Postural tachycardia syndrome (POTS)

A heterogenous and multifactorial disorder.

Benarroach EE, Mayo Clin Proc 2012; 87:(12) 1214-1225.

A recent review article by the amazing Eduardo Benarroch. 

Definition POTS:  (IN ADULTS) HR increase of 30 bpm within 10 minutes of standing or head up tilt (HUT) without orthostatic hypotension.  Definition may be inadequate for yong teens or those with low resting HR.  Lesser degrees of abnormality is called "orthostatic intolerance."

Symptoms: of cerebral hypoperfuson and (reflex) sympathetic hyperactivity relieved by incumbency.  They include, light headedness, blurred vision, cognitive difficulty, generalized weakness (for hypoperfusion) and palpitations, chest pain, tremulousness (for sympathetic part).   One third have secondary vasovagal syncope with typical exacerbating factors (heat exposure, heavy meals, exertion, prolonged incumbency, menses and certain drugs).

Demography:  females have more (4.5:1), age usually 15-25, half have preceding viral illness and 25 % have a positive family history of.Deconditioning, psychological factors are important and autonomic defined abnormalities are relatively uncommon. 

Pearls:
1.  "Neuropathic" POTS is a subtype with LE sympathetic denervation with loss of sweating, quantitative sudomotor testing, impaired NE release in LE's in response to orthostatic stress. Its probably due to inpaired vasoconstriction and venous pooling in legs. They are also the high flow subtype in blood flow testing of legs. 14 % have ganglionic acetylecholine receptor antibody, hence may be autoimmune/

2.  Hyperadrenergic POTS-- 30-40 % have high NE levels (>600 pg/mL), HTN during HUT, tachycardia, HTN and hyperhidrosis episodes triggered by orthostasis OR emotional stimuli/physical activity.  This is low volume group with supine vasoconstiction, supine tachycardia, pale and cold skins.  Hyperadrenergic state due to norepinephrine transporter (NET) blockade by drugs (TCA's. methylphenidate and related stimulants and others) or secondary to mast cell disorders.  Consider hyperthyroidism or pheochromocytoma in these patients. 

3.  Hypovolemic POTS (28 %).  May be due to low renin/aldosterone secretion or to inappropriately high ACE-2 activity.  May be related to primary GI disorder of N/V/D. 

4. Comorbidities:  Visceral pain and dysmotility, CFS, FM, sleep disorders, myofascial pain, Ehler Danlos syndrome especially type III with variations in tenascin X.  May be related to early onset of chronic pain, with anxiety and sensory amplification state.  Headache with or without CSF leak.

Cogan syndrome: analysis of reported neurologic manifestations

Antonios N, Silliman S.  The Neurologist 2012; 18:55-62

 

This is a review paper/,eta-analysis of all reported cased.  Cogan syndrome, or  non-syphilitic ocular keratitis and vestibuloauditory dysfunction, is diagnosed clinically not by any test, except to exclude other conditions.  353 cases were reviewed. 

 

Hearing loss, usually high tone SN hearing loss, may start unilaterally but almost always becomes bilateral and is followed by dizziness or vertigo (90 %), nausea and vomiting, and may mimic Meniere's syndrome.  It has a fluctuating and progressive course and can recur after as much as 13 years.  Interstitial keratitis occurs in 77 %, often as the second major symptom complex, often starting with scleral redness, photophobia, eye pain, decreased acuity, due to corneal clouding and may be fluctuating from day to day.  Less common symptoms iclude iritis,uveitis, cataracts, scleritis, conjunctivitis and others.Slit lamp shows corneal stromal scarring and neovascularization. 

 

Systemic symptoms that may occur include fever, rash, lymphadenopathy, arthritis, polychondritis, aortitis, and others.

