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.

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.

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.

Congenital Horner and Scimitar syndrome in a newborn: a previously unreported combination.

Here we report a case of a term newborn presenting with left palpebral ptosis, anisocoria and heterochromia as well as cleft palate and heart murmur. Congenital Horner syndrome was suspected and a thoracoabdominal CT scan was performed to rule out neuroblastoma. This revealed an anomalous drainage of right pulmonary veins to a collector that drains to the inferior vena cava, leading to the diagnosis of Scimitar syndrome. Echocardiogram showed an ostium secundum atrial septal defect, enlarged right chambers and a dilated coronary sinus due to a persistent left superior vena cava. The combination of Horner and Scimitar syndrome has never been described before. This case should encourage clinicians to use a multidisciplinary approach in order to guarantee an adequate diagnosis and management.

Nasopharyngeal carcinoma presenting as a sixth nerve palsy and Horner's syndrome.

The combination of a sixth nerve palsy and ipsilateral Horner's syndrome localises the disease process to the posterior cavernous sinus and can be a result of various pathologies in this region. A 74-year-old Chinese woman presented with a 9-month history of binocular horizontal diplopia worse when looking left. She was found to have a left sixth nerve palsy and Horner's syndrome and MRI revealed an enhancing soft tissue mass in the nasopharynx with involvement of the bones of the skull base and invasion of the left cavernous sinus. Endoscopic biopsy of the mass confirmed the diagnosis of non-keratinising squamous cell carcinoma, which was Epstein-Barr virus positive. She was treated with radiation therapy. Patients with a sixth nerve palsy and ipsilateral Horner's syndrome should have urgent neuroimaging with careful attention to the cavernous sinus since sympathetic fibres join the sixth nerve for a short distance in this location.

Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant.

A 51-year old presented with a 6-month history of increasing pelvic/lower back pain with nocturnal waking and episodes of anorexia and vomiting. Examination revealed right torticollis and Horner's syndrome, and a large abdominal mass arising from the pelvis. Magnetic resonance and positron emission tomography imaging revealed (A) a 14 cm heterogeneous enhancing mass, abutting the left kidney with standardised uptake value max = 2.9, (B) a large heterogeneous enhancing pelvic mass (C) mesenteric adenopathy standardised uptake value max = 10.3 and (D) 6 cm right lung apex mass standardised uptake value max = 4.3. Computerised tomography-guided biopsy of lesion A was reported as neurofibroma with occasional atypia, lesion B a benign uterine leiomyoma and lesion C follicular lymphoma world health organisation Grade 2. Although she had been given the diagnosis of Neurofibromatosis Type-1 (NF1) 25-years previously following removal of an intradural extramedullary schwannoma she had no cutaneous stigmata of NF1. Genetic analysis of blood lymphocyte DNA identified a pathogenic variant in SMARCB1 confirming a diagnosis of schwannomatosis. Following 6-months chemotherapy for lymphoma, surgery was performed to remove lesion A. Histology revealed a malignant peripheral nerve sheath tumour with areas of low and high-grade change. An incidental, well-differentiated small bowel neuroendocrine carcinoma was also excised. Close surveillance continues with no recurrence after 6 years. This case study describes a novel finding of three separate synchronous primary malignancies in a patient with schwannomatosis and a proven SMARCB1 pathogenic variant.

A rare cause of Horner's syndrome: cervicothoracic spinal root cysts.

OBJECTION: We herein report cervicothoracic spinal root cysts as a rare cause of Horner's syndrome. CASE REPORT: A 62-year-old woman was admitted to our neuro-ophthalmology clinic complaining of drooping of her right upper eyelid. The history, physical examination, and laboratory tests were normal. The extraocular movements were full. The right eyelid was ptotic and the right pupil was smaller than the left. Right Horner's syndrome was diagnosed by the neuro-ophthalmologic examination and pharmacological tests. Cervical magnetic resonance imaging showed multiple spinal nerve root cysts between C7 and T1 segments of the spinal cord. CONCLUSION: This report showed for the first time that cervicothoracic spinal root cysts could cause Horner's syndrome and should be kept in mind in performing neuroimaging studies.

Transient iatrogenic Horner's syndrome following carotid endarterectomy.

We present a case of a 72-year-old man who developed a transient Horner's syndrome in the immediate postoperative period after right carotid endarterectomy which resolved within 3 months. Although Horner's syndrome has been reported with several disorders of the carotid artery, our case documents a rare iatrogenic oculosympathetic paresis after elective carotid endarterectomy.

Síndrome de Horner como presentación de tuberculosis pulmonar / Horner's syndrome as presentation of pulmonary tuberculosis

El síndrome de Horner es el resultado de un bloqueo de la inervación simpática del ojo en cualquier punto de su trayectoria. Puede tener variadas etiologías, y es una forma muy inusual de presentación de tuberculosis pulmonar. Se describe el caso de un paciente que presenta un síndrome de Horner secundario a afectación del ápex pulmonar por tuberculosis.

