Dr Miller January Endocrine CPC migraine by mikeholy

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									Endocrine CPC

Tim Miller, MD
1/22/2008
Case

 CC: I have been having headaches

 HPI: Pt is a 33 year old Hispanic male
  seen for routine yearly follow up
 Reports headaches for 3 months
 Seen at walk-in clinic and diagnosed with
  migraines
 Midrin and Maxalt were not effective
Case

 Headaches start behind both eyes and
  encompass his entire head
 Recently (past 2 weeks) headaches
  occur on a daily basis and are more
  severe
 Headaches mainly occur at night
 Seem to be worse with exertion
Case

 Associated Signs/Symptoms
   Nausea
   Photophobia
   Phonophobia
   Roommate says he “looks pale” with attacks
   Sweats will accompany headaches, mainly
    at night
   Experiences “fluttering” in his chest and
    notes that his pulse is around 120
Case

 Pertinent Negatives
   No lacrimation
   No vision changes
   Location does not change
Case

 PMHx:
   Seasonal allergies
   Eczema
   Positive PPD – currently on INH therapy
   NAFLD with elevated LFT’s (discovered
    prior to initiation of INH)
   Hypertriglyceridemia
Case
 PSHx:           SocHx:
    None           Nonsmoker
                    Occasional EtOH
 FH:               No drugs
    Not given      Entomology graduate
                     student at Texas A&M
                    Born in Cuba and
 Meds:              moved to Texas to
    INH             study
                    Traveled to Peruvian
 All:               rainforest for research
    NKDA            within the last year
Case

 Review of Systems:
   General: no weight loss
   HEENT: no vision/hearing changes, no
    odynophagia/dysphagia
   Pulmonary: no SOB, no cough
   Cardiovascular: no chest pain, (+) palpitations
   GI: no jaundice, no abd pain, no changes in bowel
    habits
   GU: no changes in bladder habits
Case
 Physical Exam:
   BP 144/84 P 110 T 98.4 BMI 26
   General: well nourished, healthy appearing male
   HEENT: NCAT, PERRLA, EOMI, no icterus, mm
    moist, conjunctiva pink
   Fundoscopic: no papilledema
   Neck: supple, no LAD, no bruits, no supraclavicular
    fat pad
   CV: tachycardic (110) and regular, no murmurs,
    gallops, or rubs
Case
 Physical Exam cont:
   Resp: clear bilaterally, good resp effort
   Abd: mild TTP with deep palpation on the right,
    lateral to umbilicus, (+) BS, no HSM, no
    rebound/guarding
   Ext: no C/C/E
   Neuro: CN 2-12 intact, strength 5/5 x 4, no gait
    abnormalities
   Psych: normal mood and affect
   Skin: eczematous changes to palms and bilateral
    antecubital fossae
Case

 Labs & Studies:
     CBC normal
     CMP normal other than ALT 131
     Hepatitis Panel negative
     Ferritin normal
     Thyroid function tests normal
     2-view CXR normal
     CT head w/wo contrast negative
              Problem List

 ACUTE                            CHRONIC
 Headache x 3 months              Positive PPD
     Progressively worsening      INH Therapy
   Looks pale with attacks        Seasonal Allergies
   Sweats with headaches          Eczema
   Palpitations                   NAFLD Elevated LFT’s
   Hypertension                   Hypertriglyceridemia
   Tachycardia
   Travel to Peruvian
    Rainforest
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
    Cluster             Cushing’s Disease
    Tension             Pituitary Adenoma
  TB Meningitis
  CNS Neoplasm
  Hypertensive
   Headache
Headache - Epidemiology

  Among the most common medical
   complaints
  Overall prevalence 12-16%; accounts for
   1-2% of ER visits and up to 4% of outpatient
   office visits
  90% of headaches fall into categories of
   migraine, cluster, and tension
Headache - Classification

  Acute
    New onset or clearly different than previous
     headaches
    Often are a sign of serious illness
      Subarachnoid hemorrhage
      Meningitis
      Acute glaucoma
      Hypertensive encephalopathy
Headache - Classification

  Subacute
    Occurs over a period of weeks to months
    May or may not be related to underlying
     systemic illness
      Recent subdural hematoma
      Subacute meningitis
      Primary or metastatic tumor
      Giant cell arteritis
      Optic Neuritis
Headache - Classification

  Chronic
    Continues
     intermittently for years
    Usually has a benign
     cause
         Migraine
         Cluster
         Tension
         Sinusitis
         Dental Disease
Headache – Approach to
Diagnosis
  Age of onset
  Timing of pain
  Precipitating factors
  Characteristics of pain
  Location of pain
  Associated symptoms
  History of prior
   headaches and course
   of this type of headache
  Physical Exam
Migraine Headache

