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 a symptom complex, not a disease

 nonprogressive central motor impairment syndrome due to insult to or anomaly of the immature CNS, extent of
intellectual impairment varies
 incidence: 1.5-2.5:1,000 live births (developing countries)
 life expectancy is dependent on the degree of mobility and intellectual impairment, not on severity of CNS lesion


 often obscure, no definite etiology identified in 1/3 of cases

o only 10% related to intrapartum asphyxia
o 10% due to postnatal insult (infections, asphyxia, prematurity with intraventricular hemorrhage and trauma)
o association with low birth weight babies

Clinical Presentation

Table 47. Types of Cerebral Palsy

% of
Type Characteristics Area of Brain Involved
total CP
UMN of pyramidal tract
Truncal hypotonia in 1st year
diplegia associated with periventricular
Increased tone, increased reflexes, clonus
leukomalacia (PVL) in premature babies
Spastic 70-80% Affects one limb (monoplegia), one side of body
• quadriplegia associated with HIE
(hemiplegia), both legs (diplegia), both arms and legs
(asphyxia), associated with higher
incidence of MR
Athetosis (involuntary writhing movements) ±
Basal ganglia (may be associated with
Athetoid/Dyskinetic 10-15% chorea (involuntary jerky movements)
Can involve face, tongue (results in dysarthria)
Poor coordination, poor balance (wide based gait)
Ataxic <5% Cerebellum
Can have intention tremor
Marked hypotonia, hyperreflexia, severe cognitive
Atonic <5%
Mixed 10-15% More than one of the above motor patterns

Other Signs

 swallowing incoordination - aspiration

 microcephaly (25%)
 seizures
 mental retardation, learning disabilities
 delay in motor milestones


 may include metabolics, chromosome studies, serology, neuroimaging, EMG, EEG (if seizures), ophthalmology,


 maximize potential through multidisciplinary services; important for family to be connected with various support
 orthopaedic management (e.g. dislocations, contractures, rhizotomy)
 management of symptoms: spasticity (baclofen, botox), constipation (stool softeners)

Contact Dermatitis


 cutaneous inflammation from the interaction between external agent(s) and the skin

Clinical Pearl
Top Ten Allergens as Identified by The North American Contact Dermatitis Group

Test Substance
Nickel Sulfate 14.2 found in some jewelry, buckles
13.1 most commonly used topical antibiotic
Balsam of Peru 11.8 fragrance material
a mix of eight different fragrance components which was developed to allow for
Fragrance mix 11.7
allergen testing in cosmetics
Thimerosal 10.9 a common preservative that is used in vaccines, contact lens solution, cosmetics
Sodium gold 9.5 used in jewellery, dentistry, thiosulfate electronics
a colourless gas found in many workplaces, cosmetics, medications, textiles, resins,
Formaldehyde 9.3
plastic bottles
Quaternium-15 9.0 a component in many shampoos, moisturizers, conditioners and soaps
Cobalt chloride 9.0 a hard metal found in cosmetics, jewellery, buttons, tools
Bacitracin 8.7 a topical antibiotic

Table 8. Contact Dermatitis

Irritant Contact Dermatitis Allergic Contact Dermatitis

Mechanism of cell-mediated delayed (Type IV)
toxic injury to skin; non-immune mechanism
reaction hypersensitivity reaction

Type of reaction erythema, dryness, fine scale, burning acute: quick reaction, sharp
erythema with a papulovesicular
margins (e.g. from acid/alkali exposure) cumulative insult: slow to
eruption, swelling, pruritus
appear, poorly defined margins (e.g. from soap), more common
minority; patient acquires
Frequency of majority; will occur in anyone given sufficient concentration of
susceptibility to allergen that persists
contact dermatitis irritants
dorsum of hand usually involved;
Area of
palmar surface of hand usually involved often discrete area of skin

See above (Clinical Pearl) for most

Examples soaps, weak alkali, detergents, organic solvents, alcohol, oils common examples
many allergens are irritants, so may
coincide with irritant dermatitis
Patch testing to determine specific
Avoid allergen and its cross-reactants
Avoidance of irritants Wet compresses soaked in Burow’s
Compresses solution (drying agent)
Barrier moisturizers Steroid cream (hydrocortisone 1%,
Topical/oral steroids betamethasone valerate 0.05% or
0.1% cream; bid)
Systemic steroids prn (prednisone
1mg/kg, taper over 2 weeks)

