Harlequin syndrome in a patient with probable hemicrania continua and exertional headache - is there a link? a case report.

BACKGROUND: The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported. CASE PRESENTATION: This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome. CONCLUSIONS: This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.

Cortico-hypothalamic pathway of Horner syndrome derived from isolated lenticulostriate stroke.

BACKGROUNDS: Horner syndrome presents with ipsilateral ptosis, miosis, and anhidrosis due to interruption of the oculosympathetic pathway. Patients with acute ischemic stroke may present with Horner syndrome, which may help locate the lesion. However, the underlying pathways involved in Horner syndrome caused by isolated lenticulostriate ischemic stroke remain unclear. METHODS: We screened consecutive patients with acute ischemic stroke admitted to the Second Affiliated Hospital of Guangzhou Medical University from 1 January 2020 to 31 December 2021, and searched for cases of isolated lenticulostriate strokes presenting with Horner syndrome. Strokes involving the brainstem or hypothalamus, or those caused by carotid dissection or carotid cavernous fistula were excluded based on neuroimaging and cerebrovascular examination. RESULTS: Among the 1706 acute stroke patients, three patients developed temporary or long-term Horner syndrome due to an ipsilateral lenticulostriate ischemic lesion. Diffusion-tensor imaging revealed disruption of an uncrossed pathway from Brodmann areas 3, 1, and 2 through the basal ganglia to the ipsilateral hypothalamus. CONCLUSION: These findings suggest that Horner syndrome may be due to a disruption of an uncrossed cortico-basal ganglia-hypothalamic sympathetic pathway.

Harlequin syndrome.

A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described headache as sharp, stabbing pain around the left eye associated with nausea, vomiting, photophobia, and phonophobia lasting for hours associated with flushing on the left side of the face. The picture of his face during these episodes showed flushing of the left side of the face, ptosis of the right eyelid, and miosis (panel A). Flushing in his face would resolve with the abortion of the headache. At the time of presentation to the clinic, his neurological exam was only significant for mild left eye ptosis and miosis (panels B and C). Extensive workup including MRI brain, cervical spine, thoracic spine, lumbar spine, CTA head and neck, and CT maxillofacial was unremarkable. He had tried several medications in the past including valproic acid, nortriptyline, and verapamil without significant benefit. He was started on erenumab for migraine prophylaxis and was given sumatriptan for abortive therapy following which his headaches improved. The patient was diagnosed with idiopathic left Horner's syndrome and his migraines with autonomic dysfunction would present with unilateral flushing opposite to the site of Horner's presenting as Harlequin syndrome [1, 2].

Multifocal neuropathy and Horner's syndrome due to disseminated pyomyositis in an intravenous drug user.

BACKGROUND: The formation of abscesses with necrosis within large, striated muscles leads to pyomyositis, a condition relatively rarely encountered outside the tropics. Intravenous drug users and other immunocompromised individuals are predisposed toward this infection, which may occur due to local or haematogenous spread of infection to skeletal muscles previously damaged by trauma, exercise, or rhabdomyolysis. METHODS: We report a young male intravenous drug user with rhabdomyolysis due to use of a synthetic opioid, in whom disseminated pyomyositis was detected following evaluation for sciatic and radial neuropathies and Horner's syndrome and review available reports of peripheral nerve dysfunction in the setting of this uncommon infection. We searched online databases to identify all published reports on adult patients with pyomyositis complicated by peripheral nerve dysfunction. CONCLUSIONS: Peripheral nerve dysfunction may rarely occur via local spread of infection or compression from abscesses.

Horner Syndrome subsequent to ultrasound-guided cervical lymph node fine-needle aspiration - A case report and literature review.