 

Vestibulocohlear/ocular diseases that can be excluded include Susac's syndrome, GCA, lupus, relapsing polychondritis, Churg-Strauss and Behcet's.   Hearing loss is usually permanent and occurs in first three years,  Topical steroids can be used to preserve vision and topical atropine to reduce eye pain.

 

Neurological involvement (not counting above symptoms) occur in 29 % and include CNS such as stroke, encephalitis, aseptic meningitis, myelopathy, cerebral venous thrombosis and optic nerve disorders.  PNS symptoms are just as common and include cranial neuropathy , especially facial paresis, mononeuropathy, peripheral neuropathy and myopathy. 

 

Authors analyze reports and doubt trueness of reports of ischemic stroke of CVST although acknowledge most of the rest of the above.  Pathology may show arteritic changes.  Treatment with 1 mg/kg prednisone is helpful.

Erythropoetin asneuroprotective in heart surgery

Importance of erythropoietin in brain protection after cardiac surgery: a pilot study; Lakic N, Surlan K, Jerin A, Meglic B, Curk N, Bunc M; Heart Surgery Forum 13 (3), E185-9 (Jun 2010)

BACKGROUND: Neurologic complications after cardiac operations present an important medical problem, as well as a financial burden. They increase the morbidity and hospital stays of patients who have otherwise undergone successful heart operations. The current protocols for perioperative brain protection against ischemic events are not optimal. Because of its different pleiotropic mechanisms of action, recombinant human erythropoietin might provide neuroprotection. METHODS: In this study, we included 20 patients who were older than 18 years and required surgical revascularization of the heart with the use of the heart-lung machine. Ten patients received 3 consecutive intravenous doses (24,000 IU) of recombinant human erythropoietin (rHuEpo). Neurologic and magnetic resonance imaging (MRI) examinations were done before and in the first 5 days after surgery. RESULTS: The erythropoietin-treated and control groups were comparable with respect to study protocol outcomes: number of coronary artery bypass grafts (3.3 and 3.2 grafts/patient, respectively), operative time (4.12 and 4.6 hours), and transfusion volume per patient (708 and 674 mL). The groups were also comparable with respect to blood pressure values at all stages of the operation. MRI scans revealed that 4 of 10 patients from the control group had fresh ischemic brain lesions after open heart surgery. None of the patients in the erythropoietin-treated group had fresh ischemic brain lesions. CONCLUSION: Although the number of patients was small, the results regarding brain protection with rHuEpo are encouraging. rHuEpo is a promising neuroprotective agent.

25

Hand stereotypies and Autism v. Rett's diagnosis

Hand stereotypies distinguish Rett syndrome from autism disorder; Goldman S, Temudo T; Movement Disorders (Jun 2012)

BACKGROUND: Rett syndrome (RTT) and autism disorder (AD) are 2 neurodevelopmental disorders of early life that share phenotypic features, one being hand stereotypies. Distinguishing RTT from AD often represents a challenge, and given their distinct long-term prognoses, this issue may have far-reaching implications. With the advances in genetic testing, the contribution of clinical manifestations in distinguishing RTT from AD has been overlooked. METHODS: A comparison of hand stereotypies in 20 children with RTT and 20 with AD was performed using detailed analyses of videotaped standardized observations. RESULTS: Striking differences are observed between RTT and AD children. In RTT, hand stereotypies are predominantly complex, continuous, localized to the body midline, and involving mouthing. Conversely, in AD children, hand stereotypies are simple, bilateral, intermittent, and often involving objects. CONCLUSIONS: These results provide important clinical signs useful to the differential diagnosis of RTT versus AD, especially when genetic testing for RTT is not an option. © 2012 Movement Disorder Society.

