Carotid artery webbing

case report at AAN 2010 also known as "atypical FMD" a 41 year old woman with headache  for months, facial droop and aphasia cutely.  MRI showed an M1 occlusion, and delayed time to peak in entire MCA.  Catheter showed bilateral ICA webbing with stagnant flow.  No standard of care is known.

MCA arrow sign in MCA aneursmal SAH

In a review, arrow sign was present in 4 patients with SAH all Fisher 3.  That is 16 % of total MCA aneurysms and was not seen in any other type of aneurysm.

below is an arrow sign for an MCA trifurcation aneurysm (from neurology.org)

Ptosis and astasia with thalamic infarcts: case report (s)

Jain D. et al.  Cerebral ptosis and astasia  "Lateral pulsion" due to a left anterior thalamic lesion.  Mechanisms are reviewed.AAN 2010:PO2:102

Alderazi Y.  Thalamic infarction causing astasia-abasia, ataxia and asterixis.  clinical and radiological features of two cases.  PO2:108.    Wide based gait past pointing and intention tremor on right, with left posterolateral thalamic infarct.    Second case with left arm drift, left asterixis, inability to stand unassisted with right lateral thalamic acute stroke and old left cerebellar hemorrhage. 

Sneddon's syndrome need for angiography

Faris et al.  Sneddon's syndrome without antiphospholipid antibodies:  a report of 26 cases with cerebral angiography (Rabat).  Neurology 2010 74:9:PO2:099.

Authors emphasize Sneddon's syndrome (stroke  plus livedo racemosa) is NOT identical to APL syndrome.  26 patients were studied retrospectively with a combination of focal motor deficits and dementia.  Imaging always showed infarcts with white matter involvement.  Angio showed a distal arteriopathy in 18 cases with pial networks in  cases.  Two had hematomas.  The authors suggested angiography to prevent the unwarranted and dangerous potential use of anticoagulation.

NSE after cardiac arrest during hypothermia predicts outcome

AN 2010 Po1.048  JEF Fugate, Wijdicks et al.  NSE was measured serially in comatose patients undergoing hypothermia.  A cutoff was used of 33 ug/L.  NSE was measured at day one and day three,  with higher NSE suggesting poor prognosis (defined as one year mortality).  48 patients, 41 had first day NSE, 14 had third day NSE.  For first day NSE, the number with high level is given with number of survivors at one year in parenthesis 19 (3) with a low NSE being 22 (11).  Trend in NSE on day 3 was highly predictive (p<.015).  The sensitivity for first day NSE was 59 % for one year mortality with improvement to 66 % if 3 day NSE is included.

Four score is predictor of outcome in coma after cardiac arrest

Neurology AAN 2010 PO1.045 Wijdicks et al.

The Four Score differs from the GCS because it has 4 components-- eye, motor, brainstem and respiration (latter two are not included in GCS) .  Prospectively looked at patients from 2006-2009 (n=131) and looked at outcome after one year.  91 died.  31 had four score less than 4 at day one and of these, zero survived.  Of patients with GCS of 3, 4 (7 %) survived at one year.  The Four Score had a specificity for absent survival at one year of 100 % versus 90 % for GCS of 3. 

Description of the FOUR Score


The FOUR score has 4 components: eye responses, motor responses, brainstem reflexes, and respiration pattern. Each component has a maximal value of 4 (Figure 1). Assessing all components of this score usually takes only a few minutes.5 The eye response component of the FOUR score allows differentiation between a vegetative state (eyes open but do not track) and a locked-in syndrome (eyes open, blink, and track vertically on command). The motor assessment component of the FOUR score combines the withdrawal reflex and decorticate rigidity responses because these conditions are often difficult to distinguish clinically. The motor component includes a complex command (the patient is asked to produce a thumbs-up hand signal, a fist, and the peace sign) that determines whether patients are alert.7 Similarly, the motor component of the FOUR score can detect signs of severe cerebral dysfunction, such as myoclonic status epilepticus. Such dysfunction is often a poor prognostic sign for patients with suspected anoxic brain injury.8 The brainstem components of the FOUR score assess the pons, the mesencephalon, and the medulla oblongata in various combinations. The FOUR score also includes an assessment of Cheyne-Stokes respiration and irregular breathing; such signs can indicate bihemispheric or lower brainstem dysfunction of respiratory control. For patients who have undergone intubation, the FOUR score records the presence or absence of a respiratory drive.

