Christopher J. Boes

Management of Trigeminal Autonomic Cephalgias and Hemicrania Continua

The trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features. This group of headache disorders includes cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). Although hemicrania continua has previously been classified amongst the TACs, its nosological status remains unclear. Despite their similarities, these disorders differ in their clinical manifestations and response to therapy, thus underpinning the importance of recognising them. We have outlined the clinical manifestations, differential diagnoses, diagnostic workup and the treatment options for each of these syndromes.

History of standard scoring, notation, and summation of neuromuscular signs. A current survey and recommendation.

Abstract  In this article, we trace the history of scoring, notation, and summation of the neuromuscular signs of muscle weakness and decrease of tendon reflexes and sensation. We recommend a standard system to promote consistency in the effort introduced by Mitchell and Lewis to ‘represent systems and force by their signs.’ The scoring of neuromuscular signs began with Mitchell and Lewis in the 19th century who used pluses, minuses, and N (for normal) to express the activity of muscle stretch reflexes. Henry Plummer introduced an ordinal scoring approach for muscle weakness, reflex decrease and increase, and sensation loss. In 1919, he and Walter Sheldon and Henry Woltman introduced standard pre‐printed examination forms with written instructions for notation and scoring. Robert Lovett, a Boston orthopedist, scored weak muscles of poliomyelitis patients from 2 (mild weakness) to 6 (paralyzed), 1 being normal. Lovetts approach was used, after reversing the order of the grades and decreasing each grade by 1, by a Committee of the Medical Research Council for evaluating return of muscle weakness after nerve injury. Despite dissimilarity to existing reflex and sensation scores and uneven width of grades, this approach was widely adopted for use in neurologic practice. We introduced the Neuropathy Impairment Score using a combination of the Mitchell, Plummer, and Lovett approaches, summing all individual scores of a standard set of neuromuscular examinations. In a non‐representative survey of 19 neuromuscular physicians from different countries, we find that there is a considerable variability in the approaches used for grading. Assuming that scoring is useful, we herein suggest (a) impairments should be scored separately from hyperfunction and (b) for the scoring of impairments (muscle weakness, reflex decrease, and sensation loss), the same ordinal scoring approach should be used with 0 as normal and 1, 2, … representing increasing impairment based on the judgment of percentage abnormality with corrections made for age, sex, physical fitness, and physical characteristics.

SUNCT syndrome: Prolonged attacks, refractoriness and response to topiramate

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare form of strictly unilateral headache accompanied by cranial autonomic features. The pain is abrupt in onset, of short duration, stabbing or burning in quality, and accompanied by marked ipsilateral cranial autonomic features, usually lacrimation and conjunctival injection.1 The natural history of the condition is poorly understood. Generally, a clustering pattern is reported, with active phases erratically alternating with remissions. During symptomatic periods, the frequency of attacks varies from less than 1 per day to more than 30 per hour. Functional MRI in spontaneous attacks has demonstrated activation of the ipsilateral posterior hypothalamic gray.2 We describe a patient with SUNCT syndrome with previously unreported responsiveness to topiramate, who has atypical relatively long-lasting pain paroxysms and refractoriness after attacks. A 63-year-old medical practitioner presented with a 3-year history of headache. He described strictly right-sided episodes of severe, shooting or burning pain centered on the temple and forehead with occasional radiation to the orbit. The pain usually lasted 30 to 45 seconds but could range from 2 seconds to 1 minute. He had up to 15 …

Refining the Clinical Spectrum of Chronic Paroxysmal Hemicrania: A Review of 74 Patients

Objective.—To refine the clinical spectrum of chronic paroxysmal hemicrania (CPH).

NEW DAILY PERSISTENT HEADACHE

Chronic daily headache, defined as headache on 15 days or more per month for more than three months, is covered generally in another section of this supplement. However, because the syndrome of “new daily persistent headache” (NDPH) is so clinically distinct (table 1), and has an interesting range of secondary possibilities that are curable, we have elected to highlight the problem here. This is not to imply any linked pathophysiology but simply to highlight what is a clinically useful concept. From a nosological point of view most of what is covered here could be placed in the various parts of the classification system of the International Headache Society.1 It serves both patients and clinicians to highlight this syndrome because the implications of missing the diagnosis can be so profound. NDPH can have both primary2 and secondary forms (table 2). More general texts address the many surrounding issues of headache diagnosis and management.3,4 View this table: Table 1 Proposed diagnostic criteria for new daily persistent headache View this table: Table 2 Differential diagnosis of new daily persistent headache The patient with NDPH presents with a history of headache on most if not all days that began from one day to the next. The onset of headache is abrupt, often moment-to-moment, but at least in less than a few days (where three is suggested as an upper limit).4 The classical history, if that term is yet appropriate, will be for the patient to recall the exact day and circumstances, so from one moment to the next a headache develops that never leaves them. This presentation triggers certain key questions about the onset and behaviour of the pain. These need to be woven with the more generic questions that one asks a patient with persistent headache, to form a provisional diagnosis. The pressing issues arise from considering the …

Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures.