High dose verapamil for cluster headache and other pearls

Management of Cluster Headache; Tfelt-Hansen PC, Jensen RH; CNS Drugs (Jun 2012)

The prevalence of cluster headache is 0.1% and cluster headache is often not diagnosed or misdiagnosed as migraine or sinusitis. In cluster headache there is often a considerable diagnostic delay - an average of 7 years in a population-based survey. Cluster headache is characterized by very severe or severe orbital or periorbital pain with a duration of 15-180 minutes. The cluster headache attacks are accompanied by characteristic associated unilateral symptoms such as tearing, nasal congestion and/or rhinorrhoea, eyelid oedema, miosis and/or ptosis. In addition, there is a sense of restlessness and agitation. Patients may have up to eight attacks per day. Episodic cluster headache (ECH) occurs in clusters of weeks to months duration, whereas chronic cluster headache (CCH) attacks occur for more than 1 year without remissions. Management of cluster headache is divided into acute attack treatment and prophylactic treatment. In ECH and CCH the attacks can be treated with oxygen (12 L/min) or subcutaneous sumatriptan 6 mg. For both oxygen and sumatriptan there are two randomized, placebo-controlled trials demonstrating efficacy. In both ECH and CCH, verapamil is the prophylactic drug of choice. Verapamil 360 mg/day was found to be superior to placebo in one clinical trial. In clinical practice, daily doses of 480-720 mg are mostly used. Thus, the dose of verapamil used in cluster headache treatment may be double the dose used in cardiology, and with the higher doses the PR interval should be checked with an ECG. At the start of a cluster, transitional preventive treatment such as corticosteroids or greater occipital nerve blockade can be given. In CCH and in long-standing clusters of ECH, lithium, methysergide, topiramate, valproic acid and ergotamine tartrate can be used as add-on prophylactic treatment. In drug-resistant CCH, neuromodulation with either occipital nerve stimulation or deep brain stimulation of the hypothalamus is an alternative treatment strategy. For most cluster headache patients there are fairly good treatment options both for acute attacks and for prophylaxis. The big problem is the diagnosis of cluster headache as demonstrated by the diagnostic delay of 7 years. However, the relatively short-lasting attack of pain in one eye with typical associated symptoms should lead the family doctor to suspect cluster headache resulting in a referral to a neurologist or a headache centre with experience in the treatment of cluster headache.

tramadol and tapentadol compared

Mechanistic and functional differentiation of tapentadol and tramadol; Raffa RB, Buschmann H, Christoph T, Eichenbaum G, Englberger W, Flores CM, Hertrampf T, Kögel B, Schiene K, Straßburger W, Terlinden R, Tzschentke TM; Expert Opinion on Pharmacotherapy (Jun 2012)

 

Introduction: Many opioid analgesics share common structural elements; however, minor differences in structure can result in major differences in pharmacological activity, pharmacokinetic profile, and clinical efficacy and tolerability. Areas covered: This review compares and contrasts the chemistry, pharmacodynamics, pharmacokinetics, and CNS 'functional activity' of tapentadol and tramadol, responsible for their individual clinical utilities. Expert opinion: The distinct properties of tapentadol and tramadol generate different CNS functional activities, making each drug the prototype of different classes of opioid/nonopioid analgesics. Tramadol's analgesia derives from relatively weak µ-opioid receptor (MOR) agonism, plus norepinephrine and serotonin reuptake inhibition, provided collectively by the enantiomers of the parent drug and a metabolite that is a stronger MOR agonist, but has lower CNS penetration. Tapentadol's MOR agonist activity is several-fold greater than tramadol's, with prominent norepinephrine reuptake inhibition and minimal serotonin effect. Accordingly, tramadol is well-suited for pain conditions for which a strong opioid component is not needed-and it has the benefit of a low abuse potential; whereas tapentadol, a schedule-II controlled substance, is well-suited for pain conditions requiring a strong opioid component-and it has the benefit of greater gastrointestinal tolerability compared to classical strong opioids. Both drugs offer distinct and complementary clinical options.