Table 9. Topical Treatment of Psoriasis

Treatment Mechanism Comments

lubricants Reduce fissure formation Petrolatum is effective
salicylic acid 1-12% Remove scales
tar (LCD: Liquor carbonis
detergens) Inhibits DNA synthesis, increases cell turnover Poor long term compliance
20% coal tar solution
Binds to skin
1,25-dihydroxyvitamin D3 to inhibit Not to be used on face or skin folds
(Dovonex, Dovobet)
keratinocyte proliferation
Use appropriate potency steroid in different areas
corticosteroid ointment Reduce scaling and thickness
for degree of psoriasis
tazarotene (Tazarac)
Retinoid derivative Use on nails
UVB 290-320 nm or 311 nm
UVB Use with topicals
Narrow Band UVB (NBUVC)

Clinical Pearl
Calcipotriol is a vitamin D derivative
Dovobetâ„¢ = calcipotriene combined with betamethasone dipro-portionate and is considered to be the most potent topical
psoriatic therapy.

Table 10. Systemic Treatment of Psoriasis

Treatment Adverse Effects
Methotrexate Bone marrow toxicity, hepatic cirrhosis
psoralens and long wave ultraviolet radiation (PUVA) Pruritus, burning, cataracts, skin cancer
acitretin Alopecia, cheilitis, teratogenicity, epistaxis, xerosis, hypertriglyeridemia
cyclosporine Renal toxicity, hypertension, immunosuppression
“Narrow band” UVB (311-312 nm) Well tolerated

Table 23. Skin Manifestations of Internal Conditions

Disease Related Dermatoses

Buerger's disease Superficial migratory thrombophlebitis, pallor, cyanosis, gangrene, ulcerations
Cutaneous lupus Sharply marginated annular or psoriaform bright red plaques with scales, telangiectasia, marked scarring,
erythematosus diffuse non-scarring alopecia
Periorbital and perioral violaceous erythema, heliotrope with edema, Gottron's papules (violaceous flat-
topped papules with atrophy), periungual erythema, telangiectasia, calcinosis cutis
Polyarteritis nodosa Polyarteritic nodules, stellate purpura, erythema, gangrene, splinter hemorrhages, livedo reticularis
Rheumatic fever Petechiae, urticaria, erythema nodosum, erythema multiforme, rheumatic nodules
Raynaud's, nonpitting edema, waxy/shiny/tense atrophic skin (morphea), ulcers, cutaneous calcification,
periungual telangiectasia, acrosclerosis
Malar erythema, discoid rash (erythematous papules or plaques with keratotic scale, follicular plugging,
Systemic lupus
atrophic scarring on face, hands, and arms), hemorrhagic bullae, palpable purpura, urticarial purpura,
patchy/diffuse alopecia, mucosal ulcers, photosensitivity
Ulcerative colitis
Pyoderma gangrenosum
Viral (HSV, HZV, HPV, cytomegalovirus, molluscum contagiosum, oral hairy leukoplakia), bacterial
Infections (impetigo, acneiform folliculitis, dental caries, cellulitis, bacillary epithelioid angiomatosis, syphilis), other
Seborrhea, psoriasis, pityriasis rosea, vasculitis
Malignancies Kapos’s sarcoma, lymphoma, BCC, SCC, malignant melanoma

Diagnostic Criteria of SLE: 4 or more of 11 must be present serially or simultaneously (American College of
Rheumatology, 1997 update)
Note: 4, 7, 11 rule => 4 out of 11 criteria (4 lab, 7 clinical) for diagnosis

