Body Lateropulsion in Stroke: Case Report and Systematic Review of Stroke Topography and Outcome.

INTRODUCTION: Body lateropulsion (BLP) is seen in neurological lesions involving the pathways responsible for body position and verticality. We report a case of isolated body lateropulsion (iBLP) as the presentation of lateral medullary infarction and conducted a systematic literature review. METHODS: MEDLINE and EMBASE databases were searched up to December 3, 2020. INCLUSION CRITERIA: age ≥ 18, presence of BLP, confirmed stroke on imaging. EXCLUSION CRITERIA: age < 18, qualitative reviews, studies with inadequate patient data. Statistical analysis was performed using IBM® SPSS® Statistics 20. RESULTS: A 64-year-old man presented with acute-onset iBLP. Brain MRI demonstrated acute infarction in the right caudolateral medulla. His symptoms progressed with ipsilateral Horner syndrome over the next 24 hours and contralateral hemisensory loss 10 days later. Repeat MRI showed an increase in infarct size. BLP resolved partially at discharge. Systematic review: 418 abstracts were screened; 59 studies were selected reporting 103 patients. Thirty-three patients had iBLP (32%). BLP was ipsilateral to stroke in 70 (68%) and contralateral in 32 (32%). The most common stroke locations were medulla (n = 63, 59%), pons (n = 16, 15%), and cerebellum (n = 16, 15%). Four strokes were cortical, 3 frontal and 1 temporoparietal (3%). The most common etiology was large-artery atherosclerosis (LAA) in 20 patients (32%), followed by small-vessel occlusion in 12 (19%). Seventeen (27%) had large-vessel occlusion (LVO), 12 involving the vertebral artery. Sixty (98%) had some degree of resolution of BLP; complete in 41 (70%). Median time-to-resolution was 14 days (IQR 10-21). There was no relationship between time-to-resolution and age, sex, side of BLP or side of stroke. CONCLUSION: BLP was commonly seen with medullary infarction and was the isolated finding in one-third. LAA and LVO were the most common etiologies. Recovery of BLP was early and complete in most cases.

Investigation of Pupillary Changes After Carotid Endarterectomy and Carotid Stent Placement Using Automated Pupillometry.

INTRODUCTION: Horner's syndrome has been reported after carotid artery endarterectomy (CEA) and carotid artery stenting (CS). This study evaluates pupillary changes after these procedures using automated pupillometry. METHODS: Retrospective analysis from a prospective database of pupillometry readings. Cases (14 patients with CEA/CS) were matched to controls (14 patients without CEA/CS). t test models were constructed to examine pupillary light reflex measures for CEA, CS, and controls. RESULTS: The 28 subjects had a mean age of 70 years, 50% were male, and 96% were Caucasian. There was no significant difference in the mean pupil size, constriction velocity (CV), dilation velocity (DV) between the procedural side compared to the contralateral side. However, the mean DV in the left eye after a left sided procedure among CS patients (.67) was lower than mean DV in left eye among controls (.88; P < .0001) and patients undergoing CEA (1.03; P < .0001). DISCUSSION: CS may result in disruption of the carotid artery plexus and decreased sympathetic response thereby reducing DV in the ipsilateral pupil. In addition, decreased CV can also been seen. CONCLUSION: The findings confirm and extend those of previous authors suggesting that pupillary changes may be seen after CS and automated handheld pupillometry may aid in the detection of Horner Syndrome.

Harlequin Syndrome in Acute Thalamic Hemorrhage.

Harlequin syndrome is a disorder of the autonomic nervous system. It clinically presents as a distinct line of hemifacial sympathetic denervation. We describe a case of Harlequin syndrome with co-existing central first-order Horner syndrome in the setting of a large thalamic hemorrhage with intraventricular extension.

Twenty-Four Syndrome: An Untold Presentation of Pontine Hemorrhage.

Pontine hemorrhages are relatively uncommon. Various atypical manifestations of pontine stroke like eight-and-a-half syndrome, fifteen-and-a-half syndrome, and sixteen syndrome have been described in the past. We came across a case of pontine bleed that presented with bilateral facial palsy, bilateral horizontal gaze palsy, and contralateral sensorineural hearing loss accounting to the hitherto not described "twenty-four syndrome" with Horner's syndrome and left hemiparesis.

Horner's Syndrome after Scalene Block and Carotid Dissection.