Spontaneous abscess think and look for HHT

Polymicrobial brain abscess in hereditary haemorrhagic telangiectasia (Osler's disease)]; Polak P, Snopkova S, Husa P; Deutsche Medizinische Wochenschrift 137 (33), 1635-8 (Aug 2012)

History and admission findings: A 38-year-old woman who suffered from migraine was admitted because of severe, worsening headache for 24 hours (dissimilar to the previous migraine attacks), with impaired vision and weakness of the right arm. Mild hemiparesis and expressive aphasia indicated an intracranial tumor.Investigations: Cranial computed tomography revealed a focal lesion with a diameter of 2.5 cm in the left frontoparietal lobe, with signs of intracranial hypertension, indicating cerebral metastasis or an abscess. Magnetic resonance imaging confirmed the diagnosis of a brain abscess.Treatment and course: An urgent craniotomy was performed and the abscess was evacuated. An empirical antibiotic combination with chloramphenicole and metronidazole (switched to cefotaxime because of thrombocytopenia) was initiated. Cultivation of pus revealed Streptococcus constellatus, Aggregatibacter aphrophilus and Fusobacterium spp. Within the first two weeks of treatment progession of the abscess was noted, therefore a second craniotomy with debridement was performed. An elective CT-angio scan revealed several arteriovenous malformations in the caudal segments of both lungs which were embolized without complications. Only retrospectively, cutaneous teleangiectasias were recognized. At present, the patient and her direct relatives are submitted to genetical screening for Osler's disease.Conclusion: In patients with brain abscesses of unknown origin and with a history of repeated epistaxis and/or gastrointestinal bleeding, Osler's disease (hereditary hemorrhagic telangiectasia) should be considered and pulmonary arteriovenous malformations excluded. Physicians should search for cutaneous or mucous teleangiectasias. Family screening and long-term follow-up according to international guidelines is recommended.

Comment  Another reminder of historical questions to ask about when examining a patient with brain abscess.

Omega-3 fa's for protection against paclitaxel induced PN

Omega-3 fatty acids are protective against paclitaxel-induced peripheral neuropathy: A randomized double-blind placebo controlled trial; Ghoreishi Z, Esfahani A, Djazayeri A, Djalali M, Golestan B, Ayromlou H, Hashemzade S, Asghari Jafarabadi M, Montazeri V, Keshavarz SA; BMC Cancer 12 (1), 355 (Aug 2012)

ABSTRACT: BACKGROUND: Axonal sensory peripheral neuropathy is the major dose-limiting side effect of paclitaxel.Omega-3 fatty acids have beneficial effects on neurological disorders from their effects on neurons cells and inhibition of the formation of proinflammatory cytokines involved in peripheral neuropathy. METHODS: This study was a randomized double blind placebo controlled trial to investigate the efficacy of omega-3 fatty acids in reducing incidence and severity of paclitaxel-induced peripheral neuropathy (PIPN). Eligible patients with breast cancer randomly assigned to take omega-3 fatty acid pearls, 640 mg t.i.d during chemotherapy with paclitaxel and one month after the end of the treatment or placebo. Clinical and electrophysiological studies were performed before the onset of chemotherapy and one month after cessation of therapy to evaluate PIPN based on"reduced Total Neuropathy Score". RESULTS: Twenty one patients (70 %) of the group taking omega-3 fatty acid supplement (n = 30) did not develop PN while it was 40.7 %( 11 patients) in the placebo group(n = 27). A significant difference was seen in PN incidence (OR = 0.3, .95 % CI = (0.10-0.88), p = 0.029). There was a non-significant trend for differences of PIPN severity between the two study groups but the frequencies of PN in all scoring categories were higher in the placebo group (0.95 % CI = ([MINUS SIGN]2.06 -0.02), p = 0.054). CONCLUSIONS: Omega-3 fatty acids may be an efficient neuroprotective agent for prophylaxis against PIPN. Patients with breast cancer have a longer disease free survival rate with the aid of therapeutical agents. Finding a way to solve the disabling effects of PIPN would significantly improve the patients' quality of life.Trial registrationThis trial was registered at ClinicalTrials.gov (NCT01049295).

