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					                                                                                  Case #1
                                                         • 43y/o AA male presents with six week h/o
                                                            – Right lower extremity edema
         Clinical Problem Solving                        • c/o right sided scrotal pain
                 7/19/2005                                  – Increased with walking and relieved by rest
                                                         • DOE at 1 block, no pleuritic chest pain, No
                Presenter: Stuart Cohen MD                 orthopnea or PND
           Discussant: Gustavo Heudebert MD              • No previous h/o DVT, recent travel or immobility
                                                         • Denies fevers, chills, night sweats or cough




                         Case #1                                                  Case #1
• Denies dysuria, frequency, urgency or other            • PMH:
  lower tract obstructive symptoms                          –   Hypertension
                                                            –   Migraine Headaches
• No recent lower extremity trauma                          –   GERD
• Denies h/o of previous STD’s                              –   Chronic lower back pain
• evaluated in ED 3 weeks PTA                            • Meds:
                                                            –   Atenalol/ daily
   – Duplex Ultrasound negative for DVT                     –   Amitriptyline/ hs
                                                            –   Omeprazole/ daily
                                                            –   Sumatriptan/ prn
                                                            –   Ibuprofen/ prn
                                                            –   Percocet/ prn




                         Case #1                                                  Case #1
• Soc hx:                                                • PE:
   –   Lives in Birmingham with mother                      – AF, 20, 75, 132/74, 99% RA sat
   –   Tobacco (1/2 ppd x 20 yrs)                           – HEENT: normal
   –   Occ ETOH and marijuana                               – CV: rrr without m/r/g
   –   Denies IVDA                                          – Lungs: CTA bilaterally w/o wheezes, crackles or rubs
   –   works as a stocker                                   – Abd: soft, non distended, no organomegaly, BS present, No
• FH:                                                         ascites, no bruits
   – Father with CAD (dx at 55)                             – Gu/rectal: normal rectal tone, prostate minimally enlarged, no
                                                              scrotal edema or testicular tenderness, guiac negative
   – No family h/o of cancer or blood disorders
                                                            – Ext: 3+ RLE edema, no discoloration or cellulitis, 2+ distal
• ROS:                                                        pulses. LLE without edema. No significant arthritic changes
   – Negative except for persistent migraine headaches      – Straight leg raising negative, negative psoas sign




                                                                                                                               1
                      Case #1                                                   Case #1
• Labs:                                                   • Radiology:
  –   WBC 11, HCT 40, plts 504                               – Helical CT: No PTE
  –   BUN 14, Cr 1.6, HCO3 28, BS 92                         – Lower Ext U/S: DVT in right common femoral and ext
  –   AST 15, Alb 4.2, Bili 0.4, INR 1.0                       iliac veins with right hydoureteronephrosis
  –   U/A: Negative
  –   DDimer: Positive                                    • CT Abd/Pelvis
                                                             – Soft tissue mass circumferentially surrounding the
                                                               right common iliac artery extending into the pelvis.
                                                             – Dilation of the right renal collecting system and ureter
                                                             – Right lower extremity DVT




                      Case #1                                                   Case #1
• CT ABD/pelvis                                           • Pathology:
                                                             – Fibrosis with increased lymphocytic response
                                                             – Flow cytometry : no monoclonal population or
                                                               evidence of lymphoma
                                                          • Further history:
                                                             – Has tried multiple medications over the years for
                                                               migraines including Ergot derivatives.




Idiopathic Retroperitoneal Fibrosis                        Idiopathic Retroperitoneal Fibrosis
• Also known as Ormand’s disease                          • Diagnostic Evaluation:
• Clinical Manifestations:                                   – Ultrasound
  – Most commonly seen between 40-60 years of age            – CT is examination of choice to visualize the extent of
                                                               the fibrosis and assess for the presence of
  – 2:1 male predominance                                      lymphadenopathy and tumor
  – Initially, dull, noncolicky pain occuring in girdle
    distribution
                                                          • Biopsy
  – Insidious onset makes diagnosis difficult                – Almost always required to exclude
                                                               malignancy/lymphoma (open biopsy preferred)
  – Urinary obstruction occurs later
  – Lower extremity edema can occur due to arterial
    obstruction most commonly of IVC




                                                                                                                          2
 Idiopathic Retroperitoneal Fibrosis                         Idiopathic Retroperitoneal Fibrosis
                                                            • Drugs implicated in causing RPF
                                                               – methysergide, methyldopa, DHE, pergolide,
                                                                 Beta-blockers
                                                               – LSD, amphetamines, and cocaine.