Criteria Description
Malar rash Classic “butterfly rash”, sparing of nasolabial folds, no scarring
Discoid rash May cause scarring due to invasion of basement membrane
Photosensitivity Skin rash in reaction to sunlight
Oral/nasal ulcers Usually painless
Arthritis Symmetric, involving >2 small or large peripheral joints, non-erosive
Serositis Pleuritis or pericarditis
Neurologic disorder Seizures or psychosis
Proteinuria (>0.5 g/day or 3+)
Renal disorder
Cellular casts (RBC, Hb, granular, tubular, mixed)
Hematologic disorder Hemolytic anemia, leukopenia, lymphopenia, thromboctyopenia
Anti-dsDNA Ab, anti-Sm Ab
Immunologic disorder Antiphospholipid antibodies based on the finding of serum anticardiolipin Ab, lupus anticoagulant,
or false positive VDRL
Antinuclear antibody Most sensitive test (98%)

Scleroderma/Progressive Systemic Sclerosis (PSS)

a non-inflammatory disorder characterized by widespread small vessel vasculopathy and fibrosis, which occurs in the setting of
immune system activation and autoimmunity


 diagnostic criteria: 1 major or >2 minor criteria

o major criterion: proximal scleroderma
o minor criteria: sclerodactyly, digital pitting scars or loss of substance from finger pads, bibasilar pulmonary
 serology
o anti-topoisomerase 1: specific but not sensitive for systemic sclerosis
o anti-centromere favours diagnosis of CREST variant (limited systemic sclerosis)

Etiology and Pathophysiology

 idiopathic vasculopathy (not vasculitis) leading to atrophy and fibrosis of tissues

o intimal proliferation and media mucinous degeneration ––> progressive obliteration of vessel lumen >
fibrotic tissue
o resembles malignant hypertension


 F:M = 3-4:1, peaking in 5th and 6th decades

 associated with HLA-DR1
 associated environmental exposure (silica, epoxy resins, toxic oil, aromatic hydrocarbons, polyvinyl chloride)

Figure 10. Forms of Scleroderma

CREST Syndrome
Calcinosis - calcium deposits on skin
Raynaud’s phenomenon
Esophageal dysfunction - acid reflux
Sclerodactyly - tightening of skin
Telangiectasia - superficial dilated blood vessels

Signs and Symptoms

Table 9. Clinical Manifestations of Scleroderma

System Features
Initial phase characterized by painless non-pitting edema
Progressive bilateral swelling of fingers, hands and feet leading to skin tightening
Characteristic face: mask-like facies with tight lip, beak nose, radial perioral furrows
Atrophy, ulcerations, hypo/hyperpigmentation, telangiectasias, calcinosis, periungual erythema, pruritus
Episodes (minutes to hours) of well-demarcated blanching and/or cyanosis of digits followed by
erythema (Raynaud’s phenomenon), tingling and pain
Vascular Due to vasospasm following cold exposure or emotional stress
If severe, can result in infarction of tissue at fingertips => digital pitting scars, frank gangrene or
autoamputation of the fingers or toes
GI tract becomes a rigid tube leading to decreased motility
Distal esophageal hypomotility > dysphagia
Loss of lower esophageal sphincter function => GERD, ulcerations, strictures
Small bowel hypomotility => bacterial overgrowth, diarrhea, bloating, cramps, malabsorption, weight
Large bowel hypomotility => pathognomonic radiographic finding on barium study is large bowel wide
mouth diverticuli
Mild proteinuria, creatinine elevation and/or hypertension are common
Renal “Scleroderma renal crisis (10-15%) may lead to malignant arterial hypertension, oliguria and
microangiopathic hemolytic anemia
Pulmonary Interstitial fibrosis, pulmonary HTN, pleurisy, and pleural effusions
Cardiac Left ventricular dysfunction, pericarditis, pericardial effusion, arrhythmias
Polyarthralgias => polyarthritis affecting both small and large joints
Subcutaneous calcifications (calcinosis)
Resorption of distal tufts (radiological finding)
Proximal weakness 2o to disuse, atrophy, low grade myopathy
Endocrine Hypothyroidism common

Clinical Pearl

Scleroderma is the most common cause of secondary Raynaud’s phenomenon.