Horner´s syndrome results from a blockage of the sympathetic innervation to the eye at any point along its trajectory. It presents various etiologies, and it is a very unusual form of presentation of pulmonary tuberculosis. We describe the case of a patient with a Horner syndrome secondary to involvement of the pulmonary apex due to tuberculosis.

Lymphatic malformation with acquired Horner syndrome in an infant.

An infant presented with right upper eyelid ptosis and was subsequently diagnosed with acquired Horner syndrome. Further evaluation revealed a right-sided cervicothoracic lymphatic malformation. At 13 weeks of age, the child underwent percutaneous intracystic sclerotherapy with a mixture of sodium tetradecyl sulphate and ethanol. Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis. Percutaneous sclerotherapy not only effectively treated the space-occupying lymphatic malformation but also reversed the Horner syndrome that was presumably induced by neural tension (more likely) or compression.

Lymphatic malformation with acquired Horner syndrome in an infant.

An infantpresented with right upper eyelid ptosis and was subsequently diagnosed with acquired Horner syndrome. Further evaluation revealed a right-sided cervicothoracic lymphatic malformation. At 13 weeks of age, the child underwent percutaneous intracystic sclerotherapy with a mixture of sodium tetradecyl sulphate and ethanol. Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis. Percutaneous sclerotherapy not only effectively treated the space-occupying lymphatic malformation but also reversed the Horner syndrome that was presumably induced by neural tension (more likely) or compression.

[Villaret syndrome and lung adenocarcinoma: an unusual association]. / Síndrome de Villaret y adenocarcinoma de pulmón: Una asociación infrecuente.

Villaret syndrome is defined by the affection of the glossopharyngeal (IX), vagal (X), accessory (XI) and hypoglossal (XII) cranial nerves associated with ipsilateral Horner syndrome. It is caused by the compression of these nerves and the neighboring sympathetic plexus fibers at the base of the skull, particularly in the retroparotid space. Even though the invasion of the central nervous system in patients with advanced lung cancer is a frequent and well known occurrence, this particular symptomatic association is extremely rare. We are reporting the case of a newly diagnosed lung adenocarcinoma patient who is simultaneously developing this syndrome.

Síndrome de Villaret y adenocarcinoma de pulmón: una asociación infrecuente / Villaret syndrome and lung adenocarcinoma: an unusual association

El síndrome de Villaret se define por la afección de los nervios craneales glosofaríngeo (IX), vago (X), espinal (XI) e hipogloso mayor (XII), en conjunción con el síndrome de Horner homolateral a la lesión. Se produce por compresión de estos nervios y de las fibras vecinas del plexo simpático pericarotídeo en la base del cráneo, en particular, en el espacio retroparotídeo. Si bien es un hecho conocido la invasión del sistema nervioso central en el cáncer de pulmón avanzado, esta particular asociación sintomática es extremadamente infrecuente. Presentamos una paciente con diagnóstico reciente de adenocarcinoma de pulmón que desarrolló, en forma simultánea, este síndrome.

Villaret syndrome is defined by the affection of the glossopharyngeal (IX), vagal (X), accessory (XI) and hypoglossal (XII) cranial nerves associated with ipsilateral Horner syndrome. It is caused by the compression of these nerves and the neighboring sympathetic plexus fibers at the base of the skull, particularly in the retroparotid space. Even though the invasion of the central nervous system in patients with advanced lung cancer is a frequent and well known occurrence, this particular symptomatic association is extremely rare. We are reporting the case of a newly diagnosed lung adenocarcinoma patient who is simultaneously developing this syndrome.

The Yield of Diagnostic Imaging in Patients with Isolated Horner Syndrome.

We sought to determine, with a retrospective chart review, the imaging yield for patients with clinically isolated Horner syndrome. MRI/MRA of the head and neck extending from the supraorbital ridge to T4 with fat suppression and with postcontrast images was obtained. Of 88 patients with isolated Horner syndrome who were imaged, 20% had a causative etiology on imaging. The most common cause of an isolated Horner syndrome was a carotid artery dissection. There was 1 patient with a primary malignancy found to be the causative lesion in this group, and 1 patient with spread of their known metastatic disease.

Unilateral Suppression of Brown Fat on FDG PET/CT in Horner Syndrome.

A 29-year-old woman underwent resection of a left anterior mediastinal thymoma and pleurectomy. Postsurgical FDG PET/CT scan demonstrated FDG avidity in the right neck and upper thoracic fat but relatively absent FDG-avid fat in the left neck and upper thorax. Bilateral FDG-avid fat was also apparent in the lower chest and upper abdomen. After surgery, the patient demonstrated Horner syndrome, with left-sided ptosis, miosis, and facial anhidrosis. It is hypothesized that left-sided sympathetic nerves were compromised during surgery, leading to Horner syndrome and denervation of ipsilateral brown fat. The unilateral FDG avidity should not be mistaken for malignancy.