Clinical Flashcards
100% of modular RA will have which factor present
Rheumatoid factor
What are the hematologists findings to support SLE
- Hemolytic anemia with reticulocytes is
- Leukopenia
- Lymphopenia on >1 occasion
- Thrombocytopeina
What is the most common cause of arthritis
Osteoarthritis due to the increased pts with obesity and older age
When does osteoarthritis get better and worse
Better with rest, worse with activity
What is circulate balantitis
Lesions and crustation on the glans penis as a result of reactive arthritis
What is the allele that has an increased risk for RA
HLA-DB4
What is Parsonage Turner syndrome and what is the cause
Severe pain in the shoulder area followed wiring a few days of weaknes and atrophy in the shoulder girdle
*Most likely due to autoimmune causes since it follows infections, but will self resolve, but can be helped with steroids
ANA helps to support diagnosis of which condition
SLE
High arching foot with flexed toes usually are an indication for which conditions
Chronic Neuropathy
CMT1
Radiculopathy at the nerve root C7
Elbow extension weakness (triceps)
Wrist extent
Finger ext
ANTI CCP and RF positivity is indicative for which condition
99.5% specificity for RA
What are the presentations in those with psoriatic arthritis
30-50, equal sex ratio
-psoriasis (only 20-50% ahve B27)
-SI and axial
Which region of the body does the dermatome cover with regards to: L4
Medial calf
What is the location in the vertebra that tends to be affected by RA
Only C1/C2
Fabry’s disease is assocaited with which problems and which enzyme
Alpha galactosidease
-Polyneuropathioes and renal issues
High levels of Rheumatoid factor usually correlate which what
Increased levels correlate with an increased aggressiveness
What are the lab and test findings in multifocal motor neuropathy
- GM1 antibody (50-80%)
- EMG shows conduction block
- Normal CSF
What is the term for 5 or more joints involved
Pauli
Which region of the body does the dermatome cover with regards to: T4
Nipple line
What are the clinical manifestations a pt with bulbar palsy will show
Dysarthria, dysphagia, dysphonia, chewing issues, drooling, respiratory difficulties
At 12 months of age, what should a child be able to do with regards to gross motor
Stands momentarily
*Should be able to say momma/dadda (specific)
Which test can SLE give a false positive
Syphilis
What is MUSK syndrome
Aka antibody negative myasthenia gravis
- Oculopharyngeal weakness
- Neck, shoulder, respiratory weakness
- Indistinguishable from Ab positive MG
What is the term used for 3 or more joints involved
Oligo
What is CREST syndrome
Calcium deposition in skin Reynauds Esophageal dysmotility Sclerodatcyly Telangiectasias
What is DISH and what is normally seen
Diffuses idiopathic Skeletal hyperosteosis
*Calcification along lateral aspect of four contiguous vertebral bodies, but the SI joint is fine
What form of CMT (hereditary motor sensory neuropathies) is most common
Type 1, which is demyelinating
*2 is less common, as is axonal degradation
What are the conditions that rheumatoid factor are present in
- Sjogrens (95%)
- Cyroglobulinemia (40-100%)
- primary biliary cirrhosis (70%)
- mixed connective tissue (60%)
- Endocarditis (50%)
- SLE (35%)
What are the nerve findings in the cause of GBS
Slow conduction velocity, focal conduction block with prolonged F waves
What is the damaged structure in the cause of Bronchial plexopathy caused by neoplastic
Medial cord
*Painful in the breast or lung
What is the genetic cause of DMD
Frameshift so no dystrophin
X linked recessive
What is the upside and downside of gadolinium
Upside: taken up by inflamed tissues so good to see in RA
Downside: toxic to the kidneys
What are the clinical signs of soon with CMT
Child who is clumsy, trouble walking or running
-slowing of nerve conductions
What are the important negatives seen in ALS
Negatives: -No eye involvement -Normal mental status -No extraocular muscle involvment -Bowl and bladder symptoms absent -Decubiti rare (aka bed sores) Fasciculations are rarely the presenting sx
What is the most common myopathy
DMD
What is the therm for 6 or more joints involved.