FIGURE 1.


Description of Full Outline of UnResponsivenes (FOUR) score. Eye response: E4 = eyelids open or opened, tracking, or blinking to command; E3 = eyelids open but not tracking; E2 = eyelids closed but open to loud voice; E1 = eyelids closed but open to pain; E0 = eyelids remain closed with pain. Motor response: M4 = thumbs-up, fist, or peace sign; M3 = localizing to pain; M2 = flexion response to pain; M1 = extension response to pain; M0 = no response to pain or generalized myoclonus status. Brainstem reflexes: B4 = pupil and corneal reflexes present; B3 = one pupil wide and fixed; B2 = pupil or corneal reflexes absent; B1 = pupil and corneal reflexes absent; B0 = absent pupil, corneal, and cough reflex. Respiration pattern: R4 = not intubated, regular breathing pattern; R3 = not intubated, Cheyne-Stokes breathing pattern; R2 = not intubated, irregular breathing; R1 = breathes above ventilatory rate; R0 = breathes at ventilator rate or apnea.

The Broken Heart Syndrome

llan Wittstein has published and given lectures and states there are clinical criteria, diagnostic criteria and treatment and prognosis information that can be readily identified. Synonyms include neurogenic stunned myocardium, acute coronary syndrome, stress myocarditis, and Takotsubo syndrome (named after the japanese pot used to capture octopus).  The syndrome is a REVERSIBLE disorder with very abnormal EKG, U waves, ST elevations, Q waves, elevated troponins, normal coronaries on cath, and return of EF to normal within days to weeks.  The pathology includes contraction band necrosis in myocardium, and is linked to hypersympathetic state.  It is a huge problem involving 2 percent of patients undergoing cath and 5-7 % of women.  Most patients are postmenopausal Caucasian women with risk factors who present with chest pain and shortness of breath.  Many have mood disorders


Diagnostic criteria are divided into "helpful" and "required"
HELPFUL-
1.  Acute trigger-- could be emotional (anxiety, joy, grief, fear, anger) or physical (procedure, respiratory drugs) even surprise party. 
2.  Characteristic EKG-- presenting EKG has steep ST elevation without reciprocal changes, T wave inversions everywhere, QT prolongation,  that becomes milder within 2-4 days
3.  Troponin elevation is mild-- less than 5, never more than 20

REQUIRED
1.  Absent coronary thrombosis
2.  Wall motion abnormalities extend beyond a single coronary artery territory  ( 3 patterns:  apical, basal, and midventricular)
3.  Rapid recovery of systolic function within 2 weeks at most

Diagnostic tests that are helpful (but possibly hard/unlikely to obtain esp. acutely)
1.  MRI heart unlike ECHO differentiates dead and stunned tissue.  Dead cardiac tissue lights up with Gadolinium but stunned heart will not

Therapy:
1.  supportive-- possibly not in ICU- arbs, ACEi's, diuretics.  Anticoag if apex not moving to prevent clot kicking, avoid pressors (catechols are a problem)
2.  Balloon pump better than pressors
3.  HHH  good for brain, bad for heart

Prognosis
1.  recurrence 3-10 percent with 2 % mortality
2.  Death is due to etiology not to cardiac dysfunction per se.

Pathophysiology
contraction band necrosis- direct myocyte injury related to calcium overload.

air embolism and air travel

The Neurologist 2010   A  62 year old woman with cerebral artery air embolism during commercial air travel.  16: 136-137.

 

Notes

usual list of associations with air embolus-- surgery, scuba diving, induced abortion, angiography and pneumothorax, orogenital sex on a woman with cerebral air emboli (see Crit Care Med 1988).

A new one is air travel.  In this case, pulmonary bullae due to emphysema occurred as the bullae expanded as the pressure in the cabin was reduced, leading to rupture of the bullae, pneumothorax and air embolism.  Barotrauma was presumably the proximate cause.