Horner Syndrome (HS) is characterized by symptoms of ipsilateral miosis, ptosis, enophthalmos, and facial anhidrosis, which is caused by the damaged oculosympathetic pathway. HS is rarely reported as postoperative complications of fine-needle aspiration (FNA). We report a case of HS triggered by Ultrasound-guided FNA during thyroid cancer management and conducted the literature review. A 31-year-old male with differentiated thyroid cancer underwent total thyroidectomy and regional lymph node dissection as well as radioactive iodine ablation, presented with persistently elevated tumor marker of thyroglobulin and suspicious left level IV and V cervical lymph nodes by neck ultrasound. Ultrasound-guided left cervical lymph nodes FNA for cellular diagnosis was performed, and typical manifestations of HS appeared immediately after the procedure. Subsequent ultrasound evaluation of the same area demonstrated a subtle strip of the hypo-echogenic area in the superior pole of the suspected level IV structure, suggesting sympathetic ganglia with the visible originating nerve fiber on the superior pole. All of the patient's symptoms of HS were resolved 2 months after the incidence. Cervical sympathetic ganglia can be similar in size, shape, and ultrasound characteristics to a malignant lymph node. Thorough ultrasound examination by directly comparing the potential ganglia with a typical malignant lymph node, and paying attention to any potential root fibers on the target is key to avoiding ganglia injury before the neck invasive procedures.

[Horner's syndrome in the delivery room : a rare complication of labour epidural analgesia]. / Cas clinique. Syndrome de Claude Bernard Horner en salle de travail : une complication rare de l'analgésie péridurale obstétricale.

Horner's syndrome is a rare and benign complication of neuraxial analgesia during labour. We report the case of a Gravida 1 patient complaining of ptosis, enophthalmia and meiosis during labour, a few hours after a lumbar epidural analgesia was initiated.

Le syndrome de Claude Bernard Horner est une complication rare et bénigne de l'analgésie neuraxiale pour le travail obstétrical. Nous présentons ici le cas d'une patiente primigeste présentant une symptomatologie associant ptosis, énophtalmie et myosis en cours de travail, quelques heures après l'initiation d'une analgésie péridurale lombaire.

Horner syndrome after thyroid-related surgery: a review.

BACKGROUND: Horner syndrome (HS) is caused by damage to the cervical sympathetic nerve. HS is a rare complication after thyroidectomy. The main manifestations of HS include miosis and ptosis of the eyelids, which seriously affect esthetics and quality of life. At present, there is a lack of research on HS after thyroidectomy, and its etiology is not completely clear. This review aimed to evaluate how to reduce the incidence of HS and promote the recovery from HS as well as to provide a reference for the protection of cervical sympathetic nerves during surgery. RESULTS: HS caused by thyroid surgery is not particularly common, but it is still worthy of our attention. After searching with "Horner Syndrome," "Thyroid" as keywords, a total of 22 related cases were screened in PubMed. The results showed that open surgery, endoscopy, microwave ablation, and other surgical methods may have HS after operation. In addition, the statistics of 1213 thyroid surgeries in our hospital showed that the incidence of HS after endoscopic surgery (0.39%) was slightly higher than that after open surgery (0.29%). Further, this review analyzed potential causes of HS after thyroidectomy, so as to provide a theoretical basis for reducing its incidence. CONCLUSION: Preventing HS during thyroidectomy is a difficult problem. The close and highly variable anatomical relationship between the thyroid and cervical sympathetic nerves increases the risk of sympathetic nerve damage during thyroidectomy. Surgery and the use of energy equipment are also closely related to the occurrence of HS.

Spontaneous spinal epidural hematoma in an infant presenting with Horner syndrome.

BACKGROUND: Spontaneous spinal epidural hematoma (SSEH) is a rare neurologic entity, especially in infants, that develops in the absence of underlying coagulopathy, bleeding diathesis, infection, vascular malformation, trauma, iatrogenic, or other identifiable cause. In contrast to adults, diagnosis is frequently delayed or missed in infants due to non-specific symptoms and limited clinical examination. CASE ILLUSTRATION: An 11-month-old female demonstrated symptoms of irritability, intermittent diarrhea, lethargy, decreased oral intake, and difficulties crawling before presenting to the emergency room. At time of presentation, she was noted to have minimal spontaneous movement of the lower extremities and anisocoria with ptosis of the right eye. Given her clinical presentation, a magnetic resonance image (MRI) of the spine was obtained which revealed an epidural hematoma with compression extending from C7-T3. She underwent C7-T3 laminoplasty and hematoma evacuation. Following surgical intervention, she demonstrated significant improvements in her lower extremity strength and resolution of Horner syndrome. CONCLUSION: SSEH in infants is a rare neurologic condition, with diagnosis often delayed due to nonspecific symptomatology. Prompt diagnosis and intervention are essential in the treatment of SSEH to prevent permanent neurologic dysfunction. Physicians should have a high index of suspicion for SSEH in these instances, and investigation with spinal MRI imaging is recommended.