BACKGROUND: Horner's syndrome refers to the clinical triad of ptosis, miosis, and anhidrosis resulting from disruption of the ocular and facial sympathetic pathways. A myriad of etiologies can lead to Horner's syndrome; awareness of the underlying anatomy can assist physicians in identifying potential causes and initiating appropriate care. CASE REPORT: Two patients presented to our Nashville-area hospital in 2014. Patient 1 was a 26-year-old man who noticed facial asymmetry one day after an outpatient orthopedic procedure. His symptoms were attributed to his posterior interscalene anesthesia device; with deactivation of this device, the symptoms rapidly resolved. Patient 2 was a 42-year-old man who presented to our emergency department with persistent headache and ptosis over several weeks. Computed tomography angiography revealed ipsilateral carotid dissection and the patient was admitted for further management. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The pathologies underlying Horner's syndrome are exceedingly diverse. Although classic teaching often focuses on neoplastic causes, and more specifically Pancoast tumors, neoplasms are discovered only in a small minority of Horner's syndrome cases. Other etiologies include trauma, cervical artery dissection, and infarction. With a better understanding of the pertinent anatomy and array of possible etiologies, emergency physicians may have more success in identifying and treating the causes of Horner's syndrome.

Cardiovascular autonomic function in lateral medullary infarction.

Even though the medulla contains the baroreceptor regulatory centers, few studies have focused on the autonomic dysfunction of lateral medullary infarction (LMI). Therefore, cardiac parasympathetic and sympathetic functions were compared in LMI patients and age-matched controls. We prospectively recruited 25 LMI patients who had ipsilateral Horner's sign and cardiac autonomic function testing without a history of diabetes, cardiac disease, or previous stroke. Parasympathetic function tests included beat-to-beat heart rate variation during deep breathing, 30:15 heart rate ratio testing while standing, and the valsalva ratio. Sympathetic function tests included blood pressure during active standing and sustained handgrip. The composite autonomic score (CAS) was measured as a total of 10 points; parasympathetic dysfunction was designated as ≥3 points in the parasympathetic subscores and sympathetic dysfunction as ≥2 points in the sympathetic subscores. Multiple regression analysis was performed to predict parasympathetic or sympathetic dysfunction. The mean age and stroke risk factors of the study population were not significantly different between the LMI group (n = 25) and control group (n = 29). However, cardiac autonomic functions were significantly different in the groups: parasympathetic dysfunction (14 vs. 4 patients, p = 0.011) and sympathetic dysfunction (3 vs. 13 patients, p = 0.008). In univariate analysis, male-gender (p = 0.011), right-side involvement (p = 0.035) and ventral involvement (p = 0.007) were significantly associated with parasympathetic dysfunction (CAS subscore ≥3). In multivariable analysis, the ventral involvement remained to be the independent predictor for parasympathetic dysfunction (OR 16.0; 95% CI 2.2-118.3, p = 0.007). This study suggests that LMI patients are susceptible to cardiac parasympathetic dysfunction, especially in the ventral medulla.

[Ocular signs in anterior and posterior cervical sympathetic syndrome].

Features of anatomy and morphological changes of cervical spine resulting in sympathetic innervation defects, blood supply deficiency and ocular symptoms are reviewed. Results of experimental and clinical studies showing correlation of sympathetic cervical ganglions irritation and ocular pathologic conditions are presented. Ocular involvement in neurologic changes in anterior and posterior cervical sympathetic syndrome are reviewed.

Impact of T3 thoracoscopic sympathectomy on pupillary function: a cause of partial Horner's syndrome?

BACKGROUND: Thoracoscopic bilateral sympathicolysis of the T3 sympathetic ganglia is an effective treatment for palmar hyperhidrosis, though not without potential complications and consequences such as Horner's syndrome. The objective of our study is to evaluate the repercussion of T3 sympathetic denervation on pupillary tone in patients with primary hyperhidrosis. METHODS: A prospective descriptive study of 25 patients (50 pupils) ranging in age from 18 to 40 years with an indication of T3 sympathectomy for palmar hyperhidrosis or palmar-plantar hyperhidrosis from 1 December 2009 to 31 December 2010 was carried out. We excluded all patients with previous eye surgery or other ocular pathologies and those with pathologies that contraindicate denervation surgery and ocular study. All patients were evaluated before surgery and at 24 h and 1 month after sympathetic denervation. Pupil/iris (P/I) ratio was measured before and after instillation of sympathicomimetic eye drops containing 1% apraclonidine. RESULTS: No statistically significant differences were found when we compared the preoperative P/I ratio of the left eyes versus the right eyes (P = 0.917). We found statistically significant differences (P < 0.001) between the preoperative P/I ratio [0.40 mm (standard deviation, SD 0.07 mm)] and the postoperative basal ratio [0.33 (SD 0.05)] at 24 h. The P/I ratio at 24 h increased from 0.33 to 0.36 (SD 0.09), a nonsignificant increase (P = 0.45), after instillation of medicated eye drops. No differences were observed between the preoperative [0.40 (SD 0.07)] and 1-month basal values [0.38 (SD 0.07)], and instillation of apraclonidine no longer induced a hypersensitivity response. CONCLUSIONS: T3 sympathectomy leads to subclinical pupillary dysfunction with a tendency for miosis, even though this impairment is not generally evident on standard physical examination or reported by patients. This subclinical dysfunction may be caused by injury to an undefined group of presympathetic nerve cell axons in caudocranial direction that communicate with the cervical sympathetic ganglia and whose function is mydriatic pupillary innervation.