Poly
Large nerve fibers transmit which type of information and what would be the negative and positive signs if those nerves were affected
Vibration, joint position
Positive: tingling, pins and needles, numbness
Negative: loss of vibrations, Joint position, areflexia, ataxia, hypotonia
What nerves are affected in progressive bulbar palsy
Lower cranial nerves (CN 9, 10,)
At 9 months of age, what should a child be able to do with regards to gross motor
Pulls up
Cruises
Sits well without support
Radiculopathy at the nerve root C5
Shoulder abduction weakness (deltoid)
What condition is commonly associated with lambert Eaton myasthenia syndrome (LEMS)
Cancers (especially small cell lung cancer)
At 48 months of age, what should a child be able to do with regards to gross motor
Balance on one foot
Hop on one foot
What is the damaged structure in the cause of Bronchial plexopathy caused by radial injury
Upper trunk, lateral cord
*Painless
What is the clinal Presentation in someone with multifocal motor neuropathy
Adult males were there is neuropathy of one nerve, then slowing progressing from distal weakness
-No UMN signs, or sensory signs
What are the most common cervical radiculopathies involved
C5-C6 (C6 nerve compression)
C6-C7 (C7 nerve root compression)
What is a treatment to help a patient with DMD
Steroids if older than 5
What is the damaged structure in the cause of Bronchial plexopathy caused by ischemia
Usually diabetic causes in the lumbar area
What are keratoderma blennorrhagicum
Painless lesions on the feet as a result with reactive arthritis and reiter’s syndrome
Why is the EMG normal on a small nerve polyneuropathy
Because it is unmyelinated
Which form of arthritis is seen with those diabetic patients
- Charcot’s
- Cheiroarthropathy
Radiculopathy at the nerve root C8
Finger abd
Finger flex
At 6 months of age, what should a child be able to do with regards to gross motor
Sits momentarily
Which region of the body does the dermatome cover with regards to: T10
Umbilicus
What is chronic inflammatory demyelinating polyneuropathy (CIDP)
Basically similar to GBS, except is chronic and occurs over a longer time and is more persistent
Which term is used for one joint involved
Arthritis
Radiculopathy at the nerve root C6
Shoulder abduction weakness (deltoid)
Elbow flex (biceps)
What muscle groups tend to be involved in spinal muscular atrophy
Upper extremity involvement
What complication is seen in those with DMD
Cardiomyopathy
What is commonly present in chronic inflammatory demyelinating polyneuropathy (CIDP)
15% have a monoclonal antibody (IgM or IgG)
If someone had breast cancer that was causing weakness in the extremities, how would you be able to tell if it was from breast cancer or the radiation afterwards
Radiation causes painless weakness while neoplastic will be painful
What are the classical clinical presentations of juvenile dermatomyositis
- Proximal muscle weakness
- Red or purplish heliotrope rash over the eyelids
- thrombi or hemorrhage in the periungual capillary beds
- Raised erythematous papules over the extensors Joints
What is podagra
Inflammation of the big toe, seen in gout
What is the best imaging for erosion on bones and inflammatory arthritis
CT
Spinal muscular atrophy involves which set of motor neurons.
Only the lower motion neurons. Showing atrophy
What is a classical sign of ALS
Mixed upper and lower motor neuron signs, usually in the same limb
Upper: Babinski sign, hyperreflexia, spasticity
Lower: Atrophy, fasciculations
What is the Gowers maneuver.
When a child is laying down on their stomach. Then kind of walks their way up to standing
How does lambert Eatoon myasthenic syndrome differ from MG with regards to increased situation
MG gets worse while LEMS gets better due to increased stimulation on the calcium channels to release the ACh into the synaptic cleft
If the EMG is normal in trying to determine the form of CMT, which form is present
Type 2, because it is axonal loss, rather than type 1 that is demyelinating that slows nerve conduction
At 24 months of age, what should a child be able to do with regards to gross motor
Walks up stairs
Kicks ball forward
What is Feltys syndrome
Splenomegaly Neutropenia Fever Anemia Thrombocytopenia
What is the most common idiopathic inflammatory myopathy in children
Juvenile dermatomyositis
What is the most common type of adult motor neuron diseases
ALS
Seeing clubbing and arthritis in a patient is indicative of which condition until proven otherwise
Interstital lung disease
Radiculopathy at the nerve root L5
Hamstring weakness
Foot forsiflexion, inversion, eversion
Sensory: lateral calf, dorsum of foot
What is Miler fisher syndrome
In 5% of GBS patients, they proceed to:
- Ophthalamoplkegia, ataxia, arreflexia
- Facial weakness, dysarthria, dysphagia
Small nerve fibers transmit which type of information and what would be the negative and positive signs if those nerves were affected