The diagnostic yield of advanced imaging in dogs with Horner's syndrome presenting with and without additional clinical signs: A retrospective study of 120 cases (2000-2018).

PURPOSE: To evaluate the diagnostic yield of advanced imaging in dogs with Horner's syndrome (HS) both with and without additional clinical signs. METHODS: Retrospective review of clinical records from a UK referral hospital (2000-2018). Cases were excluded if HS was trauma- or surgery-related and if no advanced imaging with MRI or CT was performed. Imaging findings were assessed as well as any additional investigations performed. RESULTS: One hundred twenty cases met the inclusion criteria; 88 with additional clinical signs and 32 with isolated HS. MRI or CT of head and neck was performed in 115 cases and/or thoracic CT in eight cases. Causative lesions were identified in 98% (86/88) of cases with additional clinical signs and 3% (1/32) of cases with isolated HS. This was statistically significant (p < .001). Pharmacological localization using phenylephrine (0.1%-10%) was accurate in predicting the site of a causative lesion in 79% (19/24) of tested cases. Overall, 28% (33/120) of cases were idiopathic, of which 49% (16/33) were golden retrievers. When compared to the overall hospital population, and UK Kennel Club records, this confirmed a significant predisposition in this breed (p < .001). No associations between idiopathic HS and concurrent systemic disease were identified. CONCLUSION: This is the first study to evaluate the diagnostic yield of advanced imaging in dogs with HS. Isolated HS is significantly more likely to be idiopathic than cases presenting with additional clinical signs; this could guide decision-making regarding further investigations performed in a clinical setting. Idiopathic HS is over-represented in the golden retriever.

The evaluation of patient demographics, etiologies and apraclonidine test results in adult Horner's syndrome.

PURPOSE: We aimed to demonstrate the patient demographics, etiologies and apraclonidine test results in adult Horner's syndrome. METHODS: This retrospective study was performed by the analysis of medical data of patients who were given 0.5% apraclonidine test. Patients' past medical history, demographic data, etiologies, accompanying neurological findings and pharmacological test results were assessed. RESULTS: Forty patients (21 females and 19 males) with a mean age of 50.3 ± 11.6 years were evaluated. Apraclonidine 0.5% test was positive in 37 patients (92.5%). An etiology could be identified in 20 patients (central [9 patients, 45%], preganglionic [9 patients, 45%] and postganglionic [2 patients, 10%]). Neurological findings accompanying Horner's syndrome were present in 8 patients. CONCLUSION: Despite detailed investigations, in a significant number of patients with Horner's syndrome an underlying cause may not be detected. Among the identifiable lesions, central and preganglionic involvements are still the first leading causes of Horner's syndrome. In addition, apraclonidine test may not be positive in all patients and a negative response does not exclude Horner's syndrome.

Acute Painful Horner's Syndrome Revealing Eagle's Syndrome: A Report of Two Cases.

Eagle's syndrome (ES) is characterised by an abnormally elongated styloid process. It remains a complex pathology and the ophthalmological symptoms are relatively rare. Herein, we report two cases who presented to our clinic with unilateral and painful Horner's syndrome. Computed tomography scanning showed the presence of ES with unilateral extrinsic compression of the sympathetic plexus by the styloid.

Over a decade of single-center experience with thoracoscopic sympathicolysis for primary palmar hyperhidrosis: a case series.