  Ergot derivatives and fibrosis                                                  Case #2
• Inflammatory fibrosis is a rare adverse reaction          • 75 y/o male presents with 5 month h/o of
  associated with prolonged use of methysergide               weakness
  and other ergot derivatives.                                 – Seeking VA disability for “arthritis”
• Retroperitoneal fibrosis, pulmonary fibrosis, or             – Initially generalized weakness with arthralgias
  fibrosis in cardiac tissue can occur.                        – Progressive weakness of primarily upper extremities
• Drug withdrawal usually reverses the condition,              – Now unable to dress self and perform ADLs including
                                                                 feeding self
  – treatment should be interrupted for at least a 3-week
    interval every 6 months.                                   – Fallen on several occasions




                    Case #2                                                       Case #2
• Denies                                                    • PMH:
  – Fevers, chills, night sweats                               – CAD (s/p CABG in past)
                                                               – Hypothyroidism
  – Nausea, vomiting, constipation
                                                               – Hypokalemia
  – Numbness, parasthesias or muscle pain                      – Degenerative Arthritis
  – Shortness of breath, air hunger or cough                • Meds:
  – Dysphagia, choking when eating                             –   Atenalol
                                                               –   Levothyroxine
                                                               –   Potassium Chloride
                                                               –   Ibuprofen




                                                                                                                       3
                      Case #2                                                        Case #2
• Sochx:                                                      • PE:
  – Lives alone (neighbor/friends taking care of pt             – AF, 128/88, 88, 18, 168 lbs
    for past several months)                                    – HEENT: PEERLA, EOMI, sclera anicteric
  – Retired truck driver                                        – Neck: No JVD, thyromegaly or carotid bruits
  – Smokes, remote alcohol, No IVDA                             – CV: rrr w/ 2/6 systolic murmur, no S3
• Famhx:                                                        – Lungs: CTA bilaterally
  – Mother died of MI in 80’s                                   – ABD: S/NT/ND/BS present, No HSM
  – Father died from complications of ETOH                      – Ext: No C/C/E, skin dry and scaly




                      Case #2                                                        Case #2
• PE: Neuro                                                   • Sensation intact to
  – CN: PERRLA, EOMI, visual fields full , mild facial          – light touch/pinprick/proprioception
    weakness bilaterally (puffing cheeks), sensation
    intact, no tongue fasciculations, shoulder shrug intact   • DTR’s
• Upper Extremity                                               –   Absent in UE bilaterally except 1+ in triceps
  –   1/5 shoulder flexion/extension/abduction                  –   1+ and symmetric at knees and ankles
  –   3/5 biceps/triceps                                        –   Plantar reflex: down going bilaterally
  –   3/5 wrist flexion & extension                             –   No pathologic reflexes
  –   3/5 hand grip                                           • Mild Interosseous, thenar and hypothenar
• Lower Exremity                                                atrophy
  – 4/5 hip flexion/extension
  – 5/5 distal muscle groups
                                                              • Fasciculations noted in deltoids




                      Case #2                                                        Case #2
• Labs:                                                       • Radiology:
  – Chem 7: normal, Calcium 8.6                                 – MRI Brain:
  – CBC: normal                                                      • No evidence of previous CVA, mass lesion or
                                                                       demyelination
  – CPK: 172
                                                                – MRI C-spine
  – AST 16, ALT 7, LDH 200, Bili 0.5, INR 1.02
                                                                     • Severe C3-C6 discogenic degenerative changes
  – ANA <1:40, ESR 24                                                  with moderate stenosis. No other abnormalities
  – TSH 4.02, FT4 1.0                                                  noted
  – B12 247, Folate13.7                                         – CXR
  – Cortisol 4.0                                                     • Emphysematous changes




                                                                                                                        4
                                                                         Amyotrophic Lateral Sclerosis
                       Case #2
                                                                                    (ALS)
• EMG/NCS:                                                           • Also known as
                                                                        – Lou Gehrig’s Disease
  – Acute primary axonal motor neuropathy
                                                                        – Motor Neuron Disease (England)
     • (Consistent with motor neuron disease)
                                                                     • Progressive neurodegenerative disorder that affects
                                                                       motor neurons located in the anterior (ventral) horn
                                                                       region of the spinal cord, lower cranial nerve motor
                                                                       nuclei in the medulla and the frontal cortex.




