Chronic persistent Horner's syndrome in trigeminal autonomic cephalalgia subtypes and alleviation with treatment: two case reports.

BACKGROUND: The trigeminal autonomic cephalalgias are a group of primary headache syndromes marked by severe head pain and associated cranial autonomic symptoms which can include a full or partial Horner's syndrome. Rarely, the eye-related symptoms will become fixed even between headache attacks. There is minimal documentation that the Horner's syndrome can be reversed if successful treatment of the underlying headache disorder is initiated. CASE REPORTS: Two cases are presented of trigeminal autonomic cephalalgia subtypes with chronic persistent Horner's syndromes that alleviated with treatment of the underlying primary headache disorder. Patient 1, an 82-year-old Caucasian woman, presented with hemicrania continua with a partial Horner's syndrome that was present for 2 years. She was unable to take indomethacin as she was on anticoagulation. After a C2-3 diagnostic facet injection, not only did she become pain free but her ptosis completely resolved. She then underwent a radiofrequency facet neurotomy with complete alleviation of head pain and complete resolution of her ptosis. Patient 2, a 21-year-old Caucasian woman, presented with long-lasting autonomic symptoms with hemicrania syndrome and a fixed miosis and ptosis of 6 months' duration. After achieving 2 months of pain freedom on indomethacin her Horner's syndrome completely resolved. CONCLUSION: A chronic fixed partial or full Horner's syndrome can occur in trigeminal autonomic cephalalgia subtypes, but it can also be reversed in patients with treatment even after months to years of duration. This would suggest that the sympathetic dysfunction leading to the eye-related symptoms is from irritation of the sympathetic chain rather than permanent injury as the result of vasodilatory trauma after trigeminal autonomic reflex activation.

Tics in TACs: A Step into an Avalanche? Systematic Literature Review and Conclusions.

BACKGROUND: Trigeminal autonomic cephalalgias (TACs) comprise cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua. In some cases, trigeminal neuralgia (TN, "tic douloureux") or TN-like pain may co-occur with TACs. AIM: This article will review the co-occurrence and overlap of TACs and tics in order to contribute to a better understanding of the issue and an improved management of the patients. METHODS: For performing a systematic literature review Pubmed was searched using a total of ten terms. The articles identified were screened for further articles of relevance. SUMMARY: TACs are related to tics in various ways. TN or TN-like paroxysms may co-occur with CH, PH, and HC, labeled as cluster-tic syndrome, PH-tic syndrome, and HC-tic syndrome. Such co-occurrence was not only found in the primary TACs but also in secondary headaches resembling TACs. The initial onset of TAC and tic may be simultaneous or separated by months or years. In acute attacks, tic and TAC may occur concurrently or much more often independently of each other. The term "cluster-tic syndrome" was also used in patients with a single type of pain in a twilight zone between TACs and TN fulfilling none of the relevant diagnostic criteria. Short-lasting neuralgiform headache attacks overlap with TN in terms of clinical features, imaging findings, and therapy.

Side-locked headaches: an algorithm-based approach.

The differential diagnosis of strictly unilateral hemicranial pain includes a large number of primary and secondary headaches and cranial neuropathies. It may arise from both intracranial and extracranial structures such as cranium, neck, vessels, eyes, ears, nose, sinuses, teeth, mouth, and the other facial or cervical structure. Available data suggest that about two-third patients with side-locked headache visiting neurology or headache clinics have primary headaches. Other one-third will have either secondary headaches or neuralgias. Many of these hemicranial pain syndromes have overlapping presentations. Primary headache disorders may spread to involve the face and / or neck. Even various intracranial and extracranial pathologies may have similar overlapping presentations. Patients may present to a variety of clinicians, including headache experts, dentists, otolaryngologists, ophthalmologist, psychiatrists, and physiotherapists. Unfortunately, there is not uniform approach for such patients and diagnostic ambiguity is frequently encountered in clinical practice.Herein, we review the differential diagnoses of side-locked headaches and provide an algorithm based approach for patients presenting with side-locked headaches. Side-locked headache is itself a red flag. So, the first priority should be to rule out secondary headaches. A comprehensive history and thorough examinations will help one to formulate an algorithm to rule out or confirm secondary side-locked headaches. The diagnoses of most secondary side-locked headaches are largely investigations dependent. Therefore, each suspected secondary headache should be subjected for appropriate investigations or referral. The diagnostic approach of primary side-locked headache starts once one rule out all the possible secondary headaches. We have discussed an algorithmic approach for both secondary and primary side-locked headaches.