BACKGROUND: Primary palmar hyperhidrosis is a severely debilitating condition that can affect patients of any age. We report our experience with thoracoscopic sympathicolysis in a large cohort of children less than 14 years of age. METHODS: All children who underwent thoracoscopic sympathicolysis from April 2005 through January 2017 were evaluated retrospectively. The procedure entailed bilateral bipolar fulguration of the second and third thoracic ganglia with transverse disruption of collateral nerve fibers along the third and fourth rib. Demographic information, as well as postoperative outcome, complications, and satisfaction were analyzed. RESULTS: Over the 12 year study interval, a total of 102 children underwent thoracoscopic sympathicolysis for palmar hyperhidrosis. Complete follow-up was available for 98 patients (median age 12 [range 5-14] years; 38 boys [39%]). Median follow-up was 4 [range 2-12] years. Complete palmar dryness was achieved in 93 (95%) cases. One patient suffered postoperative unilateral ptosis, 6 reported gustatory sweating, and 65 experienced compensatory sweating. Average postoperative rating on a 1 (lowest) to 10 (highest) rating scale was 9, with 97 (99%) patients saying that they would undergo the procedure again. CONCLUSION: Our technique of thoracoscopic sympathicolysis in children was associated with very high postoperative satisfaction, despite a high rate of compensatory sweating and occasional autonomic gustatory sweating. Other more severe complications in this age group were rare.

Harlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome.

A 1-year-old boy presented with a 4-month history of hypertension, ptosis of the right upper eyelid, left hemifacial sweating, and flushing. He was diagnosed with Harlequin syndrome associated with Horner syndrome. Computed tomography revealed a mass lesion in the right superior mediastinum. Therefore, the patient underwent total tumor resection. Histological examination demonstrated ganglioneuroblastoma. The MYCN oncogene was not amplified, and the mitosis-karyorrhexis index was low. Accordingly, radiation and chemotherapy were not performed. No recurrence was observed within 8 months after surgery, and the patient's blood pressure was normalized. However, the ptosis, hemifacial sweating, and flushing persisted.

Imaging of Horner syndrome in pediatrics: association with neuroblastoma.

Neuroblastoma is the most common neoplasm associated with pediatric Horner syndrome. The laboratory and imaging evaluation of isolated pediatric Horner syndrome is controversial. We review the literature published in the last several decades and present the rationale for the imaging work-up in this patient cohort.

Case report and literature review: Horner syndrome subsequent to endoscopic thyroid surgery.

BACKGROUND: Horner syndrome (HS), mainly characterized by symptoms including ptosis, miosis, and anhidrosis on the affected face, is a condition that is well documented but rarely reported as a postoperative complication of thyroidectomy, particularly in endoscopic thyroid surgery (ETS). We hereby report a case of HS due to ETS with a brief literature review on this topic. CASE PRESENTATION: A 31-year-old female was admitted to our hospital with an unexpected physical examination finding of two thyroid nodules that were hypoechoic, had an irregular shape, and exhibited calcification. Subsequently, the results of a fine-needle aspiration (FNA) biopsy from the thyroid nodules and BRAFV600E mutation further confirmed the malignancy of these nodules. Thus, total thyroidectomy combined with central lymph node dissection (CLND) by ETS via the bilateral axillo-breast approach was performed on this patient. Histology confirmed the diagnosis of papillary thyroid microcarcinoma (PTMC) concurrent with Hashimoto's thyroiditis (HT). However, this patient developed HS with ptosis in her left eye on postoperative day 3. All symptoms gradually resolved before the 3-month follow-up. CONCLUSION: HS subsequent to ETS is a rare complication. Thus, standardized and appropriate operative procedures, as well as subtle manipulation, are essential in preventing and reducing the occurrence of HS. In addition, the early diagnosis and management of this rare complication are also important for a favorable outcome.

High thoracic ossification of ligamentum flavum causing partial Horner syndrome.

We report a case of high thoracic ossification of the ligamentum flavum (OLF) causing a partial Horner's syndrome. A 57-year-old man developed a walking disorder, as well as right-sided miosis and anhidrosis. Magnetic resonance imaging demonstrated a spinal cord compressing T2-T3 OLF. The patient improved after surgery.

Neurosurgical management of vascular compression presenting as visual symptoms secondary to elongated styloid processes (Eagle syndrome).