    Amyotrophic Lateral Sclerosis                                        Amyotrophic Lateral Sclerosis
               (ALS)                                                                (ALS)
• Hallmark of disease is the combination of UMN                      • Clinical Pattern of progression
  and LMN findings                                                      – Combination of UMN and LMN in one limb
  – LMN findings                                                        – Despite muscle weakness and atrophy, DTR’s
     • Weakness, atrophy and fasciculations result form muscle            are paradoxically brisk
       denervation   “amyotrophic”
                                                                        – Weakness eventually spreads to neck,
  – UMN findings                                                          tongue, pharyngeal and laryngeal muscles
     • Hypereflexia and spasticity result from degeneration of the
       lateral corticospinal tract which is hardened at autopsy         – Later, weakness spreads to trunk and lower
       “lateral sclerosis”                                                extremities
                                                                        – Sensory changes are absent
                                                                        – Cognitive decline noted in minority of patients




    Amyotrophic Lateral Sclerosis                                        Amyotrophic Lateral Sclerosis
               (ALS)                                                                (ALS)
• Survival:                                                          • Diagnosis of ALS requires
  – Most die within 3- 5 yrs although long term
    survival is not rare                                                – Evidence of LMN degeneration by clinical, elctrodiagnostic or
                                                                          pathological exam
     • 10 - 20% live > 10 yrs                                           – Evidence of UMN degeneration by clinical exam AND
• Diagnosis                                                             – Progressive spread of symptoms or signs within a region or to
                                                                          oher regions
  – Established by clinical criteria in association
    with electrodiagnostic testing.                                     The absence of:
                                                                        – Electrophysiological, pathological or neuroimaging evidence of
  – NO specific test-> remains clinical                                   other disease processes that might explain the findings
    diagnosis




                                                                                                                                           5
   Amyotrophic Lateral Sclerosis
                                                                       Case #3
              (ALS)
• Treatment:                                         • 33 y/o WM presents with 3 day h/o fever,
  – Riluzole- reduces the presynaptic release of       myalgias, nausea/vomiting and diarrhea.
    glutamate                                        • Ate at a picinic on day prior to illness
  – Meta-analysis of three large trials suggests       (hamburgers/hotdogs)
     • Prolonged survival of about 2 months
                                                     • Denies BRBPR or melena
     • Are we prolonging life at terminal stage or
       providing increased quality time?             • No abdominal pain or cramps
                                                     • Sore throat and rash




                    Case #3                                            Case #3
• Rash began approximately 2 days after              • No recent travel
  illness began                                      • Walks dog daily (in woods)
• Pruritic red spots first noticed on face
  followed by chest and back
• Generalized myalgias but no joint pain
• No SOB, cough, chest pain, DOE
• No dysuria or penile discharge




                    Case #3                                            Case #3
• PMH:                                               • PE:
  – None                                               – 102.4, 130/65, 76, 18
                                                       – HEENT: PERRLA, EOMI, no oral ulcerations no
• Meds:                                                  scleral icterus
  – None                                               – Neck: No nuchal rigidity, LAN or JVD
• Soc hx:                                              – CV: rrr w/o m/r/g
                                                       – Lungs: CTA bilaterally
  – homosexual
                                                       – ABD: S/NT/ND/BS present. No HSM
  – No tob or IVDA                                     – EXT: NO C/C/E
  – Occ ETOH                                           – Neuro: CN 2-12 intact, non-focal




                                                                                                       6
                        Case #3                                                                  Case #3




                    Chickenpox
• Caused by primary infection of varicella-zoster
  virus (VZV)
• Highly contagious
  – Secondary household attack rate of > 90% in
    susceptible hosts
  – Contact with Aerosolized droplets or direct cutaneous
    contact
• Incubation period 10-21 days
• Period of infectivity
     • 48 hrs prior to onset of rash until skin lesions have fully
       crusted


                                                                            Variola (smallpox)




                                                                                            Chickenpox
Chickenpox                          Smallpox                         • Complications:
• Begins on face/scalp              • Begins centrally, then            –Encephalitis
  and spreads to trunk                spreads outward to                –Pneumonia
                                      face and extremities              –Hepatitis
                                                                        –Rye Syndrome
                                                                          • N/V/HA/delirium-> coma (*precipitated by
• Lesions in various                • All lesions are always                salicylate use)
  stages of                           in single stage of             • Immunocompromised Hosts (impaired cellular immunity)
  development                         development                       – Very susceptible to disseminated disease
                                                                        – In adults with malignancy, mortality approaches 50%




                                                                                                                                7
                    Chickenpox
• Treatment
  – Acyclovir (initiated within 24 hrs of rash onset)
     • reduced the total time to full crusting of lesions from 7.4 days
       to 5.6 days
     • reduction in the maximum number of lesions
     • shorter duration of fever and lesser severity of symptoms

• Vaccination
  – Varicella vaccine (live attenuated VZV) is now
    recommended for all children
  – Waning of immunity
     • Cases of "breakthrough varicella" are now relatively common
     • Revaccination may be required




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posted:11/24/2011
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