An approach to anisocoria.

PURPOSE OF REVIEW: Anisocoria is a finding seen on a daily basis in nearly every eye clinic. Although often benign, it can also represent the sole sign of a life-threatening disease making an up-to-date understanding of pathophysiology and diagnosis essential for anyone practicing medicine. RECENT FINDINGS: Many aspects of the traditional approach to anisocoria still hold true today, but advancements in imaging technology and changing trends in pharmacologic diagnosis and localization have led many to rethink that approach. In addition, the differential diagnosis for anisocoria continuously expands with identification and improved understanding of causal disease processes. SUMMARY: The present article discusses an approach to the classic anisocoria diagnostic algorithm modified by current knowledge from the most recent literature.

The temporal evolution of a facial pain syndrome associated with neurovascular contact: a case report.

BACKGROUND: Trigeminal autonomic cephalalgias are primary headaches characterized by unilateral pain and cranial autonomic symptoms. However, associated autonomic symptoms have also been reported in other headaches and facial pains, e.g. trigeminal neuralgia, with the clinical differentiation proving a complex task. CASE: A 54-year-old man presented with right-sided, sharp, intense facial pain in the distribution area of the trigeminal nerve. Pain duration was from seconds to a few minutes, and trigger factors included ipsilateral touching of the skin and hair. Over the next ten years, symptoms progressed and changed presentation, also displaying as right-sided, severe, orbital pain, lasting 60 to 90 minutes, with conjunctival injection and rhinorrhea. Neurological examination was normal. Numerous medications were tried with limited or no effect. In 2010, magnetic resonance imaging revealed a right-sided deviation of the basilar artery at the level of pons, creating neurovascular contact with the trigeminal nerve. Microvascular decompression was performed, and symptoms resolved within days. CONCLUSION: Differentiating between trigeminal autonomic cephalalgias and trigeminal neuralgia with autonomic symptoms can be challenging. The distinct change and evolution over time in the clinical presentation of the patient's head pain suggests a temporal plasticity of the pain in head and facial syndromes, irrespective of underlying pathoanatomic features.

Hemicrania continua with contralateral cranial autonomic features: a case report.

BACKGROUND: Hemicrania continua is characterized by continuous strictly unilateral head pain with episodic exacerbations. Episodic exacerbations are associated with ipsilateral cranial autonomic features. CASE DESCRIPTION: We report a 24-year female with a 2-year history of continuous right-sided headache with superimposed exacerbations. Episodic exacerbations were associated with marked agitation and contralateral cranial autonomic features. The patient showed a complete response to indomethacin within 8 hours. DISCUSSION: The dichotomy of pain and autonomic features is in accordance with the concept about the possibility of two separate pathways for pain and autonomic features in trigeminal autonomic cephalalgias.

An unusual case of episodic SUNCT responding to high doses of topiramate.

Trigeminal autonomic cephalalgias (TAC) are rare. Cluster headaches comprise the majority, with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) being the rarest and shortest in duration. The majority of SUNCT are primary with a few cases occurring secondary to posterior fossa or pituitary lesions. Although activities like exercise or blowing of the nose can trigger SUNCT, onset during orgasm has not been described. Short-lasting aura has been described in TACs including SUNCT, but persistence of focal symptoms and signs without an underlying structural lesion have not been described. Lastly, treatment of SUNCT is difficult, with lamotrigine being the most common effective reported. We report a case of episodic SUNCT with symptoms suggestive of brainstem stroke that completely resolved spontaneously for which no underlying structural cause was found. The onset of first attack occurred during orgasm, and the patient responded to a high dose of topiramate.