  Unilateral and usually
   pulsatile
  Often associated with
   nausea, photo- and
   phonophobia
  Moderate to severe intensity
  55% occur initially prior to
   age 20
  Usually familial
  Auras occur in 10% of
   patients
  Most often occur in women
Migraine Headache

  Clinical Presentation
    Classic Migraine (preceded by aura)
       Visual, noxious, or sensory alterations followed
        by intense unilateral throbbing headache Usually
        do not occur more frequently than weekly
       Last between 4 and 72 hours
    Common Migraine (lacks aura)
       Usually unilateral and throbbing
Cluster Headache

  Clinical Presentation
    Brief, severe, constant, nonthrobbing
     headache
         Lasts 15-180 minutes without treatment
      Always unilateral
      Much more common in men
      Mean age of onset is 25
      Episodes are separated by months or even
       years
Cluster Headache
  Begins with
   sensation by the
   nose or behind the
   eye
  Ipsilateral
   lacrimation,
   conjuntival injection,
   or ptosis may occur
  Key to diagnosis is
   brevity of symptoms
Tension Headache

  Clinical Presentation
    Lacks features of migraine or cluster
    Frequent (possibly daily), bilateral occipital,
     nonthrobbing
    Often a band distribution
    Rarely nausea, vomiting, or visual prodrome,
     although may occasionally have features of
     migraine
Tension Headache?

             Women more
              common then men
             Secondary gain?
Headache - Imaging
  Danger signs that indicate need for imaging
    Sudden onset of worst headache ever or headache
     that reaches full intensity in minutes
    Absence of similar terrible headaches in past
    Worsening pattern
    Focal neurologic signs or papilledema
    Fever
    Change in mental status or personality
    Rapid onset with strenuous exercise
    Presentation to any ER with headache
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
    Cluster             Cushing’s Disease
    Tension             Pituitary Adenoma
  TB Meningitis
  CNS Neoplasm
  Hypertensive
   Headache
CNS Tuberculosis
  3 Categories
      TB Meningitis
      Intracranial Tuberculoma
      Spinal tuberculous arachnoiditis
  Account for 1% of all TB cases
  Quite rare in United States
  Fatal in 15-40% of cases, usually within 5-8 weeks
  Active TB outside the CNS is not necessary to make
   diagnosis
  Send CSF for acid fast smear, TB culture, and TB PCR
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
                         Cushing’s Disease
                         Pituitary Adenoma

  TB Meningitis
  CNS Neoplasm
  Hypertensive
   Headache
CNS Neoplasm

    Headache is a common symptom in
     patients with brain malignancies
          Worst symptom in half of patients
    Usually described as dull and constant,
     occasionally throbbing
    Typically tension-type (77%); can be
     migraine-type (9%), or other (14%)*
    Severe headaches are infrequent

*Forsyth, PA, Posner, JB. Headaches in patients with brain tumors. Neurology 1993; 43:1678.
CNS Neoplasm
  Other clues to malignant headaches
     40% have nausea and vomiting
     Change in prior headache pattern
     Abnormal neurologic exam
     May worsen with maneuvers that increase the ICP (bending
      over, sneezing, coughing)
     Tend to be worse at night (vasodilatation from transient
      increase in PCO2)
     Typical location is bifrontal but may be generalized if ICP is
      increased
         Headache, nausea, papilledema ( ICP)
     Other signs of CNS malignancy include seizures, syncope,
      cognitive dysfunction, focal neurologic deficits
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
                         Cushing’s Disease
                         Pituitary Adenoma


  CNS Neoplasm
  Hypertensive
   Headache
Hypertensive Headache

   Routine essential hypertension has not been
    shown to directly cause headaches*
   Can lead to conditions that cause headaches
         Intracerebral or subarachnoid bleeding
                Sudden onset of severe headaches
   Hypertensive encephalopathy
         Insidious onset of headache, N/V, followed by
          restlessness and confusion, and eventually seizure
          and coma if not treated

*Strovmer LJ, Vatten L, et al. Blood pressure and risk of headache. J Neurol Neurosurg Psychiatry 2002;72:463–6.
Hypertensive Headache
     Law, et al studied whether blood pressure meds
      lowered headaches in a meta-analysis
           94 randomized placebo-controlled trials using 4 major types of
            BP meds (thiazides, b-blockers, ACE-I, and ARB)
           Separated into treatment and placebo groups
           Average SBP fell by 9.4 and DBP fell by 5.5 in treatment
            groups
           8% reported headaches in treatment groups and 12.4%
            reported headaches in placebo groups
                  RRR 33% (P<.001)
     However, many observational studies do not support
      hypertension as a cause of headaches

Law M, Morris JK, Jordan R, Wald N. Headaches and the treatment of blood pressure. Circulation. 2005; 112: 2301–
2306.
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
                         Cushing’s Disease
                         Pituitary Adenoma