Objective: To characterize the clinical features and MRI abnormalities of leucine-rich glioma-inactivated 1 (LGI1)-autoantibody (Ab) faciobrachial dystonic seizures (FBDS). Methods: Forty-eight patients with LGI1-Ab encephalopathy were retrospectively identified by searching our clinical and serologic database from January 1, 2002, to June 1, 2015. Of these, 26 met inclusion criteria for this case series: LGI1-Ab seropositivity and FBDS. In a separate analysis of all 48 patients initially identified, the MRIs of patients with (n = 26) and without (n = 22) FBDS were compared by 2 neuroradiologists blinded to the clinical details. Results: The median age of the 26 included patients was 62.5 years (range 37–78); 65% were men. FBDS involved arm (26), face (22), and leg (12). Ten were previously diagnosed as psychogenic. Ictal EEGs were normal in 20 of 23 assessed. Basal ganglia T1 and T2 signal abnormalities were detected in 11 patients (42%), with excellent agreement between neuroradiologists (κ scores of 0.86 and 0.93, respectively), and included T1 hyperintensity alone (2), T2 hyperintensity alone (1), or both (8). The T1 hyperintensities persisted longer than the T2 hyperintensities (median 11 weeks vs 1 week, p = 0.02). Improvement with immunotherapy (18/18) was more frequent than with antiepileptic medications (10/24). A separate analysis of all 48 patients initially identified with LGI1-Ab encephalopathy showed that basal ganglia MRI abnormalities were present in 11 of 26 with FBDS but not present in those without FBDS (0/22) (p < 0.001). In contrast, mesial temporal MRI abnormalities were less common among those with FBDS (42%) than those without (91%) (p < 0.001). Conclusions: Basal ganglia T1 hyperintensity is a clinically useful MRI biomarker of LGI1-Ab FBDS and suggests a basal ganglia localization.

Adult polyglucosan body disease: Case description of an expanding genetic and clinical syndrome

A non‐Jewish patient is described who had adult polyglucosan body disease (APBD) and glycogen branching enzyme (GBE) deficiency without GBE mutation. A heterozygous polymorphism (Val160Ile) was found, and also discovered in 1 of 50 normal individuals. Magnetic resonance imaging demonstrated increased T2 signal in the midbrain, medullary olives, dentate nuclei, cerebellar peduncles, and internal and external capsules, with vermian atrophy. Both muscle and nerve biopsy revealed perivascular inflammatory infiltrates. These findings expand the clinical and genetic spectrum of APBD. Factors other than mutation of the expressed GBE gene may cause enzyme deficiency and varied expression and development of APBD. Muscle Nerve 29: 323‐328, 2004

Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis.

Objective.—To describe two cases of chronic paroxysmal hemicrania manifested by otalgia with a sensation of external acoustic meatus obstruction and to suggest that the trigeminal‐autonomic reflex is a mechanism for the sensation of ear blockage.

The paroxysmal hemicrania‐tic syndrome

Two cases of paroxysmal hemicrania (PH) associated with trigeminal neuralgia are reviewed. The paroxysmal hemicrania component in one patient was episodic, while it was chronic in the other. Each headache type responded completely to separate treatment, highlighting the importance of recognizing this association. We review the six other cases of chronic paroxysmal hemicrania-tic (CPH-tic) reported, and suggest that the term paroxysmal hemicrania-tic syndrome (PH-tic) be used to describe this association.

Benign cough headache

Benign cough headache is an uncommon primary headache disorder marked by short- lasting attacks of pain triggered by coughing. Magnetic resonance imaging of the brain is required to assure that the cough headache is truly benign. The aetiology of the pain is unclear, but is probably associated with the brief increased intracranial pressure that attends coughing. We have reviewed the clinical features, aetiology, differential diagnosis, management, and prognosis of benign cough headache.