[Migraine with prolonged eyelid edema: a series of 10 cases]. / Migraña con edema palpebral prolongado: serie de 10 casos.

INTRODUCTION: Migraine may present with cranial autonomic symptoms typical of trigeminal-autonomic cephalalgias, thus posing diagnostic difficulties. AIM. To report a series of patients with prominent eyelid oedema associated with migraine. PATIENTS AND METHODS: Ten patients attending the headache offices in three hospitals (nine women, one man; age: 26-53 years-old) with recurrent eyelid oedema as a migraine accompaniment. RESULTS: According to the diagnostic criteria of the International Classification of Headache Disorders (ICHD-III, beta version), eight patients had migraine without aura, one had migraine with aura, and one had chronic migraine. Eyelid oedema appeared during the most severe headache attacks, and had longer duration than the pain. Pharmacological or systemic causes of the oedema were ruled out in all cases. Other associated autonomic symptoms were conjunctival injection (n = 3), lacrimation (n = 2) and rhinorrhoea (n = 1). Both the pain and the oedema improved with symptomatic and preventive therapies for migraine. CONCLUSIONS: Eyelid oedema may occasionally be a migraine accompaniment. It appears in some patients during their most severe migraine attacks, and may improve with the acute and preventive treatment for migraine.

TITLE: Migraña con edema palpebral prolongado: serie de 10 casos.Introduccion. La migraña puede cursar con sintomas autonomicos craneales propios de las cefaleas trigeminoautonomicas, lo que plantea dificultades en el diagnostico. Objetivo. Describir una serie de diez pacientes con edema palpebral asociado a la migraña. Pacientes y metodos. Diez pacientes atendidos en la consulta de cefaleas de tres hospitales (nueve mujeres, un varon; edad: 26-53 años), con edema palpebral recurrente asociado a la migraña. Resultados. Segun los criterios diagnosticos de la Clasificacion Internacional de las Cefaleas (ICHD-III, version beta), ocho pacientes presentaban migraña sin aura, una tenia migraña con aura y otra, migraña cronica. El edema palpebral aparecia durante las crisis de migraña mas intensas, y tenia mayor duracion que la cefalea. Se descartaron causas farmacologicas o sistemicas del edema en todos los casos. Otros sintomas autonomicos asociados fueron la inyeccion conjuntival (n = 3), el lagrimeo (n = 2) y la rinorrea (n = 1). Tanto el dolor como el edema asociado respondieron a los tratamientos sintomaticos y preventivos de la migraña. Conclusiones. El edema palpebral es un posible acompañante de la migraña. Aparece en algunos pacientes con los episodios de mayor intensidad, y responde al tratamiento sintomatico y preventivo de la migraña.

Primary headaches in children under the age of 7 years.

Recurrent headache is increasingly recognised in young children. Migraine and tension-type headache feature commonly amongst the primary headache disorders seen at this age. Headaches at this age are more likely than in older patients to be 'unclassifiable', possibly a reflection of the difficulties in obtaining a detailed headache history from a young child. Together with recent epidemiological data this review highlights the more prevalent primary headache types with advice on making a focussed headache assessment and guidance on management in this age group.

Concomitant occurrence of different trigeminal autonomic cephalalgias: a case series and review of the literature.

INTRODUCTION: The trigeminal autonomic cephalalgias (TACs) subsume four primary headache disorders. Hemicrania continua is increasingly regarded as an additional TAC. In rare cases patients may present with two different TACs or a TAC and hemicrania continua. CASES: We report four patients with two different TACs or one TAC and hemicrania continua. Two patients presented with cluster headache and paroxysmal hemicrania, one patient with cluster headache and hemicrania continua, and one patient suffered from cluster headache and SUNCT. DISCUSSION: While the International Classification of Headache Disorders (ICHD-II) proposes specific diagnostic criteria, the variability of clinical presentation may make clear diagnosis difficult. All patients fulfilled the ICHD-II criteria. The manifestation of two different TACs or hemicrania continua in one patient is uncommon but possible and should be taken into account especially when chronic headache patients present with changing headache symptoms.

