A novel case of Horner syndrome as the presenting sign of craniosynostosis.

Craniosynostosis, the premature fusion of cranial sutures, can lead to distortion of skull shape and neurological dysfunction. We present a novel case of Horner syndrome as the presenting sign of craniosynostosis associated with elevated intracranial pressure. A 10-year-old boy presenting for strabismus follow-up was noted to have new-onset anisocoria, greater in the dark, and mild right upper eyelid ptosis. Apraclonidine testing was concerning for Horner syndrome. Neuroimaging demonstrated previously undiagnosed sagittal craniosynostosis with tortuous optic nerves and large cerebrospinal fluid spaces around both optic nerves. The patient was referred to neurosurgery and underwent a lumbar puncture with an opening pressure of 44 cm H2O. He underwent surgical cranial expansion. By six months postoperatively, his anisocoria had resolved.

Neurosarcoidosis with chronic cough and Horner's syndrome.

A 62-year-old man attended ophthalmology for a simple ptosis repair. He had a chronic cough, a Horner's syndrome with post-gustatory hyperhidrosis. He was referred to the respiratory and neurology teams. MR scan of his head and neck found evidence of multifocal disease at the skull base and carotid canal, and further tests identified additional deposits in the hilar lymph nodes, heart and sacrum. A transbronchial biopsy confirmed the diagnosis of sarcoidosis. His symptoms and imaging responded well to corticosteroids, but he still undergoes regular imaging. We discuss the features of Horner's syndrome, and the autonomic associations of a chronic cough.

Segmental Zoster Paresis Accompanied by Horner's Syndrome.

We herein report a 90-year-old immunocompromised woman who developed right upper limb weakness and right ptosis with a miotic pupil 1 week after oral therapy for zoster on the right T2 dermatome. The right pupil was dilated with instillation of 1% apraclonidine, indicating Horner's syndrome. The patient was treated with intravenous acyclovir and methylprednisolone. Focal weakness related to zoster, generally known as segmental zoster paresis, improved over five months, but Horner's syndrome remained. We suggest that aggressive intravenous treatment should be considered for rare cases of zoster that occur with a combination of these two neurological conditions.

Horner syndrome as a physiological biomarker of disease in canine cervical myelopathy.

BACKGROUND: Horner syndrome often occurs with cervical myelopathies and might provide insight into the underlying disease and prognosis. OBJECTIVES: To describe the clinical and imaging features of dogs with cervical myelopathy and concurrent Horner syndrome and to determine association of Horner syndrome with diseases or magnetic resonance images (MRI). ANIMALS: Ninety-three client-owned dogs with cervical myelopathy and concurrent Horner syndrome and 99 randomly selected client-owned dogs with cervical myelopathy without Horner syndrome (control cases). METHODS: Retrospective study. Medical records were reviewed to identify Horner and control cases and clinical findings recorded. MRI were reviewed, and lesions characterized and recorded. Descriptive and comparative statistics were performed. RESULTS: Non-compressive disease occurred more frequently in the Horner group compared with controls (58%; 95% CI: 48-68 vs 9%; 95% CI: 5-16; P < .0001). The most common diseases were fibrocartilaginous embolism in the Horner group (44/93; 47%) and intervertebral disc extrusion (76/99; 77%) amongst controls. On MRI, parenchymal hyperintensity was seen more commonly in the Horner group (95%; 95% CI: 88-98) compared with controls (51%; 95% CI: 41-60; P < .0001). In the Horner group, dogs that did not survive to discharge (N = 13) had more extensive MRI lesions relative to the adjacent vertebral length (200%; IQR 110%-575%) compared with survivors (N = 80; 110%; IQR 40%-250%; P = .02). Lateralization of Horner signs and MRI changes matched in 54% of cases. The overall survival rate was high in both Horner (80/93; 86%) and control (95/99; 96%) groups. CONCLUSIONS AND CLINICAL IMPORTANCE: Horner syndrome in cervical myelopathy is commonly associated with noncompressive intraparenchymal disease.

[Long-term results of carotid endarterectomy and carotid artery stenting in patients with high bifurcation of common carotid artery: a multiple-center study]. / Otdalennye rezul'taty karotidnoi endarterektomii i karotidnoi angioplastiki so stentirovaniem u patsientov s vysokim raspolozheniem bifurkatsii obshchei sonnoi arterii. Rezul'taty mnogotsentrovogo issledovaniya.

