Jerky movements predominately of facial muscles & extremities
What is Huntington's chorea/disease?
An incurable, inherited, autosomal dominant disorder characterized by jerky movememnts & progressive dementia (leads to loss of intellect)
What causes Huntington's?
Atrophy of the striatal neurons (caudate, putamen, & globus pallidus nuclei) resulting in motor fxn disturbances such as jerky movements
What part of body is involved in Huntington's?
The entire body, but mostly the extremities & facial muscles
When does Huntington's develop?
normally b/w 30-35 yoa
What is the Huntington gene?
Gene that regulates the normal fxn of striatal nuclei. Normally the body has 33 CAG triplets but in Huntingtons there are extra. The more extra, the earlier the onset.
What diseases/disorders sometimes accompany Huntington's?
Parkinson's or hypokinesia/rigidity
How long before death occurs in Huntington's patients?
Usually within 15 years from onset of infection from a weakening immune system (#1 cause) or suicide (#2 cause)
What is ex-vacuo hydrocephalus?
Atrophy of the neurons along the ventricular walls causes distention of the ventricles; does not lead to increased ICP
What is Syndeham's Chorea?
An acquired disorder associated w/ rheumatic fever
When is onset of Syndeham's?
In pts younger than 15 yoa, usually female
What symptoms are associated w/ Syndeham's?
Mild vasculitis of cerebral vessels
Is Syndeham's curable?
What is syringomyelia?
Condition in which a tubular cavity in the central area of the SC gradually expands & produces neuronal & tract damages. Accumulation/obstruction of CSF in the central canal causes distention
About 95% of syringomyelia pts develop what disorder?
Type 1 Arnold-Chiari malformation
What is Type 1 Arnold-Chiari malformation?
Inborn inclanation of cerebellar tonsils into the CSF
Associated w/ the cerebellum & medulla protruding through the foramen magnum which leads to compression of the brainstem (which causes breaking of the fibers) & hydrocephalus (lumen narrows, CSF can't leave)
How does Type 1 Arnold Chiari Malformation affect the body?
Affects the spinothalamic tract causing loss of pain & temp sensation
Cervical involvement of the shoulder jt shows shawl/cape like distribution, shoulder may swell, & humeral head may undergo lysis (just shoulder jt)
What is meningitis?
Inflammation of the dura mater & tissues around the subarachnoid space
What are factors that influence meningitis infection?
Strength of immunity
Predisposing risk factors
What are some predisposing risk factors to meningitis?
Exposure of head to cold
Otitis media or accumulation of pus in the middle ear
Pneumonia leading to pleural empyema (widespread accumulation of pus in pleural cavity)
Basal skull fracture
2 causes of meningitis that have the highest fatality rate
Time frame b/w onset of secondary & tertiary syphilis?
Any inflammatory disease leads to what?
Full jt fusion (eventually)
What is seronegative spondyloarthritis?
Inflammation of verterbral jts
Characteristics of seronegative spondyloarthritides
Pathological changes beginning in the ligamentous attachments to the bone, rather than in the synovium
What is an -enthesis?
Insertion of the ligament/tendon to bone; very rich w/ nociceptors (painful) and has a poor blood supply (limited healing)
What joints are involved w/ seronegative spondyloarthritis?
100% involvement of SI jts along w/ 50-75% involvement w/ peripheral inflammatory arthritis
What lab factors are associated w/ seronegative spondyloarthritis?
Absence of rheumatoid factor
HLA-B27- 95-97% of people w/ AS are HLAB27 +, while only 5% of gen population are +
What is inflammatory enthesopathy?
Ossification of the outer layers of the annulus fibrosis (sharpey's fibers)
What is a syndesmophyte?
Complete ossification forming a bony bridge (in AS- of ivd so that 2 vertebrae can't move)
What 2 inflammatory bowel diseases are associated w/ seronegative spondyloarthritis?
Crohn's & ulcerative colitis
What are aka's of ankylosing spondylitis?
What are symptoms of ankylosing spondylitis?
Severe low back pain (young people- age crucial)
Non-mechanical pain that is severe in the morning & diminishes during the day
Relieved by exercise
What tests can be used to determine if a pt has AS?
Measure under the nipple line w/ full inspiration & full expiration - normal chest expansion is 6-12 cm & less than 3 cm indicates AS
Finger to floor exam will show decreased flexibility
Decreased ROM in all 3 plane
All signs b/c spine fuses together
What are some complications of AS?
