Stark and related laws resources

What Neurologists need to know about the Stark Law,

http://www.aan.com/globals/axon/assets/6532.pdf

Steroids Help Unfreeze Bell's Palsy

 Early treatment with the corticosteroid prednisolone appeared to significantly reduce mild to moderate sequelae in Bell's palsy as judged by two scoring systems, according to results from a large Scandinavian trial.
As measured by the Sunnybrook scoring system, among more than 800 patients randomized to 1 of 4 treatment groups, those who received prednisolone had a significant reduction in mild to moderate impaired facial function at 12 months (P<0.001) compared with those who did not receive the steroid, Thomas Berg, MD, PhD, of Oslo University Hospital Rikshospitalet in Norway, and colleagues reported.
The difference between patients who received prednisolone and those did not in two House-Brackmann gradings levels was also significant (P<0.001 and P=0.01, respectively), Berg and co-authors wrote in the May issue of the Archives of Otolaryngology – Head & Neck Surgery.
Two of the treatment groups also received the antiviral valacyclovir (Valtrex), but no significant differences were found in those groups, they added.
The cause of Bell's palsy, which damages the facial cranial nerve and affects up to 40,000 Americans, is unknown.
One theory is that reactivation of a latent herpes simplex virus may cause inflammation and injury to the facial nerve, Berg and his co-authors noted, adding that treatment has been based on this theory.
About 70% of Bell's palsy patients recover completely within 6 months without any treatment, the authors noted. The remaining 30% have varying degrees of sequelae with functional, psychosocial, and aesthetic disturbances.
And despite some data that prednisolone improved complete recovery rates, large controlled studies on the effect of corticosteroids (and any additive effect of antivirals) were lacking.
To help correct this information deficit, the researchers recruited 829 patients (341 women and 488 men) over a 5-year period. They ranged in age from 18 to 75 and were enrolled at 17 public referral centers involved in the Swedish and Finnish Scandinavian Bell's Palsy Study, a prospective, randomized, double-blind, placebo-controlled, multicenter trial.
The patients were randomized within 72 hours in a factorial fashion to placebo plus placebo (n=206); prednisolone, 60 mg/d for 5 days, with the dosage then tapered for 5 days, plus placebo (n=210); valacyclovir hydrochloride, 1,000 mg 3 times daily for 7 days, plus placebo (n=207); or prednisolone plus valacyclovir (n=206).
The researchers then evaluated facial functioning at 12 months, using two separate grading systems -- Sunnybrook and House-Brackmann.
The Sunnybrook system, considered the more sensitive of the two, evaluates resting symmetry, degree of voluntary movement, and synkinesis to form a composite score, for which 0 indicates complete paralysis and 100, normal function.
The House-Brackmann system consists of a 6-grade scale (I to VI), in which I indicates normal function and VI, complete paralysis.
Follow-up visits were between days 11 to 17 and at 1, 2, 3, 6, and 12 months after randomization. If the recovery was complete (defined as a Sunnybrook score of 100) at 2 or 3 months, the next follow-up was at 12 months. Patients were grouped according to severity of sequelae by both scoring systems at 12 months.
In 184 of the 829 patients, the Sunnybrook score was less than 90 at 12 months; 71 had been treated with prednisolone and 113 had not (P<0.001).
In 98 patients, the Sunnybrook score was less than 70; 33 had received prednisolone and 65 had not (P<0.001), Berg and colleagues wrote.
The difference between patients who received prednisolone and who did not in House-Brackmann gradings higher than I and higher than II was also significant (P<0.001 and P=0.01, respectively).
No significant difference was found between patients who received prednisolone and those who did not in Sunnybrook scores less than 50 (P=0.10) or House-Brackmann grades higher than III (P=0.80).
Synkinesis was assessed with the Sunnybrook score in 743 patients. Among those, 96 patients had a synkinesis score more than 2, of whom 33 had received prednisolone and 63 had not (P=0.001). There were 60 patients who had a synkinesis score more than 4, of whom 22 had received prednisolone and 38 had not (P=.005).
The authors cited several limitations to their study.
"Subgroup analyses led to a reduction of patients in the analysis groups, which makes statistical comparisons more hazardous" they wrote. Nor did they "make the distinction between incomplete and complete palsy at baseline," but analyzed the median baseline scoring levels, which were found to be similar in the different treatment groups.
The investigators concluded that while "treatment with prednisolone significantly reduced mild and moderate sequelae in Bell's palsy at 12 months, prednisolone did not reduce the number of patients with severe sequelae," and valacyclovir had no effect.