  Hypertensive
   Headache
Cushing’s Disease

  Chronic excess of glucocorticoid
  Clinical features:
    Progressive obesity including moon facies,
     supraclavicular fat pad and buffalo hump
    Skin changes of easy bruisability and striae
    Osteoporosis
    Proximal muscle weakness
    Hypertension
    Headache in up to 47% of patients
Pituitary Adenoma
  Microadenoma < 1 cm
  Macroadenoma > 1 cm
  Arise from anterior pituitary
  Secrete hormones depending on type of tumor
   (Prolactin, ACTH, GH)
  Clinical Presentation
      Visual defects are most common presenting symptom of
       nonfunctioning adenomas
         Bitemporal hemianopsia
      Headaches are common and nonspecific
      Pituitary apoplexy causes severe headache and diplopia and
       cranial nerve deficits
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
                         Cushing’s Disease
                         Pituitary Adenoma
Pheochromocytoma -
General
  Catecholamine secreting tumors of chromaffin
   cells arising from the adrenal medulla
    Epinephrine and norepinephinre; rarely dopamine
    Stimulate alpha and beta adrenergic receptors
  Approximately 0.2% of patients diagnosed with
   hypertension will have a pheochromocytoma
  Equal incidence in men and women
  Peak incidence between 3rd and 5th decade
Catecholamines

  Epinephrine, Norepineprhine, and
   Dopamine secreted from the adrenal
   medulla
  Act on α1 (peripheral vasoconstriction)
   and α 2 receptors and β1 (heart rate and
   inotropy), β2 (muscle and liver
   vasodilatation)
Effects of Catecholamines
  Increase force of cardiac
   contractility
  Increases heart rate (epi >
   norepi)
  Peripheral vasoconstriction
  Dilates bronchioles
  Glycogynolysis (mobilizing
   glucose)
  Increased metabolic rate
  Dilates pupils
  Inhibits non-essential
   processes such as GI motility
                     Norepinephrine and epinephrine levels in human venous
                       blood in various physiologic and pathologic states




Cryer PE: Physiology and pathophysiology of the human sympathoadrenal neuroendocrine system. N Engl J Med 1980;303:436.
Pheochromocytoma -
Clinical Presentation
  Clinical Triad
     Episodic Headaches
     Sweating
     Tachycardia
      (or palpitations)