 treatment is tailored to organ system involved

 dermatologic
o good skin hygiene
o low dose prednisone, methotrexate (limited evidence)
 vacular (Raynaud’s)
o patient education on cold avoidance
o vasodilators (CCBs, local nitroglycerine cream, systemic PGE2 inhibitors)
 gastrointestinal
o gastroesophageal reflux disease (GERD): PPIs are first line, then H2-receptor agonists
o small bowel bacterial overgrowth: broad spectrum antibiotics (tetracycline, metronidazole)
 renal disease: ACE inhibitors
 cardiac
o pericarditis: systemic steroids
o pulmonary hypertention: bosetan (Tracleerâ„¢), epoprostenol (Flolanâ„¢). sildenafil (ViagraTâ„¢)
 musculoskeletal
o myositis: systemic steroids

Patterns of Joint Involvement

 symmetrical vs. asymmetrical

 small vs. large
 mono vs. oligo vs. polyarticular
 axial vs. peripheral

Table 3. Differential Diagnosis of Joint Pain: Patterns of Joint Involvement

Symmetrical Asymmetrical
Large Joint Polyarthritis
• Seronegative disorders
• Ankylosing spondylitis
• Psoriatic arthritis
• Rheumatoid arthritis
• Reactive arthritis
• Polymyalgia rheumatica
• Infectious arthritis
• Osteoarthritis
• Crystal-induced arthritis
Small Joint Polyarthritis
• Psoriatic arthritis
• Seropositive disorders (RF+, ANA+)
• Tophaceous gout
• Psoriatic arthritis

Extra-Articular Features

 consider skin and appendages, eyes, lungs, cardiac, pulmonary, GI, GU, neurologic, psychiatric

Table 4. Seropositive vs. Seronegative Rheumatic Diseases

Seropositive Seronegative
Demographics F>M M>F
Usually larger joints, lower extremities
Symmetrical (Psoriatic arthritis may be the exception)
Peripheral Arthritis Small and large joints Dactylitis
DIP less involved Enthesitis
DIP in Psoriatic arthritis
Pelvic/Axial Disease No (except for C-spine) Yes
Enthesitis No Yes
Iritis (= Anterior Uveitis)
Oral ulcers
Extra-Articular GI
Raynaud’s phenomenon
Dermatological features

Joint Pain Causes: SOFTER TISSUE

Seropositive arthritides
Seronegative arthritides
Urate (gout)/other crystal
Extra-articular rheumatism (polymyalgia/fibromyalgia)

Red Flag
Septic Arthritis is a Medical Emergency! Consider empiric antibiotic treatment until septic arthritis is excluded by history,
physical exam and synovial fluid analysis. (see Infectious Diseases)
Figure 2. Differential Diagnosis of Joint Pain

 primary (idiopathic)
o most common, of unknown etiology
 secondary
o post-traumatic or mechanical
o post-inflammatory (e.g. RA) or post-infectious
o heritable skeletal disorders (e.g. scoliosis)
o endocrine disorders (e.g. acromegaly, hyperparathyroidism, hypothyroidism)
o metabolic disorders (e.g. gout, pseudogout, hemochromatosis, Wilson’s disease, ochronosis)
o neuropathic (also known as Charcot joints)
 atypical joint trauma due to loss of proprioceptive senses (e.g. diabetes, syphilis)
o avascular necrosis (e.g. fracture, steroids, alcohol, gout, sickle cell)
o other (e.g. congenital malformation)

Etiology and Pathophysiology

 abnormal physical forces lead to altered joint function and damage

 primary event is deterioration of articular cartilage due to local biomechanical factors and release of proteolytic and
collagenolytic enzymes
o OA develops when cartilage catabolism > synthesis
o loss of proteoglycans and water exposes underlying bone
 abnormal local bone metabolism further damages joint
 synovitis is secondary to cartilage damage therefore may see small effusions in OA

 most common arthropathy (12% of age 25-74)
 increased prevalence with increasing age (35% of 30-year olds, 85% of 80-year olds)

Risk Factors

 genetic predisposition, advanced age, obesity (for knee OA), female, trauma

Signs and Symptoms

 signs and symptoms localized to affected joints (not a systemic disease)

 pain is often insidious, gradually progressive, with intermittent flare-ups and remissions

Table 5. Signs and Symptoms of OA

Symptoms Signs
Joint pain with motion; relieved with rest joint line tenderness; stress pain
short duration of stiffness (<1/2 hr) after immobility bony enlargement at affected joints
Joint instability/buckling malalignment/deformity (angulation)
Joint locking due to “joint mouse” (bone or cartilage fragment) limited ROM
loss of function or other internal derangements (e.g. meniscal tear) crepitus on passive ROM
inflammation (mild if present)
periarticular muscle atrophy

OA of MCP joints can be seen in hemochromatosis or chondrocalcinosis.

