Mark W. Green

Practice guideline update summary: Botulinum neurotoxin for the treatment of blepharospasm, cervical dystonia, adult spasticity, and headache Report of the Guideline Development Subcommittee of the American Academy of Neurology

Objective: To update the 2008 American Academy of Neurology (AAN) guidelines regarding botulinum neurotoxin for blepharospasm, cervical dystonia (CD), headache, and adult spasticity. Methods: We searched the literature for relevant articles and classified them using 2004 AAN criteria. Results and recommendations: Blepharospasm: OnabotulinumtoxinA (onaBoNT-A) and incobotulinumtoxinA (incoBoNT-A) are probably effective and should be considered (Level B). AbobotulinumtoxinA (aboBoNT-A) is possibly effective and may be considered (Level C). CD: AboBoNT-A and rimabotulinumtoxinB (rimaBoNT-B) are established as effective and should be offered (Level A), and onaBoNT-A and incoBoNT-A are probably effective and should be considered (Level B). Adult spasticity: AboBoNT-A, incoBoNT-A, and onaBoNT-A are established as effective and should be offered (Level A), and rimaBoNT-B is probably effective and should be considered (Level B), for upper limb spasticity. AboBoNT-A and onaBoNT-A are established as effective and should be offered (Level A) for lower-limb spasticity. Headache: OnaBoNT-A is established as effective and should be offered to increase headache-free days (Level A) and is probably effective and should be considered to improve health-related quality of life (Level B) in chronic migraine. OnaBoNT-A is established as ineffective and should not be offered for episodic migraine (Level A) and is probably ineffective for chronic tension-type headaches (Level B).

Headaches: Psychiatric Aspects

Headache, and in particular, migraine, is often associated with comorbid psychiatric illness. The complex relationships between these disorders are slowly becoming understood. Successful management requires an integrated approach of neurologic and psychiatric management.

Tolosa-Hunt Syndrome: Appraising the ICHD-3 beta diagnostic criteria

Introduction The term Tolosa-Hunt Syndrome was first used more than half a century ago to describe painful ophthalmoplegia accompanied by cranial nerve palsies. In the decades since, its diagnostic criteria have evolved considerably. The beta version of the 3rd Edition of the International Classification of Headache Disorders narrows these criteria to require the demonstration of granulomatous inflammation on MRI or biopsy. We believe this may introduce challenges to accurate diagnosis. Discussion Requiring the demonstration of granulomatous inflammation for a diagnosis of Tolosa-Hunt Syndrome may introduce the potential for false negative and false positive diagnoses. Although the disorder presents secondary to granulomatous inflammation, MRI technology may not be able to identify it reliably, and biopsy is not always indicated for its symptomatology. Additionally, several cases have been reported of Tolosa-Hunt Syndrome diagnosed with MRI-confirmed granulomatous inflammation that later prove to be attributable to other pathologies. The emphasis on neuroimaging may therefore exclude some true Tolosa-Hunt Syndrome cases and include others resulting from other latent pathologies that are not visible on MRI. Conclusion We wish to offer several potential modifications to the International Classification of Headache Disorders guidelines for Tolosa-Hunt Syndrome, including making the demonstration of granulomatous inflammation on MRI or biopsy non-mandatory and lengthening patient follow-up to two years for cases in which MRI is unrevealing.

Collecting the History in the CDH Patients

Chronic daily headache (CDH) is a frequently used but nonspecific term that includes all the headaches which are experienced on more than 15 days per month for at least 3 months. There are numerous headache disorders, both primary and secondary, that behave like this. They represent a great challenge to medical professionals and a burden to the patient and society. The primary CDHs (for which no underlying cause is found) are the most common. Of those, chronic migraine is the leading problem encountered in specialized headache clinics, much more common than other primary CDHs of long duration (≥4 h/day). Most important primary CDHs of short duration (<4 h/day) are the trigeminal autonomic cephalalgias (TACs), i.e., chronic cluster headache. There is a host of secondary causes of CDH, and they need to be carefully diagnosed based on history, examination, and additional investigations.

Chapter 6 – Complicated Migraine

The term “complicated migraine” (CM) is often applied to unexplained transient neurological symptoms by clinicians. CM is a diagnosis requiring an exclusion of other pathologies using rigorous criteria. The varied symptoms and presentations of complex aura are a fascinating window into the pathophysiology of migraine. Unilateral, bilateral, positive, and negative phenomena all occur, and are transient, reversible, and generally without permanent neurological sequelae. CM requires prompt medical attention to rule out other neurological disorders that may be amenable to treatment. In a presentation in which cerebral infarction is a likely differential diagnosis, triptans are avoided. Connecting its multiple presentations is a common denominator: the difficulty in diagnosis and lack of data to support preventive and acute therapies. This can be frustrating for the patient and clinician. There is need for further research and discovery in this field.

Overview of Migraine: Recognition, Diagnosis, and Pathophysiology

Migraine affects 12% of the population with recurrent attacks, and the features of these vary widely. This condition is likely due to a variety of genetic predilections, which lower the threshold for activating the trigeminovascular system in the brain. Migraine is also an inflammatory disorder. Failure to treat an attack rapidly reduces the chance of rendering the sufferer pain free. There is evidence that migraine can be progressive, is adversely influenced by the burden of migraine, and that aggressive management may be disease modifying.