Coexistence of hemiplegic migraine with SUNCT or SUNA: a case series.

BACKGROUND: Hemiplegic migraine (HM) is a rare subtype of migraine with aura that includes transient hemiparesis of different degrees. The dominantly inherited type, called familial hemiplegic migraine, along with the sporadic type, in which no family history of similar attacks exists, have been recently recognized by the revised International Classification of Headache Disorders (ICHD-2). Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are also very rare conditions that are classified as trigeminal autonomic cephalalgias. This report provides the first description of an association between HM and SUNCT/SUNA. CASES: Eight females and two males with both these disorders were identified between 2007 and 2010. CONCLUSION: The coexistence of such rare primary headache disorders is probably due to a common underlining mechanism, with a sodium channelopathy being an attractive potential candidate.

Acute unilateral poliosis concurrent with trigeminal autonomic cephalalgia: a possible aetiological association.

We report a 24-year-old man who presented with the sudden onset of unilateral poliosis associated with acute trigeminal autonomic cephalalgia, suggesting a pathophysiology in common and a possible neural hypothesis in the development of segmental vitiligo. Although rare, associations with neurological abnormalities should be considered in cases of focal depigmentation disorders.

Coexisting trigeminal autonomic cephalalgias and hemicrania continua.

The trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) share many clinical characteristics including unilateral pain and ipsilateral autonomic features. We report a patient with a history of migraine without aura who developed cluster headache and HC simultaneously. The distinctive clinical features and differential response profiles to various treatments indicates that they are distinct disorders. We then review previous reports of patients with coexisting TACs and HC and discuss the relationship between these families of primary headache disorders.

Trigeminal autonomic cephalalgias: a review and implications for dentistry.

BACKGROUND: The authors review the epidemiology, clinical features, pathophysiology, diagnosis, treatment, orofacial presentations and dental implications of trigeminal autonomic cephalalgias (TACs): cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). TYPES OF STUDIES REVIEWED: The authors conducted PUBMED searches for the period from 1968 through 2007 using the terms "trigeminal autonomic cephalalgias," "cluster headache," "paroxysmal hemicrania," "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing," "epidemiology," "pathophysiology," "treatment," "oral," "facial" and "dentistry." They gave preference to articles reporting randomized, controlled trials and those published in English-language peer-reviewed journals. RESULTS: TACs refers to a group of headaches characterized by unilateral head pain, facial pain or both with accompanying autonomic features. Although their pathophysiologies are unclear, CH, PH and SUNCT may be differentiated according to their clinical characteristics. Current treatments for each of the TACs are useful in alleviating the pain, with few refractory cases requiring surgical intervention. Patients with TACs often visit dental offices seeking relief for their pain. CLINICAL IMPLICATIONS: Although the prevalence of TACs is small, it is important for dentists to recognize the disorder and refer patients to a neurologist. This will avoid the pitfall of administering unnecessary and inappropriate traditional dental treatments in an attempt to alleviate the neurovascular pain.

Neuro-ophthalmologic manifestations of primary headache disorders.

Headaches are the most common disorders of the central nervous system affecting 46% of the adult population worldwide. Headaches may be lifelong illnesses, often associated with substantial disability for the individual and the population as a whole. The International Classification of Headache Disorders (ICHD-II) codifies headache disorders into fourteen categories, predominantly primary headaches and secondary headache disorders. Primary headache disorders, mainly migraine and trigeminal autonomic cephalgias (TACs), are frequently associated with neuro-ophthalmologic manifestations. Ophthalmologists are often the first physicians to be involved in the deciphering of headache-related visual disturbances. This article reviews two major primary headache disorders, migraine and trigeminal autonomic cephalgias, and discusses their neuro-ophthalmic complications, clinical presentation, and treatment.