Dilation of the aortic ring w/ aortic insufficiency
What is iridocycitis?
Irregularly shaped pupil due to adhesion of the iris to lens from inflammation/exudates
What are the 3 MC causes of aortic insufficiency
1 Bacterial endocarditis
3 Ankylosing spondylitis
What part of the body does psoriatic arthritis affect?
Joints more so than spine
How much of the population has psoriatic arthritis?
2-7% have psoriasis w/ an avg 5%
2-7% of those people have arthritis & spondylitis
Is psoriatic arthritis slow or rapid?
What bone can be destroyed by psoriatic arthrits?
What are the signs & symptoms of psoriatic arthritis?
Pitting of nails
Pencil in cup deformity (on x-ray)
What is dermatosis?
Non-inflammatory disease of skin; common areas include the scalp, axilla, belly button, groin (hair portions), & elbows
What joints are involved w/ psoriatic arthritis?
Asymmetrical involvement of jts of hands/feet
Advanced-> chaotic deformity of jts
Axial arthritis- one entire finger/toe
What is axial arthritis & what diseases is it involved in?
Only in psoriatic & Reiter's
Involvement of one entire finger/toe
Causes sausage toe
What is arthritis mutilans?
Special type of psoriatic arthritis
Marked by intra-articular osteolysis
Bone is lost & tissue retracts resulting in shrinking toes/fingers
What are charateristics of Reiter's syndrome?
Infection, typically chlamydia (MC std in US) or various intestinal infections often in children
Are men or women more predispositioned to get Reiter's?
What percentage of pts fully recover from Reiter's?
What are the signs & symptoms of Reiter's syndrome?
4 major signs: Can't see, can't pee can't dance w/ me
Arthritis of peripheral & spinal jts
What part of the body does arthritis associated w/ Reiter's syndrome affect?
Spine almost all of the time
Larger or medium sized jts
Mostly leg jts rarely arms
Bilateral & symmetrical SI jts
What is urethritis?
painful urination or balanitis
What is keratoderma?
pustules on the soles or palms
What are manifestations of Reiter's syndrome?
achillobursitis associated w/ localized osteopenia stemming from inflammation of soft tissue near the bone
Tearing of the achilles tendon (possible not always)
What is achillobursitis?
swelling of the ankle
What is achillodynia?
pain in the bursa/tendon; may be w/ or w/o inflammation & bursitis
What is the treatment for Reiter's syndrome?
Injection of corticosteroids into bursa
What is atelectasis?
aka Collapsed lung
Loss of lung volume due to incomplete expansion of air spaces
What are the 4 types of atelectasis?
Obstructive aka Resorption/Absorption
Compressive aka Passive/Relaxation
Pathcy aka Microatelectasis
What is obstructive aka resorption/absorption atelectasis?
Obstruction of the airways in the alveolar ducts which causes space to become void of air as it is absorbed into circulation which creates a vacuum. The total pressure in the lungs becomes lower than atmospheric pressure which leads to collapse & compression. The decreased space causes the mediastinum to deviate toward the lower pressure (involved side)
What causes obstructive atelectasis?
Aspiration of foreign body
Excessive secretion of exudates within smaller bronchi (as in COPD)
Tumor of any organ in chest, leading to obstruction of the airway
Other lung pathologies- bronchial asthma, chronic bronchitis, bronchiectasis
What is compressive atelectasis?
Compression of the lung tissue from accumulation of fluid, air, or tissue in the pleural cavity (pleurisy or pneumothorax) which causes the mediastinum to deviate away from the involved lung
What are causes of compressive atelectasis?
Right sided cardiac failure (from vena cava congestion & pooling of blood into the cavities
Neoplastic effusions (tumor)
Pleurisy or pneumothorax
What are 2 types of pneumothorax?
Traumatic= stabbing- damage to parietal layer
Non-tramatic or spontaneous= sickness- damage to visceral layer (#2 cause of death by TB)
Rib fracture can involve both layers
Causes immediate lung collapse
What are the top 3 causes of pneumothorax?
3 Interstitial lung disease
What is patchy atelectasis?
Due to a loss of pulmonary surfactant
Surfactant decreases adhesion strength of the alveoli- w/o surfactant alveoli adhese together
Respiratory distress syndrome, common in peds
What is contraction atelectasis?
Intersitial lung disease causing replacement of normal lung tissue w/ fibrous CT or scar tissue