OBJECTIVE: To analyze in-hospital and long-term results of eversion carotid endarterectomy (CEE), carotid endarterectomy with patch repair and carotid artery stenting (CAS) in patients with high bifurcation of common carotid artery. MATERIAL AND METHODS: A retrospective multiple-center open study included 1983 patients who underwent internal carotid artery (ICA) repair for severe stenosis between 2010 and 2021. Three groups of patients were distinguished depending on revascularization option: group 1 (n=638) - eversion CEE; group 2 (n=351) - CEE with patch repair; group 3 (n=994) - CAS. RESULTS: In-hospital postoperative mortality and incidence of stroke and myocardial infarction were similar. All bleedings (n=39) occurred after CEE. ICA thrombosis was diagnosed in groups 1 and 2 due to intimal detachment after insertion of temporary bypass tube. Incidence of laryngeal paresis, neuropathy of hypoglossal and glossopharyngeal nerves, Horner syndrome, damage to salivary glands was comparable in groups 1 and 2. Long-term mortality was the highest (n=10; 2.8%) after CEE with patch repair due to fatal stroke. In turn, the highest incidence of ICA restenosis and restenosis-induced ischemic stroke was observed after CEE with patch repair and CAS. CONCLUSION: 1. Classical and eversion CEE in patients with high CCA bifurcation is followed by high in-hospital incidence of damage to cranial nerves and salivary glands, laryngeal paresis, Horner syndrome, bleeding and risk of ICA thrombosis. 2. In patients with high CCA bifurcation, CAS and CEE with patch repair are accompanied by high incidence of ICA restenosis, restenosis-induced stroke and mortality in long-term postoperative period. 3. Eversion CEE demonstrates the lowest rates of all adverse cardiovascular events in long-term follow-up period.

Horner Syndrome in Giant Cell Arteritis: Case Series and Review of the Literature.

BACKGROUND: Giant cell arteritis (GCA) is a systemic inflammatory vasculitis that affects medium- and large-sized arteries and can result in permanent vision loss. In rare instances, Horner syndrome has been noticed at the time of GCA diagnosis, although the mechanism of both diagnoses occurring at the same time is not entirely understood. We reviewed 53 charts of all patients diagnosed with biopsy-proven GCA in tertiary neuro-ophthalmology practice to find patients who presented with new onset of Horner syndrome at the time of GCA diagnosis. METHODS: Two patients with biopsy-confirmed GCA who presented with concurrent Horner syndrome were found. Data on age, sex, and ophthalmic and neuroradiologic examination findings were collected. RESULTS: Patient 1 was a 67-year-old man who presented with new onset of vertical binocular diplopia and was diagnosed with right fourth cranial nerve palsy. He then developed left ptosis and miosis, and was diagnosed with Horner syndrome by pharmacologic testing. He also had persistently elevated inflammatory markers. Patient 2 was a 71-year-old man who presented with new onset of binocular vertical diplopia, bitemporal headaches, and jaw ache. Both of his inflammatory markers were elevated. On examination, he had left ptosis and myosis, and small comitant left hypertropia. The diagnosis of left Horner syndrome was confirmed on pharmacologic testing and left hypertropia was attributed to skew deviation. Both patients underwent temporal artery biopsy, which confirmed the diagnosis of GCA. Treatment with high dose of oral corticosteroids commenced, and vertical diplopia has completely resolved in both patients. Horner syndrome persisted in Patient 1 and resolved in Patient 2. MRI and MR angiography of the brain and neck were unrevealing in both patients. CONCLUSIONS: This case series describes 2 patients with new diagnosis of GCA and concurrent Horner syndrome, with new diagnosis of likely nuclear/fascicular fourth nerve palsy in one patient and skew deviation in the other. In both patients, vasculitis presumptively affected vertebral arteries and their branches supplying the first-order sympathetic neurons in the brainstem. Considering the severe complication of permanent vision loss in GCA, this diagnosis should be considered in older patients presenting with concurrent new onset of Horner syndrome.

Redo surgery for neurogenic thoracic outlet syndrome is useful.