Comment-- a disease in need of a prevention and cure!

More on overutilization of TA biopsy

Temporal Artery Biopsy is not Required in all Cases of Suspected Giant Cell Arteritis; Quinn EM, Kearney DE, Kelly J, Keohane C, Redmond HP; Annals of Vascular Surgery (Jan 2012)

 

BACKGROUND: Temporal artery biopsy (TAB) is performed during the diagnostic workup for giant cell arteritis (GCA), a vasculitis with the potential to cause irreversible blindness or stroke. However, treatment is often started on clinical grounds, and TAB result frequently does not influence patient management. The aim of this study was to assess the need for TAB in cases of suspected GCA. METHODS: We performed a retrospective review of 185 TABs performed in our institution from 1990 to 2010. Patients were identified through the Hospital In-Patient Enquiry database and theater records. Clinical findings, erythrocyte sedimentation rate, steroid treatment preoperatively, American College of Rheumatology (ACR) criteria for GCA score, biopsy result, and follow-up were recorded. RESULTS: Fifty-eight (31.4%) biopsies were positive for GCA. Presence of jaw claudication (P = 0.001), abnormal fundoscopy (P = 0.001), and raised erythrocyte sedimentation rate (P = 0.001) were significantly associated with GCA. The strongest association with positive biopsy was seen with the prebiopsy ACR score (P<0.001). Twenty-four (13.7%) patients had undergone biopsy, despite no potential for meeting ACR criteria preoperatively. None of these were positive. Overall, 29 (16.4%) patients had management altered by TAB result. CONCLUSIONS: Our results confirm that TAB does not affect management in the majority of patients with suspected GCA. We conclude that TAB has benefit only for patients who score 2 or 3 on the ACR criteria for GCA without biopsy.

 

Comment-- TA biopsy, along with brain biopsy and muscle biopsy is overutilized and requires expert analysis before being ordered.

Paroxetine and venlafaxine for depression in Parkinson's disease

A randomized, double-blind, placebo-controlled trial of antidepressants in Parkinson disease; Richard IH, McDermott MP, Kurlan R, Lyness JM, Como PG, Pearson N, Factor SA, Juncos J, Serrano Ramos C, Brodsky M, Manning C, Marsh L, Shulman L, Fernandez HH, Black KJ, Panisset M, Christine CW, Jiang W, Singer C, Horn S, Pfeiffer R, Rottenberg D, Slevin J, Elmer L, Press D, Hyson HC, McDonald W, For the SAD-PD Study Group; Neurology 78 (16), 1229-1236 (Apr 2012)

 

OBJECTIVE: To evaluate the efficacy and safety of a selective serotonin reuptake inhibitor (SSRI) and a serotonin and norepinephrine reuptake inhibitor (SNRI) in the treatment of depression in Parkinson disease (PD). METHODS: A total of 115 subjects with PD were enrolled at 20 sites. Subjects were randomized to receive an SSRI (paroxetine; n = 42), an SNRI (venlafaxine extended release [XR]; n = 34), or placebo (n = 39). Subjects met DSM-IV criteria for a depressive disorder, or operationally defined subsyndromal depression, and scored>12 on the first 17 items of the Hamilton Rating Scale for Depression (HAM-D). Subjects were followed for 12 weeks (6-week dosage adjustment, 6-week maintenance). Maximum daily dosages were 40 mg for paroxetine and 225 mg for venlafaxine XR. The primary outcome measure was change in the HAM-D score from baseline to week 12. RESULTS: Treatment effects (relative to placebo), expressed as mean 12-week reductions in HAM-D score, were 6.2 points (97.5% confidence interval [CI] 2.2 to 10.3, p = 0.0007) in the paroxetine group and 4.2 points (97.5% CI 0.1 to 8.4, p = 0.02) in the venlafaxine XR group. No treatment effects were seen on motor function. CONCLUSIONS: Both paroxetine and venlafaxine XR significantly improved depression in subjects with PD. Both medications were generally safe and well tolerated and did not worsen motor function. Classification of Evidence: This study provides Class I evidence that paroxetine and venlafaxine XR are effective in treating depression in patients with PD.

