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.

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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 %).