OBJECTIVES: Surgery for neurogenic thoracic outlet syndrome (NTOS) has shown good outcome in numerous case series. However, 5% to 30% of patients will have persistent or recurrent symptoms, caused by incomplete first rib resection, reattachment of residual scalene muscle, fibrous scarring around the brachial plexus, or a wrong NTOS diagnosis. In patients with a sound diagnosis of recurrent or persisting NTOS, not responding to conservative measures, a secondary procedure can be considered. We report the results of redo thoracic outlet decompression surgery through the supraclavicular approach (SC-REDO-TOD) for persistent or recurrent NTOS. METHODS: A retrospective review of a prospective database was performed. Every patient referred from September 2016 until January 2020 was eligible for inclusion. In an SC-REDO-TOD, we perform complete (cartilage-cartilage) resection of the first rib, any bony and fibrous anomalies, complete anterior and middle scalenectomy, and complete neurolysis of the brachial plexus (complete anatomical decompression of the brachial plexus). Clinical outcomes were assessed by questionnaires including the Disability of Arm, Shoulder and Hand (DASH), Cervico-Brachial Symptoms Questionnaire (CBSQ), and TOS (thoracic outlet syndrome) Disability scale. RESULTS: In total, 45 patients had a SC-REDO-TOD. The median duration of hospital admission after SC-REDO-TOD was 1.41 days (interquartile range, 1.00 day). In total, 30 (66.66%) of 45 patients had recurrent NTOS, and 15 (33.33%) of 45 patients had persisting NTOS. Postoperative complications were seen in eight patients (18.18%). One patient had postoperative complications with permanent impairment (Horner syndrome). Seven patients had postoperative complications with full recovery (three patients had a chylous leakage that was treated with a median-chain triglycerides diet for 6 weeks, three patients had transient phrenic nerve palsy with full recovery <6 weeks, and one patient had a discrete Horner syndrome that resolved in 6 weeks). The median time of follow-up was 19.50 months (interquartile range, 14.00 months) and the response rate to the questionnaires was 91.11% at 6 months and 64.44% at 12 months. We found a positive and statistically significant difference for DASH score, CBSQ score, and TOS Disability Scale score comparing scores for all patients. (DASH score: P < .001; CBSQ score: P < .001; TOS Disability Scale: P < .001). Patients with first rib remnants showed a significant better response (lower DASH, CBSQ and TOS Disability Scale scores) compared with patients without first rib remnants (DASH score: P = .004; CBSQ score: P ≤ .014; TOS Disability Scale: P = .009). CONCLUSIONS: SC-REDO-TOD after a previous NTOS surgery shows good results with a low risk of permanent impairment. Patients with NTOS with first rib remnants after primary surgery seem to benefit the most from SC-REDO-TOD surgery.

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.

Autonomic dysreflexia and concurrent Horner's Syndrome: a rare presentation in a patient with spinal cord injury.

INTRODUCTION: Autonomic dysreflexia is an uninhibited sympathetic response evoked by a strong sensory input below the level of the injury in patients with spinal cord injury. As presented in this case, autonomic dysreflexia can be associated with unusual symptoms such as Horner's syndrome. CASE PRESENTATION: An 18-year-old man with a traumatic spinal cord injury (C7 AIS A) experienced symptoms of unilateral Horner's syndrome: miosis, ptosis and anhidrosis which occurred simultaneously with symptoms of autonomic dysreflexia: severe headache accompanied by increasing right-sided diaphoresis, flushing, blurred vision, and increased blood pressure. These symptoms were triggered by bladder distention and were resolved after catheterisation. DISCUSSION: The patient experienced a transient Horner's syndrome due to autonomic dysreflexia. Both Horner's syndrome and symptoms of autonomic dysreflexia resolved when eliminating the eliciting stimulus, indicating that Horner's syndrome occurred due to a transient pressure on the sympathetic fibres supplying the superior cervical ganglion. Autonomic dysreflexia may have caused increased pressure disrupting the sympathetic input, thus inducing unilateral miosis, ptosis, and facial anhidrosis.

First Bite Syndrome: What Neurologists Need to Know.

PURPOSE OF REVIEW: Though first bite syndrome is well known in surgical settings, it is not commonly included in the differential for sharp paroxysmal facial pain in the neurology literature. This paper will highlight the clinical features and relevant anatomy of first bite syndrome, with the goal of helping clinicians differentiate this from other similar facial pain disorders. RECENT FINDINGS: First bite syndrome is severe sharp or cramping pain in the parotid region occurring with the first bite of each meal and improving with subsequent bites. Pathophysiology has been attributed to imbalanced sympathetic/parasympathetic innervation of the parotid gland. This is seen most typically in the post-surgical setting following surgery in the parotid or parapharyngeal region, but neoplastic etiologies have also been reported. It is common for patients to present with concurrent great auricular neuropathy and/or Horner's syndrome. Evidence regarding treatment is limited to case reports/series, however, botulinum toxin injections and neuropathic medicines have been helpful in select cases. It is critical for clinicians to be able to differentiate first bite syndrome from other paroxysmal facial pain. To help with this, we have proposed diagnostic criteria for clinical assessment. Patients often improve gradually over time, but symptomatic treatment with botulinum toxin or neuropathic medicine may be required.