382 Stars

VISUAL SNOW SYNDROME

Presented by Christopher Schankin Ph D at UCSD Headache center

 

More than half of patients with "visual snow" have migraine, and almost all have other symptoms in eyes including floaters (73 %), persistent visual images (63 %), difficulty seeing at night (58 %) tiny objects moving on blue sky (57 %), light sensistivity (54 %), trails behind moving images (48 %), bright flashes (44 %), colored swirls , clouds, or waves when their eyes were closed (41 %). 

cataplexy clips on CNN

http://www.youtube.com/watch?v=ALVqaKQ5MWY&feature=related

a little long  / basic but there are lots clips of cataplexy, overt and subtle

nv

CLIPPERS syndrome

Core features of CLIPPERS (an MS mimic)

Clinical-- subacute progressive ataxia and dysarthria.  May in some cases have other brainstem features or cognitive symptoms.  These include facial paresthesias, diplopia, dysgeusia and myelopathy. 

MRI- 1.  numerous nodular or punctate enhancing lesions bilaterally in at least 2 of 3: pons, cerebellum, brachium pontis  2.  Individual lesions are small but may coalesce to form larger lesions (mass effect has never been reported)  3.  Lesions may occur in spinal cord, basal ganglia nad cerebral white matter but should be less dense the further from the pons  4.  Absent features: restricted diffusion on DWI, marked hyperintensity on T2, abnormal cerebral angiogram

Corticosteroid responsiveness-- prompt and significant clinical and radiographic response to steroids-- with relapse when steroids are withdrawn or fell below 20 mg po daily.  Uncertain:  elevated IgE in some patients is reported as are oligoclonal bands.

Histopathology--- a. white matter perivascular lymphohistiocytic infiltrate with or without parenchymal extension   b.  infiltrate contains predominant CD+ and CD4+ lymphocytes  c.  absent monoclonal or atypical lymphocyte population , necrotizing giant cells or granulomas, or histologic features of vasculitis such as destruction of vessel wall, fibronoid necrosis, leukocytoclasia, fibrin thrombi. 

Exclusion of mimicking diseases including sarcoid, neuro-Behcet's, vasculitis and lymphoma, glioma, histiocytosis, multiple sclerosis, autoimmmune disorders, Bickerstaff's encephalitis. 

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

AED selection for patients taking antiretrovirals

Due to interactions, dose adjustments are often needed. This is a position paper with mostly level C recommendations. 

1.  If taking PTN, may need to increase lopinavir/ritonavir dosage up to 50 % to maintain levels
2.  Patients on VPA may need to reduce zidovudine dose to maintain zid. levels in serum
3.  Coadministration of VPA and efavirenz does not require dose adjustment of ef.
4.  Patients on ritonavir/ atazanavir may need 50 % lamotrigine dose increase to maintain LTG levels
5.  Coadministration of raltegravir/atazanavir and LTG may not require LTG dose adjustment
6.  Coadministration of raltegravir and midazolam may not require midazolam dose adjustment
7.  Counsel patients its unclear whether combinations of AED's and ARV's require dose adjustments esp enzyme inducers.  They may lead to virologic failure, esp protease inhibitors and nonnucleoside reverse transcriptase inhibitors

Combination AED therapy with Depakote and lamotrigine

Neurology 2012; 78: 62-68  Combing records of 148 disabled adults in state run institutions, authors analyzed whether any combination of AED's were superior to others.  Out of 32 AED combinations, only the combination of lamotrigine and valproate was superior to others,AND the addition of a third AED aggregately did not add to epileptic control over the use of two medicines