All posts by JESS BEAMAN

5.18.16 – VA Ambulatory Report Pearls – Laugh Headache + Chiari Malformation

Laugh Headaches

  • The symptom of a very short-lived headache (lasting seconds) exacerbated or triggered by laughing and coughing has been identified in case reports in the literature as being associated with Chiari type I malformations.
  • Mechanisms
    • The mechanism behind short-lived headaches in Arnold-Chiari type I malformations is thought to be due to the downward displacement of the cerebellar tonsils –> stretching of pain-innervated structures (meninges, nerve roots, and vasculature)
    • Laughter/cough/valsalva –> increase in intrathoracic pressure -> increase in intrathecal pressure –> obstruction of CSF flow –> stretching of pain-inntervated structures (see above)
  • Can be associated with Chiari malformations of which there are various types. Chiari type I malformation is defined as a 5mm displacement of the cerebellar tonsils through the foramen magnum
  • Treatment
    • Varies depending on the type of Chiari malformation. Chiari type II and III are typically managed surgically. Type I malformations depend on symptoms and if syringomyelia is present.
    • Asymptomatic patients with Chiari type I malformation (and without syringomelia) are managed conservatively with clinical symptom and MRI surveillance.
    • Symptomatic patients with Chiari type I malformation (especially if impacting quality of life) and lower CN palsies, syringomyelia, myelopathy, cerebellar symptoms, or severe neck pain/occipital headache often go on to surgical decompression. As mentioned in report, symptoms are relieved in ~2/3 of patients whereas 1/3 of patients have stable (preoperative) symptoms and ~10% go on to need repeat surgeries.
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5.11.16 – VA Ambulatory Report Pearls – First time seizures: diagnosis/workup/management

First Time Seizures in Young Adults

Epidemiology: Many first seizures are unrecognized. The incidence of single seizures and recurrent epileptic seizures is estimated as 5-10% and the prevalence of epilepsy is 0.5-1% of the population. Epileptic seizures are responsible for 1% of hospital admissions and 3% of ED visits.

Recurrence and who may receive treatment after a first-time seizure:

  • Low risk patients with first seizures that have no neurological deficits, normal MRI, and EEG have a 35% risk of recurrence at five years. They are not usually offered treatment.
  • High risk patients with first seizures that have neurological deficits, MRI abnormalities, and/or EEG abnormalities have a 70% risk of recurrence at five years. They are typically offered treatment.

Per our neurology colleagues, the most common causes of a first time seizure in young adults are the following:

  • Idiopathic (can account for up to 50% of cases)
  • H/o trauma
    • Head injury accounts for a relatively small proportion of first time seizures. The risk to an individual who suffers head trauma can vary widely from minimal risk in people with a concussive head injury in which LOC or amnesia is < 30 minutes to a 12-fold increased risk over 10 years in people who suffer trauma-induced amnesia > 30 minutes, had a subdural hematoma at time of initial trauma, o a brain contusion.
  • H/o encephalitis
  • Viral encephalitis
  • Neurodevelopmental lesions* (e.g., cortical dysplasia in which there is a disruption in the proliferation of neuroblasts or abnormal migration of neurons to the developing cortex)
  • Genetic epilepsy syndromes*

The * indicates diagnoses that are less common after the age of 20 years old.

Attached: A nice review article in BMJ from 2014 on first time seizures in adults from the epidemiology –> differential –> workup and management.

5.4.16 – VA Ambulatory Report Pearls – Cardiac manifestations of apls

Common Cardiac Manifestations of APLS

  1. Valvular disease
  2. Coronary artery disease
  3. Intracardiac thrombus formation
  4. Pulmonary hypertension
  5. Dilated cardiomyopathy

More details on some of the most common cardiac manifestations listed above:

  • Valvular disease: deposition of immune complexes –> vegetations/irregular thickening of the valve leaflets –> valve dysfunction
    • Leads to increased risk for CVA
    • Note that 40% of APLS patients also have SLE and thus valvular disease can be secondary to SLE versus APLS
    • The most commonly affected valve is the mitral valve (MR) >> aortic and tricuspid valves which is thought to be due to the fact that left-sided valves are more vulnerable to micro injuries from stress/jet/turbulence
    • There is a positive correlation between APLS titers and severity of valvular heart disease
  • Coronary artery disease: patients with APLS are at risk of accelerated atherosclerosis
  • Intracardiac thrombus formation: rare complication
    • Thrombus is most likely to form on the right side
    • A quick word on other thrombogenic complications (e.g., recurrent DVTs) – of patients who present with thrombosis, up to 85% will present with venous > arterial thrombosis and repeat episodes are often of the same type
    • Thrombotic events, unlike intracardiac thrombus formation, are common and occur frequently in microvasculature or smaller vessels and are often recurrent and can impair myocardial function via recurrent microthromboses or microemboli

Reference (article attached here):

Koniari I, Siminelakis SN, Baikoussis NG, Papadopoulos G, Goudevenos J, Apostolakis E. Antiphospholipid syndrome; its implication in cardiovascular diseases: a review. Journal of Cardiothoracic Surgery. 2010;5:101. doi:10.1186/1749-8090-5-101.