Joint Involvement

 asymmetric joint involvement

 any joint can be affected especially knee, hip, hand, spine (See Figure 3 below)
 hand
o DIP (Heberden’s nodes = osteophytes => enlargement of joints)
o PIP (Bouchard’nodes)
o CMC (usually thumb squaring)
o MCP is usually spared (except the 1st MCP)
 hip
o dull or sharp pain in trochanter, groin, anterior thigh, or knee
o internal rotation and abduction are lost first
 knee
o narrowing of one compartment of the knee is the rule, medial > lateral
o standing x-rays must be done (not supine)
 foot
o common in first MTP
 lumbar spine
o very common especially L4-L5, L5-S1
o degeneration of intervertebral discs and facet joint degeneration
o reactive bone growth can contribute to neurological impingement (e.g. sciatica, neurogenic claudication) or
listhesis (slippage)
 cervical spine
o commonly presents with neck pain, especially in lower cervical area


 blood work
o normal CBC and ESR
o negative RF and ANA
 synovial fluid => non-inflammatory
 radiology: 4 hallmark findings

Hint: Bouchard's is closer to the Body


 presently no treatment alters the natural history of OA

 non-pharmacological therapy
o weight loss (minimum 5-10 lbs. loss)
o rest/low-impact exercise
o physiotherapy with heat/cold,exercise programs
o occupational therapy ––> aids, splints, cane, walker, bracing
 medical therapy
o NSAIDs, acetaminophen (see Common Medications)
o hyaluronic joint injections (Hyalganâ„¢, Synviseâ„¢, etc.)
o nutraceuticals: glucosamine ± chondroitin
 surgical treatment
o joint debridement, osteotomy, total and/or partial joint replacement, fusion

Clinical Pearl
The Radiographic Hallmarks of OA
1. joint space narrowing
2. subchondral sclerosis
3. subchondral cyst formation
4. osteophytes
Figure 3. Common sites of involvement in OA
Figure 4. Brouchard's and Heberden's nodes
Causes of Optic Disc Edema

Anterior Central Retinal Vein

Optic Neuritis Papilledema Ischemic Occlusion
Age <50 any >50 >50
Vision rapidly progressive central vision usually no visual loss until acute field defects variable visual loss
loss late
acuity affected possible transient decreased colour
decreased colour vision obscuration vision
variable acuity
normal colour vision
Other tender globe, painful on motion headache typically cardiovascular risk factor
symptoms may alternate eyes in multiple nausea unilateral usually unilateral
sclerosis vomiting
focal neurological deficits
Pupil no anisocoria anisocoria no anisocoria
RAPD present no RAPD RAPD present no RAPD
Fundus anterior have disc swelling disc swelling and retinal pale segmental blood and thunder: swollen
variable papillitis hemorrhages disc edema with disc, venous engorgement,
absent venous pulsations retinal dot and/or retinal hemorrhages
Etiologies MS, viral infection increased ICP (e.g. mass giant cell arteritis idiopathic
associated with MS in 74% of lesion pseudotumour
females and 34% of males cerebri, malignant
Treatment IV methylprednisolone may reat specific cause of consider ASA for panretinal laser
shorten attacks; oral prednisone increased ICP non-arteritic photocoagulation, steroids
may increase relapse rate consult Neurosurgery

Adult BLS sequence (From Wikipedia)

 Ensure that the scene is safe.

 Assess the victim's level of consciousness by asking loudly "are you okay?" and by checking for the victim's
responsiveness to pain.

 Activate the local EMS system by instructing someone to call 911. If an AED is available, it should be retrieved and

 If the victim has no suspected cervical spine trauma, open the airway using the head-tilt/chin-lift maneuver; if the victim
has suspected trauma, the airway should be opened with the jaw-thrust technique. If the jaw-thrust is ineffective at
opening/maintaining the airway, a very careful head-tilt/chin-lift should be performed.

