repetitive or rhythmic, but flow from one muscle to the next

Chorea

Migratory chorea of the limbs and face occurring after a streptococcal infection describes __________.

Sydenham chorea

A movement disorder caused by antibodies to Streptococcus attacking the basal ganglia is __________.

Sydenham chorea

Sydenham Chorea can cause emotional _______.

lability

What is the treatment for Sydenham Chorea?

Treatment with penicillin for latent Strep infection to prevent heart valve damage

Chorea possible causes:

Drugs, Metabolic conditions, Systemic autoimmune disorders, Neurodegenerative diseases

Testing for chorea

Neuroimaging, glucose, electrolytes, thyroid studies, CBC(complete blood count), copper studies, genetic studies

Treatment for chorea:

• Treat underlying cause, if found

• Medication helps little

______: Muscle contractions result in twisting, repetitive movements and abnormal posture

Dystonia

Time span for dystonia

The movements and postures may be constant or occur in episodes

Dystonia possible causes:

• Drugs

• Metabolic conditions

• Systematic autoimmune disorders

• Neurodegenerative diseases

Testing for dystonia

Neuroimaging, glucose, electrolytes, thyroid studies, CBC, copper studies, genetic studies

Treatment for dystonia:

• Treat underlying cause, if found

• Medication helps little

Cerebral palsy is a group of __________ disorders caused by brain damage early in life.

motor

The brain damage in cerebral palsy occurs before, during, or just after __________.

birth

Cerebral palsy affects muscle __________ and __________.

control, coordination

CP can affect muscle _________, __________, and _________.

muscle tone, posture, and balance

People with CP may also have visual, hearing, speech, epilepsy, and __________ impairment.

intellectual

Cerebral palsy is a __________ condition. (time span)

life-long

Cerebral palsy can include involuntary movements such as __________ and _________.

dystonia and chorea

The dystonia and chorea in CP often appear as __________, twisting movements.

writhing

These involuntary movements in cerebral palsy usually involve __________ sides of the body.

both

Patients with cerebral palsy may also have __________, which means muscle tightness.

spasticity

Myoclonus can occur by itself, but most often it is part of another __________ system disorder.

nervous

Myoclonus may present as generalized, focal, or __________ jerks.

multifocal

Myoclonus disorder symptoms:

• Generalized, focal, or multifocal jerks

• Continuous or intermittent

• Light, sound, touch or movement may be triggers

Myoclonus usually caused by _________.

an underlying disorder (neurological or systemic)

Myoclonus usually caused by an underlying disorder (neurological or systemic), like _____________.

• Head or spinal cord injury

• Stroke or brain infection

• Kidney or liver failure

• Inherited metabolic diseases

The presence of slight __________ helps differentiate anoxic myoclonus from true brain death.

twitches

Physiologic myoclonus occurs in neurologically __________ children

normal

The most common form of physiologic myoclonus is __________ jerking.

sleep

Physiologic myoclonus requires __________ treatment.

no

Anoxic myoclonus typically occurs after a __________.

heart attack

Anoxic myoclonus is seen in patients who are almost at the level of __________.

brain death

The presence of slight __________ helps differentiate anoxic myoclonus from true brain death.

twitches

Anoxic myoclonus indicates severe __________ injury.

anoxic (or brain)

Idiopathic myoclonus involves clinically significant __________ that can occur at any time.

jerking

Idiopathic myoclonus can cause social and/or __________ disability.

physical

The condition progresses __________ or not at all.

slowly

Idiopathic myoclonus may be hereditary with an __________ __________ inheritance pattern or __________.

autosomal dominant or sporadic

The drug of choice for treating idiopathic myoclonus, if needed, is __________.

clonazepam

Epileptic myoclonus shows simultaneous __________ activity on EEG

epileptiform

A classic example of epileptic myoclonus is __________ Myoclonic Epilepsy.

Juvenile

Juvenile Myoclonic Epilepsy includes myoclonic jerks along with generalized - or absence seizures.

tonic-clonic

Epileptic myoclonus typically begins in early __________.

adolescence

Opsoclonus–myoclonus syndrome is associated with __________, a cancer that forms in neuroblasts.

neuroblastoma

Neuroblastoma most commonly begins in the __________ gland.

adrenal

In opsoclonus–myoclonus syndrome, antibodies made against the tumor __________ and attack cerebellar neurons.

overreach

These tumor antibodies attack the __________, leading to abnormal movements.

cerebellar neurons

Case

Term infant girl had routine delivery and was admitted to Newborn Nursery. At 3 hoursof life, during diaper change she stiffened, turned blue, and was not breathing -resolved within 15 seconds. Three more similar events occurred over the next day.

Her neurologic exam was remarkable for tremor, hypertonia (stiffness) when stimulated, and she convulsed every time her nose was touched.

Family history revealed that four infants had died of SIDS in dad’s relatives

dx?

Diagnosis: Congenital Hyperekplexia (Startle Disease)

This case is classic for hyperekplexia, a rare genetic disorder of exaggerated startle with life-threatening stiffness in newborns.