  Frequency ranges from multiple times per day to
   monthly
  Worsens with time and becomes more frequent
  Half will have paroxysmal hypertension and a third
   appear to have essential hypertension
     5-15% present with normal blood pressure
Pheochromocytoma –
Clinical Presentation
  Headaches occur in 90% of symptomatic
   patients
    May be mild or severe and are variable in duration
  Sweating occurs in 60-70% of patients
  Other signs and symptoms
    Dyspnea
    Generalized Weakness
    Panic Attack
Pheochromocytoma –
Presentation
  May often be unmasked during routine
   procedure or drug administration
    Anesthesia induction
    Cold Medications
    Opiates
    Dopamine Antagonists
    Cocaine or TCA’s which inhibit catecholamine
     reuptake
    Childbirth
    Trauma
Pheochromocytoma –
Rule of 10’s
    10% Bilateral
    10% Malignant
    10% Extra-adrenal
    10% Calcify
    10% Familial
    10% Children
Pheochromocytoma –
Familial Conditions
  MEN 2a                  Von Hippel-Lindau
    Pheochromocytoma        Cerebellar
    Medullary Thyroid        Hemangioblastomas
     Cancers                 Retinal Angiomas
    Parathyroid Tumors      Renal Cell Carcinoma
  MEN 2b                    Pheochromocytoma
    Pheochromocytoma      Neurofibromatosis
    Medullary Thyroid       Café-au-lait spots
     Cancers                 Neurofibromas
    Neuromas                Pheochromocytoma
Pheochromocytoma –
Evaluation
  Which patients should be evaluated for
   pheochromocytoma?
    Patients with difficult to control hypertension
    Patients receiving more than 4 BP meds
    Patients with onset of htn before age 35
    Patients with onset of htn after age 60
    Patients with signs or symptoms of
     pheochromocytoma as mentioned before
    Patients with familial history of predisposing
     disorders
    Severe pressor response during anesthesia
Pheochromocytoma –
Lab Diagnosis
  Free plasma metanephrine level
    Screening Test for at risk patients
    96% sensitive but 85% specific (better if
     drawn during an attack)
    Standard venipuncture test
Pheochromocytoma –
Lab Diagnosis
  Urinary catecholamines and metanephrines
    Confirmatory test
    87% sensitive and up to 99% specific
    Must be collected in a 24-hour urine specimen
       Order metanephrines (best test), catecholamines,
        and vanillylmandelic acid (worst test)
       Ensure creatinine is measured to ensure adequacy
       Collect during or immediately after an attack if
        possible
       2-3x increase in levels is diagnostic
Pheochromocytoma –
Lab Diagnosis
  Other diagnostic tests
    Clonidine Suppression Test
       Confirmatory test; 90% accurate
       Clonidine normally suppresses release of catecholamines
        centrally, but not from a pheo
       Administer Clonidine and measure plasma metanephrines
        before, and 3 hours after
          Levels will decrease in essential hypertension
          Levels remain increased in pheochromocytoma
    Chromogranin A level
       Increased in 80% of patients with pheochromocytoma
       Not specific for pheochromocytoma
Pheochromocytoma –
Imaging
  Imaging should only be
   obtained after biochemical
   diagnosis has been confirmed
  MRI has reported sensitivity
   of 100% for adrenal pheos
  Better than CT for picking up
   extra-adrenal tumors as well
  MRI can distinguish between
   incidentilomas on T2-
   weighted images
  CT may miss tumors smaller
   than 1 cm
Pheochromocytoma –
Imaging
             MIBG Nuclear Medicine
              Scan is reserved for
              biochemically proven
              cases with negative MRI
              or CT
             MIBG structure
              resembles norepi and
              concentrates in adrenal
              or extra-adrenal pheos
             Used in familial
              syndromes, recurrent
              pheo, or malignant pheo
Malignant
Pheochromocytoma
  10 % of pheochromocytomas are
   malignant
    Direct invasion into surrounding tissue
    Distant metastasis
    Clinical, biochemical, or histological features
     cannot predict malignancy
    Common metastatic sites include bone, liver,
     and lymph nodes
Extra-adrenal
Pheochromocytoma
  90% of pheochromocytomas
   are located in the adrenal
   glands
     98% located in the abdomen
  Extra-adrenal
   pheochromocytomas arise in
   paraganglion chromaffin
   tissue of the sympathetic CNS
  Anywhere from the base of
   the brain to the bladder
Pheochromocytoma –
After Diagnosis
  Rule out familial pheochromocytoma
   syndromes
    PTH level and Calcium level (MEN 2a)
    Ophtho consult to rule out retinal angiomas
     and MRI head to rule out cerebellar
     hemangioblastomas (VHL)
    CT pancreas and kidneys
    Consider genetic testing for family
Pheochromocytoma –
Treatment
  Block alpha receptors first with pure alpha
   blocker Phenoxybenzamine
  After alpha blockade is achieved, begin
   nonselective beta-blockers
  Administer last doses the morning of surgery
  Hydrate well and expand volume with isotonic
   saline
  Surgically resect tumor; give stress-dose
   steroids if bilateral adrenalectomy is planned
Pheochromocytoma –
Long term follow up
  Test plasma free metanephrines 2 weeks
   post-operatively, then every 5 years
   thereafter
  Ensure resolution of hypertension and
   associated complications
              Problem List

 ACUTE                            CHRONIC
 Headache x 3 months              Positive PPD
     Progressively worsening      INH Therapy
   Looks pale with attacks        Seasonal Allergies
   Sweats with headaches          Eczema
   Palpitations                   NAFLD Elevated LFT’s
   Hypertension                   Hypertriglyceridemia
   Tachycardia
   Travel to Peruvian
    Rainforest
Differential Diagnosis

  Chronic Headache    Endocrine Headache
    Migraine            Pheochromocytoma
 Final Diagnosis:
  Pheochromocytoma


 Test of Choice:
  24-hour urine
   catecholamines,
   metanephrines, and VMA
References
1.   Forsyth, PA, Posner, JB. Headaches in patients with brain tumors: a study of
     111 patients. Neurology 1993; 43:1678.
2.   Cryer PE: Physiology and pathophysiology of the human sympathoadrenal
     neuroendocrine system. N Engl J Med 1980;303:436.
3.   Headache classification committee of international headache society.
     Classification and diagnostic criteria for headache disorders, cranial neuralgias,
     and facial pain. Cephalalgia 1988; 9 Suppl 7:1.
4.   Headache Classification Committee of the International Headache Society. The
     International Classification of Headache Disorders. Cephalalgia 2004; 24:1.
5.   Strovmer LJ, Vatten L, et al. Blood pressure and risk of headache: a prospective
     study of 22 685 adults in Norway. J Neurol Neurosurg Psychiatry 2002;72:463–
     6.
6.   Law M, Morris JK, Jordan R, Wald N. Headaches and the treatment of blood
     pressure: results from a meta-analysis of 94 randomized placebo-controlled
     trials with 24 000 participants. Circulation. 2005; 112: 2301–2306.
7.   www.uptodate.com

								
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