4.20.16 – Dermatomyositis-Associated Malignancy

Dermatomyositis-Associated Malignancy

  • Dermatomyositis (DM) and polymyositis (PM) are both associated with malignancy with the association being stronger for DM >> PM.
  • Incidence of cancer for patients with DM is increased 5- to 7-fold compared to the general population.
  • Cancer can be diagnosed before, simulataneously with, or after the diagnosis of DM and PM though (as Rabih mentioned) the peak incidence of a cancer diagnosis in DM and PM occurs simultaneously with/during the first five years after diagnosis.
  • Types of malignancies = adenocarcinoma of the cervix, lung, ovaries, pancreas, bladder, and stomach accounts for 70% of cases.
  • With a new diagnosis of DM, the following labs are routinely performed:
    • CBC, LFTs, U/A
    • FIT versus colonoscopy for colon cancer screening
    • CXR
    • For women: pap, mammo, transvaginal U/S and CA-125 levels for ovarian cancer screening
      • Of note, there is no consensus on frequency of ovarian screening. Pap smears should be performed annually for patients with immunosuppression.

4.13.16 – VA Ambulatory report pearls – premature heart disease

  • Epidemiology: autopsy study of 760 young patients that investigated the presence of coronary atheromas in patients stratified by age
    • Advanced coronary atheroma in 2% of men and 0% of women aged 15-19yo
    • Advanced coronary atheroma in 20% of men and 8% of women aged 30-34yo; of those with advanced coronary atheroma 19% of men and 8% of women had a > 40% stenosis of the LAD
  • How does coronary disease differ in younger patients versus older patients?
    • One of the largest reports of angiographic findings in young patients is from a substudy of the Coronary Artery Surgery Study (CASS) which compared the results of angiography in 504 young men < 35yo and young women < 45yo as compared to 8300 older patients.
    • Younger patients were more likely to have the following:
      • Grossly normal coronary arteries on angiography (18% of younger patients versus 3% of older patients)
      • Single vessel disease (38% of younger patients vs 24% of older patients)
      • Predilection for LAD
  • Differential for ACS in younger patients:
    • Embolization (from PFO)
    • Substance use especially cocaine or other stimulants
    • Clotting disorders and hypercoagulable states (e.g., Factor V Leiden, APLS, nephrotic syndrome)
    • H/o Kawasaki disease as child (coronary aneurysms –> stenosis)
    • Spontaneous coronary dissection (more common in young women; increased risk during peripartum period)
    • H/o chest radiation (ostial disease most common)
    • OCP use + heavy smoking in young women
    • Psychosocial factors/exposure to chronic stressful situations
    • Hypoestrogenemia (ACS may be more common during follicular phase of the menstrual cycle when estrogen levels are lowest)
    • Myocardial bridging which is a congenital anomaly where the coronaries can be embedded into the myocardium
  • Risk factors for premature CAD
    • Smoking – most common and most modifiable
    • Family history
    • Lipid abnormalities
    • DM
    • HTN
    • Obesity
    • Paradoxical embolism
    • OCP use + heavy smoking

4.11 SFGH AM Report Pearls – GOLDMARK versus MUDPILES

In report, we touched on the famous mnemonics to remember the etiologies of anion gap metabolic acidosis. We discussed the use of a new mnemonic, GOLDMARK, to replace MUDPILES. Read on for more…

GOLDMARK vs MUDPILES

G = glycols (ethylene and propylene)

O = oxoproline (alias = pyroglutamic acid; elevated levels occur with chronic acetaminophen use)

L = L-lactate

D = D-lactate (present in patients with short bowel syndromes)

M = methanol

A = aspirin

R = renal failure –> uremia

K = ketoacidosis

Some might argue: “But, I love MUDPILES! Why the new mnemonic?!?” Here is the reasoning:

MUDPILES (methanol, uremia, DKA, paraldehyde, isoniazid/iron, lactate, ethylene glycol, salicylate) is often referenced when discussing the differential for anion gap metabolic acidosis. The argument for switching from MUDPILES to GOLDMARK was the following:

  • Paraldehyde use is very very rare
  • Iron and isoniazid are just two of many drugs that lead to lactic acidosis and thus would be accounted for in the “L” in GOLDMARK
  • Three new acids and acid precursors that can lead to an anion gap acidosis have been recognized as causing anion gap metabolic acidosis: 1) D-lactate, 2) 5-oxoproline, 3) propylene glycol

The attached Lancet article is where it is first introduced.

Will you be an adopter???

4.6.16 – VA ambulatory report – cervical spondylotic myelopathy

Cervical Spondylotic Myelopathy

  • Most common cause of spinal dysfunction in geriatric populations.
  • Myelopathy occurs as a result of both mechanical factors (e.g., degenerative disc disease, subluxation) and spinal cord ischemia. The spinal cord ischemia most likely occurs at the level of the impaired microcirculation and can occur from both reduced flow as well as compression of the larger vessels (e.g., anterior spinal artery).
  • Hallmark symptoms of cervical spondylotic myelopathy are gait abnormalities and weakness or stiffness of the legs. It is usually an insidious process. In the early stages, patients complain of subtle changes in gait or balance.
  • Physical exam findings are often suggestive of UMN dysfunction (hyperactive DTRs, ankle/patellar clonus, spasticity LE > UE, and Babinski’s sign). Lower extremity motor examination most frequently reveals weakness in the iliopsoas > quadriceps. The gait is usually a stiff or spastic gait.
  • See the differential diagnosis for cervical spondylotic myelopathy below taken from the article below.

DDx for Cervical Spondylotic MyelopathyBaron, Eli M., and William F. Young. “Cervical Spondylotic Myelopathy: A Brief Review of Its Pathophysiology, Clinical Course, and Diagnosis.” Neurosurgery 60.SUPPLEMENT (2007): S35-41. Attached Here