 Assess the airway for foreign object obstructions, and if any are visible, remove them using the finger-sweep technique.
Blind finger-sweeps should not be performed, as they may push foreign objects deeper into the airway.

 Look, listen, and feel for breathing for at least 5 seconds and no more than 15 seconds.

 If the patient is breathing normally, then the patient should be placed in the recovery position and monitored and
transported; do not continue the BLS sequence.
 If patient is not breathing normally, and the arrest was witnessed immediately before assessment, then immediate
defibrillation is the treatment of choice[1].

 Attempt to administer two artificial ventilations using the mouth-to-mouth technique, the mouth-to-mask technique, or a
bag-valve-mask. Verify that the chest rises and falls; if it does not, reposition (i.e. re-open) the airway using the appropriate
technique and try again. If ventilation is still unsuccessful, and the victim is unconscious, it is possible that they have a
foreign body in their airway. Begin chest compressions, stopping every 30 compressions, re-checking the airway for
obstructions, removing any found, and re-attempting ventilation.

 If the ventilations are successful, assess for the presence of a pulse at the carotid artery. If a pulse is detected, then the
patient should continue to receive artificial ventilations at an appropriate rate and transported immediately. Otherwise,
begin CPR at a ratio of 30:2 compressions to ventilations at 100 compressions/minute for 5 cycles.

 After 5 cycles of CPR, the BLS protocol should be repeated from the beginning, assessing the patient's airway, checking
for spontaneous breathing, and checking for a spontaneous pulse. Laypersons are commonly instructed not to perform re-
assessment, but this step is always performed by healthcare professionals (HCPs). If an AED is available after 5 cycles of
CPR, it should be attached, activated, and (if indicated) defibrillation should be performed. If defibrillation is performed, 5
more cycles of CPR should be immediately repeated before re-assessment.

 BLS protocols continue until (1) the patient regains a pulse, (2) the rescuer is relieved by another rescuer of equivalent or
higher training, (3) the rescuer is too physically tired to continue CPR, or (4) the patient is pronounced dead by a medical

 At the end of five cycles of CPR, always perform defibrillation (AED), and repeat assessment before doing another five

 CPR continues indefinitely, until the patient is revived, or until the caregiver is relieved, or discharged by a higher medical

 The CPR cycle is often abbreviated as 30:2 (30 compressions, 2 ventilations or breaths). Note CPR for infants and
children uses a 15:2 cycle when two rescuers are performing CPR (but still uses a 30:2 if there is only one rescuer)


 Rescuers should provide CPR as soon as an unresponsive victim is removed from the water. In particular, rescue
breathing is important in this situation.

 A lone rescuer should give 3 cycles of CPR before leaving the victim to call emergency medical services. A cycle of
CPR consists of giving 30 chest compressions and 2 breaths to the victim.

Since the primary cause of cardiac arrest and death in drowning and choking victims is hypoxia, it is more important to provide
rescue breathing as quickly as possible in these situations, whereas for victims of VF cardiac arrest chest compressions and
defibrillation are more important.

[edit] Hypothermia

 In unresponsive victims with hypothermia, the breathing and pulse should be checked for 30 to 45 seconds as both
breathing and heart rate can be very slow in this condition.

 If cardiac arrest is confirmed, CPR should be started immediately. Wet clothes should be removed, and the victim
should be insulated from wind. CPR should be continued until the victim is assessed by advanced care providers.

Foreign body airway obstruction (choking)

 Rescuers should intervene in victims who show signs of severe airway obstruction, such as a silent cough, cyanosis, or
inability to speak or breathe.

 If a victim is coughing forcefully, rescuers should not interfere with this process.

 If a victim shows signs of severe airway obstruction, abdominal thrusts should be applied in rapid sequence until the
obstruction is relieved. If this is not effective, chest thrusts can also be used. Chest thrusts can also be used in obese
victims or victims in late pregnancy. Abdominal thrusts should not be used in infants under 1 year of age due to risk of
causing injury.

 If a victim becomes unresponsive he should be lowered to the ground, and the rescuer should call emergency medical
services and initiate CPR. When the airway is opened during CPR, the rescuer should look into the mouth for an
object causing obstruction, and remove it if it is evident.