Low pressure headaches - pearls

1.  aka SIH, or spontaneous intracranial hypotension/ spontaenous CSF leak

2.  Diagnostic criteria (journal Headache, 2011) :  a. orthostatic headache  b.  no recent dural puncture  c. not attributable to another disorder  and d.  at least one of the following: (1) low OP < 60 mm H20  (2) sustained improvement after epidural blood patches   (3) demonstrated active leak  (4) cranial MRI showing brain sagging or pachymeningeal enhancement

3.  Clinical pearl:  MRI without contrast can lead to false diagnosis of Chiari malformation and repair of which will not help.

4.  Headaches can be frontal/occipital/holocephalic/ cervical interscapular/ nonorthostatic or lingering nonorthostatic before or after orthostatic/ thunderclap headache/ cough headache/ second half of the day headache/ acephalgic forms

5. May require multiple blood patches to fix

History and physical exam pearls of TA biopsy

Headache pearls
1.  Headache can be ANY phenotype, mimic cluster, icepick HA, stabbing headache or other
2. may be dull and boring with lancinating pain, and associated scalp tenderness
3.  Worse with cool temperature
4. May be intermittent
5.  High dose steroids, TA biopsy and vision loss may all improve headache pain

TA exam pearls
1.  Beading
2.  Prominence
3.  Tenderness
4. Pulselessness
5. Erythema over artery

Clinical associated findings/pearls
1.  Fever
2.  Asthenia
3. Arthralgias/synovitis
4. myalgias
5.  weight loss
6.  cough
7.  mental status changes-delusions, depression, memory impairments, dementia

Ischemic complications
1.  jaw claudication
2.  Scalp necrosis
3.   tongue necrosis
4.  sore throat
5.  hoarseness
6.  stroke or TIA
7.  Angina or MI
8.  Upper limb claudication or pain

temporal arteritis pearls

CTA or MRA for evidence of large vessel involvement

PET for evidence of aortic inflammation

ESR is <50 in 10 % and< 40 in 5.8 %, CRP more sensitive

Other labs- anemia, increased Platelets,fibrinogen and alk phos occur

some use aspirin

65 % have visual obscurations before permanent visual loss, on average 8.5 days before

Contralateral eye may be affected, average, 5 days after first eye has visual loss.

lack of relief with prednisone is a sign of wrong diagnosis


1990 criteria require 3/5: 
new onset headache or new type of headache
age > 50
TA tender orpulseless, unrelated to atherosclerosis
WSR> 50
Abnormal biopsy with inflammation and multinucleate giant cells

Temporal arteritis biopsy formula

Younge BR et al.   Mayo Clinic Proceedings 2004 79; 483-91

Formula for positive TA biopsy
Value= (-240) + 48 (headache) + 108 (jaw claudication)+ 56 (scalp tenderness) + 1(ESR in mm/hr) + 70 (ischemic optic neuropathy) + 1 (age in years)

if symptom is not present, give zero for that element
<-110  low risk
-110 to 70  intermediate risk
>70   high risk

Pearls about hypothermia

Blondin and Greer.  The Neurologist 2011; 17:241-248.
1. Beware of accumulation of midazolam, propofol, and fentanyl during hypothermia

2. Traditional statement about prognosis with no motor signs (pupil, corneal, posturing) does not apply to hypothermia, as some survivors with good prognosis who were treated with hypothermia had no responses till day 6. The presence of a motor response at day 3 suggests a good outcome.Absent motor response does not predict a poor outcome.

3. Absent brainsten reflexes at day 3 is predictive of poor response but is not absolute.

4.  SEP responses disappear below 30 degrees centigrade. At 32-34 degrees, they should be prolonged but present

5.  EEG:  small studies; alpha coma, and burst suppression patients did not regain consciousness, and refractory status epilepticus patients did not regain consciousness. All patients with initially continuous EEG did regain consciousness, and among those with flat eeg's only those that evolved to a continuous pattern regained consciousness.

6.  Myoclonus is not predictive uniformly of a bad prognosis.It may be due to weaning neuromuscular blockade, to seizures, or metabolic status.

7.  Neuron specific enolase (NSE) is important in coma, but there is no reliable cutoff number among patients treated with hypothermia.  Otherwise, rising NSE between 24-48 hours is important.

8.  MRI ADC abnormalities >  10 % of brain volume 2 or more days out invariably had a bad prognosis.

Clinical findings that do not negate Brain death

diabetes insipidus
organ failure
sweating and tachycardia
simple and complex repetitive and spontaneous movements
bilateral finger tremor
cyclic pupillary dilatation and constriction
repetitive leg movements
myokymia of face
eyelid opening
undulating toe reflex
autocycling respirations that are really ventilator driven
(above since 1995)

cremasteric, plantar, abdominal reflexes
triple flexion response
deep tendon reflexes
respiratory like reflex
Lazarus sign
limb movements besides posturing
(above pre 1995)

cf Scripko and Greer  The Neurologist 2011;17:237-240