Pain and temp
Positive: Burning, jabbing pain
Negative: Loss of pain and temp
What is the treatment for someone with RA
1) Start with an NSAID
2) Steroid (quick fix)
3) DMARDs
Which region of the body does the dermatome cover with regards to: T1
Medial forarm
Which region of the body does the dermatome cover with regards to: C7
Middle finger
What is the inheritance of CMT 1
AD
What are the clinical manifestations of childhood motor neuron diseases
Hypotonia, arreflexia, poor sucking, breathing difficulty, death in 6 to 12 months
What is the gene that is mutated in myotonia congenita
Chloride channel CLCN1
Which joints tend to be affected by osteoarthritis
Weight bearing joints such as the knees, hips, spine
What are the key findings in the CSF in the case of GBS
Albumino-cytological dissociation
Aka increased protein with normal glucose and normal cell count
To help determine which nerve or radicupathy might be present, which finger can you look at
Rink finger. If its the whole finger, then its the median and ulnar, if its just the lateral half of the finger, then its median only
What is Lambert Eaton myasthenia syndrome (LEMS) due to
Autoimmune attach against voltage gated calcium channels on the presynaptic terminal
Why fibers can not be tested on an EMG
Unmyelinated fibers
Which region of the body does the dermatome cover with regards to: C8
Fourth/fifth finger
What symptom may be present in the case of mitochondrial disorders
Mitochondrial encephalomyopathy with lactic acidosis and stroke like symptoms or MELAS
In those patients with antibody negative MG, which antibody is present
MUSK (muscle specific tyrosine kinase) Abs
What are some atrophic locations seen in ALS
Tongue, hands, arms
Hypertrophied pronator teres can result in monneuropathy in which nerve
Median nerve
Which region of the body does the dermatome cover with regards to: C6
Thumb/index
What is Rieter’s syndrome
Can’t see (Conjunctivitis)
Can’t pee (urethritis)
Can’t climb tree (reactive arthritis)
Which antibodies are present in Miler fisher syndrome
GQqb and GT1a antibodies
Autonomic nerve fibers transmit which type of information and what would be the negative and positive signs if those nerves were affected
Negative: Hypotension, decreased sweat, impotence, urinary retention, constipation
How will hip osteoarthritis manifest
As groin pain
Rheumatoid factor is an autoantibodies of which class and to what
Most commonly IgM, usually to the Fc portion of IgG
Administration of which drug can produce a disorder very similar to myasthenia gravis
D-penicillamine, and it was recipe when removed from treatment
What are the depicting features of enteropathic arthritis
- Associated with Crohns and UC
- Axial involvement
- IBD and arthritis will correlate with one another and flare up at the same time
- Erythema nodosum
*B27
Radiculopathy at the nerve root L4
Hip flexor weakness
Knee extension weakness
Sensory: medial calf
What is the inheritance of myotonia congenita
AD
At 36 months of age, what should a child be able to do with regards to gross motor
Tricycle
What are the clinical manifestations of osteoarthritis
Crepeitus, Decreased ROM, effusion that is cold
What are the most common lumbar nerve root compressions
L4-L5 (L5 nerve root compression)
L5-S1 (S1 nerve root compression)
ACA is helpful in the diagnosis of which condition
Scleroderma
What is the usual associations with reactive arthritis
- Young male
- Arthritis of LEs
- Enthesitits (Achilles tendon/plantar fasciitis)
- Dactylitis (sausage digit, finger or toe)
What are the features of psoriatic arthritis that are Give always
- Psiariatic lesions
- Pitting nails
What is pyroderma gangrenosim and what condition is it associated with
Tender, reddish purple papule, leading to necrosis
*Seen with RA
What is the damaged structure in the cause of Bronchial plexopathy caused by traumatic injury
Traction, laceration, missed
Which HLA are associated with myasthenia gravis
HLA B8 and DR3
Which region of the body does the dermatome cover with regards to: L5
Lateral calf
Which form of arthritis can be seen in those patients with a thyroid issue
Carpal/tarsal tunnel syndrome
Radiculopathy at the nerve root S1
Hamstring weakness
Foot plantarflexion
Sensory: postolateral calf, lateral foot
Mitochondrial diseases leading to myopathies usually have what present
Abs to mitochondrial or nuclear DNA mutations
Lateral sclerosis involves which set of motor neurons
Upper motor neurons giving you hyperreflexia and Babinski sign
Which region of the body does the dermatome cover with regards to: L1
Inguinal
What are the renal manifestations that would help conform SLE
Proteinuria > 500mg/day or 3+ cases
At 70 months of age, what should a child be able to do with regards to gross motor
Skips
What are the four domains of development
- Gross motor
- Fine motor
- Language
- Cognitive/social emotional
