DYSTONIA
Overflow dystonia
dystonic movement or postures extend beyond the commonly involved body region.
Botox injection
effective in improving postural deviation and pain in approximately 80% to 90% of people with cervical dystonias
2. Secondary Dystonia
env'tal causes (exposure to CO, cyanide, methanol and underlying causes like brain tumors, cp, PD, stroke, ms, CNS injuries, infections of the brain)
Clinical presentation of head deviation or neck pain
first diagnostic sign
Heredodegenerative (x-linked dystonia parkinsonism, HD, Wilson dse and PD)
geneteically inherited disorder marked by progressive decline in neurological structure and function
Focal
limited to one body segment (cervical dystonia, writer's cramp, and blepharospasm)
Mirror dystonia
mirror movements are observed in the opposite body side
Dystonic movements
movement is a twisting nature or a pull in a preferred direction, repetitive and patterned attitude (consistent and predictable)
3. Dystonia Plus (myoclonus dystonia and dopa-responsive dystonia)
non-degenerative neurochemical disorders associated with other neurologic conditions (examples)
Hemidystonia
one side of the body
• Average age of onset (primary) is 12 year old • Focal dystonias → 30-50 years
onset (dystonia & focal dystonia)
1. Primary Dystonia
r/o secondary causes
geste antagoniste
sensory trick
dyshagia
serious side effect of botox injection
• If secondary to CP →
static for several years
(light touch to the chin for people with cervical dystonia)
what to do to alleviate cervical dystonia
Observed at peak of dystonic movement. Determining that there is no evidence for other neurologic disorders, or secondary dystonia is essential in the diagnosis of idiopathic dystonia
when is overflow dystonia observed?
because hindi nagddevelop brain sa CP kaya di na rin masyado lumalala.
why is the prognosis static in CP?
Focal dystonias
writer's cramp
Multifocal
2 or more non contiguous body regions (right arm and left leg)
1. age of onset 2. body distribution of symptoms 3. temporal pattern 4. assoc features
4 subcategories accdng to characteristics
Segmental
Adjacent body parts (cranial dystonia → blepharospasm with jaw movement)
- early onset birth to 28 yrs and older than 28 yrs a. Infancy (birth to 2 years) b. Childhood ( 3-12 years) c. Adolescence (13-20 years) d. Early Adulthood (21-40 years) e. Late Adulthood (>40 years)
Age of Onset (ADS 2 categories:
Isolated | Combined with other movement disorders
Associated features
Best predictor of prognosis → age of onset
Best predictor of prognosis →
1. Focal 2. Segmental 3. Multifocal 4. Generalized 5. Hemidystonia
Body Distribution of Symptoms
- results in 3-7 days post injxn and - last for 3-4 months
Botox injection
1. Nervous System Pathology 2. Inherited or Acquired
CLASSIFICATIONS BY ETIOLOGY
1. Age of onset 2. Area of body affected 3. Underlying cause
Characteristics used to classify dystonia:
-Genetic testing for dystonia -Test to r/o other neurologic diseases -No definite test for dystonia • Clinical presentation of head deviation or neck pain → first diagnostic sign • Patients usually has normal perinatal and developmental history • EMG sustained simultaneous contractions of agonist and antagonists
DIAGNOSIS test: (3)
neuroleptics • Neuroleptic-induced dystonia (blepharospasm, torticollis, retrocollis
Drug induced etiology DYSTONIA
is often inherited and has poor prognosis
Dystonia that begins in the first year of life
typically patterned, twisting, and may be tremulous
Dystonic Movements: describe
1. Genetic-based dystonia → most common (2/3) i. DYT1 - typically starts in one extremity and progresses to other limbs. Cranial and cervical (rarely involved) ii. DYT6 - early involvement of cervical, oromandibular, laryngeal and speech 2. Focal dystonias → writer's cramp affected: Common among keyboard operators and musicians • Result of abnormal or repetitive biomechanics • May also occur as part of per nerve d/o • Evidence of genetic basis 3. Drug induced → neuroleptics • Neuroleptic-induced dystonia (blepharospasm, torticollis, retrocollis)
ETIOLOGIC AND RISK FACTORS
1. Dystonia posture 2. Dystonic movements 3. GESTE ANTOGINISTE (sensory trick) 4. Mirror dystonia 5. Overflow dystonia
FIVE FEATURES OF DYSTONIA THAT GUIDE DIAGNOSIS
1. Primary Dystonia 2. Secondary 3. Dystonia Plus 4. Heredodegenerative x-linked dystonia parkinsonism, HD, Wilson dse and PD
FOUR CLASSIFICATIONS BY CAUSE
cause: 1. evidence of genetic basis 2. abnormal or repetitive biomechanics 3. nerve d/o
Focal dystonias aka: affected: cause: (3)
• Emphasis on stress and pain management • Energy conservation • Adaptive equipment • Mobility • Selective splinting • Sensory retraining
IMPLICATIONS FOR THE PHYSICAL THERAPIST
• 250,000 in North America • 1.1 in every 100,000 • Male to female 1:1.6
INCIDENCE
Genetic-based dystonia → most common (2/3) i. DYT1 - typically starts in one extremity and progresses to other limbs. Cranial and cervical (rarely involved) ii. DYT6 - early involvement of cervical, oromandibular, laryngeal and speech
MC etiology of dystonia and explain
• Starts near birth and (+) family history → progressively worse • If secondary to CP → static for several years
PROGNOSIS (progressively worse)
• Botox injection → effective in improving postural deviation and pain in approximately 80% to 90% of people with cervical dystonias results in 3-7 days post injxn and last for 3-4 months | most serious side effect: dysphagia • Physical and Occupational Therapy • Surgery / DBS
TREATMENT
Static | progressive | variable | persistent | action specific | diurnal | paroxysmal
Temporal Pattern
1. Dystonia posture
a body part is flexed or twisted along its longitudinal axis and sensation of rigidity and traction is present
DYSTONIA
a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both
Generalized
affects the trunk and at least two other body parts
Geste antagoniste (sensory trick)
alleviation of dystonia occurs during the geste movement.
Temporal Pattern
describes the dse course