Early diagnosis of Horner syndrome using topical apraclonidine.

The diagnosis of Horner syndrome (HS) using apraclonidine eye drops is an alternative to the use of topical cocaine drops. A number of reports have described the efficacy of apraclonidine testing, but there is some debate over its sensitivity in the acute setting. We describe a patient with HS secondary to carotid dissection who had a positive response to apraclonidine 3 hours after the onset of symptoms. The case is made for a larger study of apraclonidine use to determine its true sensitivity and specificity, identify confounding factors, and redefine the criteria for positive testing.

[Acquired Horner's syndrome]. / Ervervet Horners syndrom.

BACKGROUND: Horner's syndrome is characterized by the classic triad of eyelid ptosis, miosis and facial anhidrosis and is caused by an interruption of the oculosympathetic nerve pathway somewhere between its origin in the hypothalamus and the eye. MATERIAL AND METHODS: This review is based on own experiences and a discretionary selection of articles found through non-systematic searches in PubMed. Cases from own practice serve as examples. RESULTS: Based on localization of the nerve pathway interruption, a Horner's syndrome is often classified as central, pre- or postganglionic. For the central type the syndrome is associated with other symptoms and signs from the central nervous system. The preganglionic type is most often caused by a tumor or trauma. The postganglionic type is often associated with pain/headache; most frequently it is seen as a consequence of carotid artery dissection or during cluster headache. Anhidrosis is rarely prominent, and in the postganglionic subtype it is virtually absent. Pharmacological tests can be used in diagnostics. Apraclonidine seems to be a good alternative to cocaine to confirm Horner's syndrome. MRI is generally recommended in the evaluation, if necessary with special sequences. INTERPRETATION: The path of the long oculosympathetic fibers is complex and not fully understood. Topographic diagnostics may be challenging, but in most cases a specific cause is identified.

Effect of stellate ganglion block on the cerebrovascular system: magnetic resonance angiography study.

BACKGROUND: Several studies have shown that stellate ganglion block (SGB) is an effective treatment for certain cerebrovascular related diseases; however, the direct effect of SGB on the cerebral vasculature is still unknown. The present study investigated the effect of SGB on the cerebral vascular system using magnetic resonance angiography. METHODS: Time-of-flight magnetic resonance angiography images of 19 healthy female volunteers (mean ages of 46.4 ± 8.9 yr) were obtained before and after SGB with 1.5-T magnetic resonance imaging. The authors determined successful interruption of sympathetic innervation to the head with the appearance of Horner syndrome and conjunctival injection. We measured changes in the average signal intensity and diameter of the major intracranial and extracranial arteries and their branches, which were presented with mean (±SE). RESULTS: The signal intensity changes were observed mainly in the ipsilateral extracranial vessels; the external carotid artery (11.2%, P < 0.001) and its downstream branches, such as the occipital artery (9.5%, P < 0.001) and superficial temporal artery (14.1%, P < 0.001). In contrast, the intensities of the intracranial arteries did not change with the exception of the ipsilateral ophthalmic artery, which increased significantly (10.0%, P = 0.008). After SGB, only the diameter of the ipsilateral external carotid artery was significantly increased (26.5%, P < 0.001). CONCLUSIONS: We were able to observe significant changes in the extracranial vessels, whereas the intracranial vessels were relatively unaffected (except for the ophthalmic artery), demonstrating that both perivascular nerve control and sympathetic nerve control mechanisms may contribute to the control of intracranial and extracranial blood vessels, respectively, after SGB.

[Pupillary disorders - diagnosis, diseases, consequences]. / Pupillenstörungen - Diagnostik, Erkrankungen, Konsequenz.

Examination of the pupil offers an objective evaluation of visual function as well as the vegetative pathways to the eye. Essential information is gathered within a short time. This makes pupillary inspection a valuable part of the routine ophthalmological, neurological and general medical examinations. Due to the proximity of pupillary pathways to various anatomic structures, pupillary dysfunction can be caused by a variety of disorders, some of which may be life threatening. The ophthalmologist plays a key role in detecting pupillary disorders and in directing further investigations. Therefore, one should have a good knowledge of the diagnostic significance of pupillary function and dysfunction.

Neuroanatomical basis of Wallenberg syndrome.