Four common diseases causing sudden blindness or death in the eye emergency department.

Neuro-ophthalmological emergency disorders typically present with symptoms of visual loss, diplopia, ocular motility impairment or anisocoria. The ocular manifestations of these disorders are sometimes indicative of a more serious global neurology disease rather than an isolated ocular disease. The aim of this review is to highlight four important neuro-ophthalmological emergency disorders that must not be missed by an ophthalmologist. These include acute painful Horner's syndrome, painful cranial nerve III palsy, giant cell arteritis and transient ischaemic attack with amaurosis fugax. The delayed diagnosis of these clinical entities puts the patient at risk of blindness or death. Therefore, prompt diagnosis and management of these conditions are essential. This can be acquired from understanding the main signs and symptoms of the disease presentation together with a high index of suspicion while working at a busy eye emergency department.

Heterocromia de íris: uma revisão das condições que podem afetar a pigmentação iridiana / Heterochromia: a review of conditions that may affect iris pigmentation

RESUMO A íris é responsável pela cor dos olhos. Ela ainda realiza o controle da quantidade de luz que penetra no olho pela pupila. Variações nos genes de cada indivíduo, além da quantidade e da qualidade de melanina na íris, determinam a cor dos olhos. A heterocromia é caracterizada por diferenças na coloração da íris de um mesmo indivíduo, sendo, na maioria das vezes, benigna. Existem basicamente três tipos de heterocromia de íris: central, setorial e completa. A heterocromia de íris pode ter como causa alterações genéticas e congênitas, relacionadas ou não a síndromes específicas, como a de Sturge-Weber, a de Waardenburg, a de Parry-Romberg e a de Horner congênita. Há também causas adquiridas, como doenças ou lesões, trauma ocular e corpos estranhos intraoculares, uso de certas medicações tópicas, siderose ocular, irites ou uveítes como a síndrome uveítica de Fuchs, dentre outras. Diante de um paciente com heterocromia de íris, deve-se entender o contexto e o curso clínico desse sinal, pois pode se tratar de uma alteração de pigmentação benigna ou existir uma doença base em curso, que requer terapêutica específica. Este artigo de revisão de literatura visa abordar as principais etiologias relacionadas à heterocromia de íris, além de discorrer sobre a anatomia e a fisiologia da coloração iridiana e sobre a fisiopatologia de suas possíveis alterações.

ABSTRACT The iris is responsible for eye color and controls the amount of light that enters the eye through the pupil. Variation in each individual's genes, besides the quantity and quality of melanin in the iris, determine eye color. Heterochromia is characterized by different colors of irises in the same individual, and it is benign in most cases. There are basically three types of heterochromia: central, partial and complete. Heterochromia can be caused by genetic and congenital alterations, which may or may not be related to specific conditions, such as Sturge-Weber syndrome, Waardenburg syndrome, Parry-Romberg syndrome and congenital Horner syndrome. It may be associated to acquired causes like diseases or injuries, such as eye trauma and intraocular foreign bodies, use of some topical medications, ocular siderosis, iritis or uveitis, such as Fuchs´ uveitis, among others. When assessing a patient with heterochromia, one must understand the context and clinical course of this signal, since it may be a benign pigmentation disorder or there may be an underlying disease, which requires specific therapy. This literature review article was set out to address the main etiologies related to heterochromia, in addition to describing the anatomy and physiology of the iris color and the pathophysiology of possible alterations.

Horner Syndrome With Ipsilateral Wing Paresis in a Wild, Juvenile Yellow-Tailed Black Cockatoo (Calyptorhynchus funereus).

A juvenile yellow-tailed black cockatoo (Calyptorhynchus funereus) was presented with paresis of the right wing, ptosis, and miosis of the right eye; feather erection of the right side of the head and neck; and a penetrating injury over the right pectoral muscle. Temporary reversal of ptosis, miosis, and feather erection after administration of phenylephrine drops confirmed a diagnosis of Horner syndrome. Computed tomographic imaging revealed a fractured rib, traumatic lung lesions, and subcutaneous emphysema. The right-sided Horner syndrome and wing paresis were attributed to a sympathetic nerve trauma of the eye and feathers and to the brachial plexus, respectively. This report describes the diagnosis and resolution of ptosis and miosis within 8 weeks and recovery of feather symmetry and wing function within 11 weeks of the cockatoo's initial presentation with a conservative-management treatment plan.