Pearls on pediatric strokes

hat tip to Lori Jordan MD JHU


1.  Strokes in kids are as common as brain tumors, about 2-3/100,000
2.  In children ICH = bland infarcts, different than adults (Fullerton, Neurology, 2003).  ICH is often due to AVM's
3.  Among bland infarcts, 25-35 % are cardioembolic, 25 % are dissections, other unusual causes include moya moya, sickle cell disease, HIV and varicella (not in order).
4.  Subarachnoid hemorrhage in kids is usually aneurysmal
5.  Kids at risk often have an inciting event such as trauma or surgery
6.  Kids have a high risk of delay in diagnosis
7.  Sicklers have 10 % stroke, but 20 % more of silent stroke; with SCA and CVA stat consult Hematology for transfusion
8.  Many barriers to alteplase use exist, including diagnosis, , lack of evidence and mimics, and delays, but document why alteplase is not given
9.  MERCI and multi MERCI are not studied in kids
10.AHA guidelines for pediatric stroke published Stroke 2008
11.  Presentation in children is much more likely to include seizure (25 % v. 5 % in adults)
12 . Suggested eval: MRI, MRA H/N, hypercoagulability workup complete, TTE/bubble, HB electropheresis, HIV,
13 references
Roach ES, Golomb MR, Adams R, Biller J, Daniels S, Deveber G, et al.
Management of stroke in infants and children: A scientific statement from a
special writing group of the american heart association stroke council and the
council on cardiovascular disease in the young. Stroke 2008;39:2644-91.
• Amlie-Lefond, C. et al. Use of alteplase in childhood arterial ischaemic stroke: a
multicentre, observational, cohort study. 2009: Lancet Neurol. 8, 530-536.
• Jordan LC, Johnston SC, Wu YW, Sidney SS, Fullerton HJ. The importance of
cerebral aneurysms in childhood hemorrhagic stroke: a population-based study.
Stroke 2009;40:400-405.
• Beslow LA, Licht DJ, Smith SE, Storm PB, Heuer GG, Zimmerman RA, Feiler
AM, Kasner SE, Ichord RN, Jordan LC. Predictors of outcome in childhood
intracerebral hemorrhage: a prospective consecutive cohort study. Stroke 2009;

CAA with vasculitis and edema responsive to steroids references

inflammation related CAA as described in : Ann Neurol 2004; 55: 250-256; Brain 2005; 128: 500-515; Neurology 2007; 68 (17); 1411-1416.

ISC Abstract highlights 2010 San Antonio (pruned and edited)

1.  Restrepo et al. (UCLA) Stroke pretreatment screening for fast Mag trial, involved a 90 second screen with a neurologist, focused, 72 % of patients so diagnosed had acute ischemic stroke, 24 % ICH, rest other

2.  Albright et al. (Penn) studied the potential for the use of air ambulances to increase availability of services and found The combination of pre-hospital regionalization & air ambulance transport of acute stroke
patients would reduce the 135.7 million Americans without 60 minute access to a PSC by
half, to 62.9 million.

3.  Kleindorfer et al. (Cincinatti) stratified t-PA eligibility by age and found contrary to hypothesis, the eligibility for rt-PA significantly increased with increasing age.
Age-Based Eligibility for and Treatment with Rt-PA
Age of Pt           #  Patients           % Eligible for rt-PA  % of Eligible Treated
18–44                   97                        4 (4.1%)              2 (50.0%)
45–54                 219                       15 (6.8%)             5 (33.3%)
55–64                 320                       21 (6.6%)            12 (57.1%)
65–74                 392                       32 (8.2%)            20 (62.5%)
75–84                 502                       47 (9.4%)             23 (48.9%)
85                       300                       29 (9.7%)             10 (34.5%)
Total                  1830                     148 (8.1%)             72 (48.6%)

4.  Riccio et al (Buenos Aires) Occult v. non -occult AF compared in TIA and AIS. Age, female gender and left atrial area (LAA) are traditional determinants of AF.  Out of 194 patients, there were 36 with known AF and 24 with occult AF.  Patients with occult AF were younger, showed a higher proportion of males, had
a smaller LAA, and had more severe strokes. Traditional determinants of AF were associated
with known AF.Diabetes was associated with occult AF.

5.   Gupta et al. (multicenter) General anesthesia during stroke resulted in worse outcomes.