Hypomimia in Parkinson's Disease: What Is It Telling Us?

Introduction: Amimia is one of the most typical features of Parkinson's disease (PD). However, its significance and correlation with motor and nonmotor symptoms is unknown. The aim of this study is to evaluate the association between amimia and motor and nonmotor symptoms, including cognitive status, depression, and quality of life in PD patients. We also tested the blink rate as a potential tool for objectively measuring upper facial bradykinesia. Methods: We prospectively studied amimia in PD patients. Clinical evaluation was performed using the Unified Parkinson's Disease Rating Scale (UPDRS) and timed tests. Cognitive status, depression, and quality of life were assessed using the Parkinson's Disease Cognitive Rating Scale (PD-CRS), the 16-Item Quick Inventory of Depressive Symptomatology (QIDS-SR16), and the PDQ-39, respectively. Amimia was clinically evaluated according to item 19 of UPDRS III. Finally, we studied upper facial amimia by measuring resting blink frequency and blink rate during spontaneous conversation. Results: We included 75 patients. Amimia (item 19 UPDRS III) correlated with motor and total UPDRS (r: 0.529 and 0.551 Spearman), and its rigidity, distal bradykinesia, and motor axial subscores (r: 0.472; r: 0.252, and r: 0.508, respectively); Hoehn and Yahr scale (r: 0.392), timed tests, gait freezing, cognitive status (r: 0.29), and quality of life (r: 0.268) correlated with amimia. Blinking frequency correlated with amimia (measured with item 19 UPDRS), motor and total UPDRS. Conclusion: Amimia correlates with motor (especially axial symptoms) and cognitive situations in PD. Amimia could be a useful global marker of overall disease severity, including cognitive decline.

Nummular headache: an update and future prospects.

INTRODUCTION: Nummular headache (NH) is characterized by continuous or intermittent head pain, which remains confined to a round or oval shaped area of the scalp, generally 1 to 6 cm in diameter. Usually mild or moderate in intensity, some patients suffer severe acute exacerbations or continuous disabling pain. Areas covered: This article reviews epidemiological and clinical features of NH, the most recently proposed pathophysiological mechanisms, and state-of-the-art management according to the literature. Expert commentary: Information regarding true incidence and prevalence is lacking, but NH is a fairly common disorder in patients attending a headache clinic. Diagnostic work-up requires the exclusion of systemic and structural disease by a thorough physical examination, blood tests including immunology screening, and neuroimaging. No clinical trials have been conducted for NH, so the level of evidence for any treatment is low. Gabapentin seems to be the most effective oral medication; subcutaneous injection of the area with onabotulinum toxin type A also seems to be effective, and should be considered as an alternative to gabapentin.

Supratrochlear Neuralgia: A Prospective Case Series of 15 Patients.

OBJECTIVE: The aim of this study was to describe clinical features unique to supratrochlear neuralgia. BACKGROUND: The supratrochlear nerve supplies the medial aspect of the forehead. Due to the intricate relationship between supraorbital and supratrochlear nerves, neuralgic pain in this region has been traditionally attributed to supraorbital neuralgia. No cases of supratrochlear neuralgia have been reported so far. METHODS: From 2009 through 2016, we prospectively recruited patients with pain confined to the territory of the supratrochlear nerve. RESULTS: Fifteen patients (13 women, 2 men; mean age 51.4 years, standard deviation 14.9) presented with pain in the lower paramedian forehead, extending to the eyebrow in two patients and to the internal angle of the orbit in another. Pain was unilateral in 11 patients (six on the right, five on the left), and bilateral in four. Six patients had continuous pain and nine described intermittent pain. Palpation of the supratrochlear nerve at the medial third of the supraorbital rim resulted in hypersensitivity in all cases. All but one patient exhibited sensory disturbances within the painful area. Fourteen patients underwent anesthetic blockades of the supratrochlear nerve, with immediate relief in all cases and long-term remission in three. Six of them had received unsuccessful anesthetic blocks of the supraorbital nerve. Five patients were treated successfully with oral drugs and one patient was treated with radiofrequency. CONCLUSIONS: Supratrochlear neuralgia is an uncommon disorder causing pain in the medial region of the forehead. It may be differentiated from supraorbital neuralgia and other similar headaches and neuralgias based on the topography of the pain and the response to anesthetic blockade.

Greater occipital nerve block for the acute treatment of prolonged or persistent migraine aura.

Background Presently, there is no evidence to guide the acute treatment of migraine aura. We aimed to describe the effect of greater occipital nerve (GON) anaesthetic block as a symptomatic treatment for long-lasting (prolonged or persistent) migraine aura. Methods Patients who presented with migraine aura lasting > 2 hours were consecutively recruited during one year at the Headache Unit and the Emergency Department of a tertiary hospital. All patients underwent a bilateral GON block with bupivacaine 0.5%. Patients were followed up for 24 hours. Results A total of 22 auras were treated in 18 patients. Auras consisted of visual ( n = 13), visual and sensory ( n = 4) or sensory symptoms alone ( n = 5). Eleven episodes met diagnostic criteria for persistent aura (>1 week) without infarction. The response was complete without early recurrence in 11 cases (50%), complete with recurrence in < 24 hours in two cases (9.1%), and partial with ≥ 50% improvement in six cases (27.3%). Complete responses without recurrence were more common in cases with prolonged auras lasting < 1 week than in those with persistent auras (72.7% vs. 27.3%; p = 0.033). Conclusions GON block could be an effective symptomatic treatment for prolonged or persistent migraine aura. Randomised controlled trials are still required to confirm these results.