Wallenberg syndrome, or lateral medullar syndrome, is the clinical presentation of the infarct in the territory of posterior inferior cerebellar artery. Its signs and symptoms include vertigo, nystagmus, diplopia, ipsilateral Horner syndrome, facial ruddiness and dry skin, dysphonia, dysphagia, dysarthria, ipsilateral loss of gag reflex, ipsilateral ataxia, ipsilateral impaired taste, ipsilateral facial pain and paresthesia, decreased ipsilateral blink reflex, contralateral hypoalgesia and thermoanaesthesia in the trunk and limbs; and ipsilateral facial hypoalgesia and thermoanaesthesia. Neuroanatomical knowledge is essential to its comprehension, study and diagnosis, because the classic neurological manifestations are easy to explain and understand if function and localization of affected anatomical structures are known as if the posterior cerebral circulation is.

El síndrome de Wallenberg, o síndrome bulbar lateral, es la manifestación clínica del infarto en el territorio de irrigación de la arteria cerebelosa posteroinferior. Su presentación incluye vértigo, nistagmo, diplopía, síndrome de Horner, rubicundez y anhidrosis facial homolateral, disfonía, disfagia, disartria, pérdida homolateral del reflejo nauseoso, ataxia homolateral, disgeusia homolateral, dolor y parestesia faciales homolaterales, pérdida o disminución homolateral del reflejo corneal, hipoalgesia y termoanestesia de tronco y extremidades contralaterales, hipoalgesia y termoanestesia facial homolateral. El conocimiento neuroanatómico es imprescindible para su comprensión, estudio y diagnóstico, ya que sus manifestaciones neurológicas clásicas son fácilmente explicables y entendibles si se conocen la función y la localización de las estructuras anatómicas afectadas, así como la irrigación cerebral posterior.

Dry beriberi mimicking the Guillain-Barre syndrome.

A 44-year-old man is described with severe flaccid quadriparesis that evolved over 3 weeks. He had regularly binged on alcohol-up to 20 cans of beer per day with occasional consumption of spirits-for more than 15 years but had balanced this with regular food intake. However, for a week prior to the current episode he had not eaten anything of significance. Nerve conduction studies revealed a background peripheral, mainly sensory, neuropathy with a superimposed acute motor axonopathy. CSF was normal. He improved with high dose vitamin replacement and physiotherapy but remains dependent on a Zimmer frame for mobility and a splint for wrist drop.

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.

Artemin is a vascular-derived neurotropic factor for developing sympathetic neurons.

Artemin (ARTN) is a member of the GDNF family of ligands and signals through the Ret/GFRalpha3 receptor complex. Characterization of ARTN- and GFRalpha3-deficient mice revealed similar abnormalities in the migration and axonal projection pattern of the entire sympathetic nervous system. This resulted in abnormal innervation of target tissues and consequent cell death due to deficiencies of target-derived neurotrophic support. ARTN is expressed along blood vessels and in cells nearby to sympathetic axonal projections. In the developing vasculature, ARTN is expressed in smooth muscle cells of the vessels, and it acts as a guidance factor that encourages sympathetic fibers to follow blood vessels as they project toward their final target tissues. The chemoattractive properties of ARTN were confirmed by the demonstration that sympathetic neuroblasts migrate and project axons toward ARTN-soaked beads implanted into mouse embryos.

Benign Raeder syndrome evolving into indomethacin-responsive hemicranial headache.

Benign Raeder syndrome is characterized by a self-limiting unilateral continuous headache associated with ipsilateral ptosis, miosis, and frequently, facial hypohydrosis. Hemicrania continua is a chronic, strictly unilateral continuous headache associated with ipsilateral cranial autonomic symptoms. We report a 50-year-old man who presented with benign Raeder syndrome, which evolved into an indomethacin-responsive hemicranial headache that resembled hemicrania continua.

Split notochord syndrome with congenital unilateral Horner's sign.

A 2-year-old boy exhibited congenital right Horner's sign and right finger, wrist, and elbow flexion arthrogryposis. He had dyspnea and feeding difficulty 12 hours after birth. Radiologic examination revealed a thoracoabdominal intestinal tube and mediastinal cystic lesion at the right side, with vertebral anomaly at the cervical level. Histopathologically, the intestinal tube was diagnosed as bowel duplication. Because the mediastinal lesion could not be resected surgically, no histopathological diagnosis was made. Embryologically, the combination of transdiaphragmatic duplication, mediastinal cystic lesion, anterior spina bifida, and hemivertebra suggested notochord malformation. The diagnosis was split notochord syndrome, an extremely rare embryological malformation syndrome. Congenital unilateral Horner syndrome often has unknown etiology. In this case, cervical vertebral anomalies and mediastinal cystic lesion implied a compressed nerve root, resulting in Horner syndrome and right finger, wrist, and elbow flexion joint contracture. Split notochord syndrome should be included in differential diagnosis of congenital unilateral Horner syndrome.