BACKGROUND: Eagle syndrome (ES) was first described in 1937, to characterize elongation of the styloid process. It is rarely encountered by neurosurgeons but does present the potential for vascular sequelae and neurological complications. DEMONSTRATIVE CASES: We discuss three patients with uncommon presentations of neurovascular compromise with uncommon symptomatology, secondary to ES. Their management ranged from retrospective diagnoses following self-limited events, antiplatelet therapy, and endovascular and surgical interventions. DISCUSSION: While traumatic fractures, chiropractic manipulation, and history of prior neck surgery have been implicated as the etiologies for ES, congenital cases are common. The styloid process intimately interplays with adjacent neurovascular and nervous structures; its elongation can cause symptoms in 10% of patients. CONCLUSION: Awareness of this potentially dangerous but rare disease - more commonly seen by our otolaryngology colleagues - may help reduce diagnostic delays when an elongated styloid process is the cause, as surgery may be required.

Radiological evaluation of the localization of sympathetic ganglia in the cervical region.

PURPOSE: To determine local variations of cervical sympathetic ganglia (CSG) according to vertebral levels on preoperative neck magnetic resonance imaging (MRI) by designating carotid artery (CA) as the standard landmark at the center, in attempts to prevent injury to CSG in the anterior-anterolateral approaches performed in the cervical spinal region. MATERIALS AND METHODS: The retrospective study reviewed neck MRI images of 281 patients, of which the images of 231 patients were excluded from the study based on the exclusion criteria. As a result, the MRI images of the remaining 50 patients were included in the study. The circumference of carotid artery (CA) was divided into eight equal zones with CA defined as the standard landmark at the center. High-risk zones were determined based on the anterior-anterolateral approaches. RESULTS: At C1 level, a superior ganglion was located on the right side in 32 (64%) and on the left side in 30 (60%) patients. At this level, it was most commonly located in Zone 6. Middle ganglion was observed most frequently at C3 level, which was detected on the right side in 17 (34%) and on the left side in 17 (34%) patients. At this level, it was most commonly located in Zone 2. CONCLUSION: Variations in the localizations of superior and middle cervical ganglia should be taken into consideration prior to surgical procedures planned for this region. This study sheds light on high-risk zones in the surgical site and could guide surgeons to better understand the location of cervical sympathetic ganglia before surgical planning.

[Marginal zone lymphoma associated with Reed-Sternberg cells: A challenge for the pathologist]. / Lymphome de la zone marginale ganglionnaire associé à des cellules de Reed-Sternberg : un challenge pour le pathologiste.

We report the case of a 46-year-old male patient presenting with a Claude Bernard-Horner Syndrome. Clinical evaluation showed a clonal B-cell population, lambda restricted. PET-scan captured femoral and axillary lymph nodes. Therefore the diagnosis of a marginal zone lymphoma was posted for which an attitude of watchful waiting was suggested. Eighteen months later, the patient developed an inguinal adenopathy. This lymph node led to the diagnosis of a nodular sclerosing Hodgkin lymphoma. Initial treatment with ABVD showed a good response, but the patient relapsed after eight months. A second biopsy confirmed the diagnosis of a marginal zone lymphoma but also identified giant Reed-Sternberg cells, (CD15+, CD30+ and CD20+). The initial biopsy was revised. This last diagnosis of a nodal marginal zone lymphoma with presence of Reed-Sternberg cells is rarely described in the literature. Several scientific theories can be found. Some cases described a transformation of non-Hodgkin lymphoma that presented Reed-Sternberg cells, other cases mentioned a collision or composite tumor. An accidental finding of Reed-Sternberg cells can be seen by after an infectious disease such as EBV. The presence of only Reed-Sternberg cells in a non-Hodgkin lymphoma is not sufficient to make a diagnosis of collision tumor.

Recurrent Fevers and Neuro-ophthalmic Disorders in a Mathematical Genius.

Horner's syndrome coexisting with an ipsilateral fourth cranial nerve palsy is a rare occurrence and likely localises to pathology in the cavernous sinus. One such case may have occurred in the 18th century affecting the renowned mathematician Leonhard Euler. A review of his biographies, eulogies, and three finely detailed facial portraits suggest that these two neuro-ophthalmic conditions, along with visual loss and a decades-long intermittent febrile illness, may have been the result of an orbital cellulitis and septic cavernous sinus thrombosis, from an underlying chronic brucellosis infection.