Short-term effects of greater occipital nerve blocks in chronic migraine: A double-blind, randomised, placebo-controlled clinical trial.

Background Greater occipital nerve (GON) blocks are widely used for the treatment of headaches, but quality evidence regarding their efficacy is scarce. Objective The objective of this article is to assess the short-term clinical efficacy of GON anaesthetic blocks in chronic migraine (CM) and to analyse their effect on pressure pain thresholds (PPTs) in different territories. Participants and methods The study was designed as a double-blind, randomised, placebo-controlled clinical trial. Thirty-six women with CM were treated either with bilateral GON block with bupivacaine 0.5% ( n = 18) or a sham procedure with normal saline ( n = 18). Headache frequency was recorded a week after and before the procedure. PPT was measured in cephalic points (supraorbital, infraorbital and mental nerves) and extracephalic points (hand, leg) just before the injection (T0), one hour later (T1) and one week later (T2). Results Anaesthetic block was superior to placebo in reducing the number of days per week with moderate-or-severe headache (MANOVA; p = 0.027), or any headache ( p = 0.04). Overall, PPTs increased after anaesthetic block and decreased after placebo; after the intervention, PPT differences between baseline and T1/T2 among groups were statistically significant for the supraorbital (T0-T1, p = 0.022; T0-T2, p = 0.031) and infraorbital sites (T0-T1, p = 0.013; T0-T2, p = 0.005). Conclusions GON anaesthetic blocks appear to be effective in the short term in CM, as measured by a reduction in the number of days with moderate-to-severe headache or any headache during the week following injection. GON block is followed by an increase in PPTs in the trigeminal area, suggesting an effect on central sensitisation at the trigeminal nucleus caudalis. This trial is registered at ClinicalTrials.gov (NCT02188394).

Living With Cluster Headache: A Qualitative Study of Patients' Perspectives.

OBJECTIVE: Our aim was to explore the views and experiences of a group of Spanish men suffering from cluster headache (CH). BACKGROUND: CH has considerable effects on patients' quality of life, impairs everyday activities, and can modify lifestyle. This is the first time the experience of patients with CH is examined in a clinical study using a qualitative, phenomenological approach. METHODS: We conducted a qualitative phenomenological study exploring how 20 male patients with CH, followed at the Headache Unit of a Spanish hospital, perceived their disease. Data were collected through in-depth interviews, researchers' field notes and patients' personal letters. A systematic text condensation analysis was performed following appropriate guidelines for qualitative research. RESULTS: Mean age was 41.15 years (standard deviation, 11.25). Seventeen patients had episodic CH and three patients had chronic CH. Five main themes describing the significance of suffering CH emerged: (a) meaning of disease, (b) experience of attacks, (c) meaning of treatment, (d) healthcare, and (e) social and family interaction. Patients with CH often live in fear and uncertainty because of their condition. Intensity and frequency of attacks, the use of ineffective treatments, skepticism perceived from social and workplace environments and physician unawareness play a significant role. CONCLUSIONS: Qualitative research offers insight into the way CH patients experience their disease, and may be helpful in establishing a fruitful relationship with these patients.

Uncrossed epileptic seizures in Joubert syndrome.

Joubert syndrome and related disorders comprise a subgroup of ciliopathies defined by the presence of the 'molar tooth sign', a midbrain-hindbrain malformation identifiable by neuroimaging. Characteristically, the corticospinal tract and superior cerebellar peduncles do not decussate. Epileptic seizures are uncommon. We present a case of a 28-year-old man with a background of Leber's congenital amaurosis with nephronophthisis, requiring kidney transplantation, and mental retardation, who developed epileptic seizures consisting of a short muffled cry and involuntary shaking movements of the extremities beginning in the left upper limb; these episodes lasted several seconds and occurred in clusters. Simultaneous video-EEG recording showed an ictal pattern in the left frontal lobe. Brain MRI revealed the pathognomonic 'molar tooth sign'; diffusion tensor imaging (DTI)-tractography showed a lack of decussation of both corticospinal tracts. To the best of our knowledge, this is the first time that DTI-tractography has been used to uncover the anatomical substrate underlying the semiology of epileptic seizures.

Superficial artery aneurysms underlying nummular headache--2 cases and proposed diagnostic work-up.

INTRODUCTION: Nummular headache, considered a primary headache in the 3rd edition of the International Classification of Headache Disorders, has nonetheless been attributed in several occasions to underlying epicranial anomalies. CASE REPORTS: Vascular imaging of the head in 2 patients with nummular headache revealed fusiform aneurysms of the scalp vessel in close relation to the painful area. One of the patients underwent surgical resection of the aneurysm with excellent response. A response to triptans was noted in both. DISCUSSION: In selected patients with nummular headache, vascular imaging of the scalp may reveal anomalies amenable to surgical treatment or triptan administration, sometimes resulting in disappearance of the pain.