Left Head Tilt Research Articles

Coexistence of congenital superior rectus muscle fibrosis and Marcus Gunn jaw-winking phenomenon

A 6-year-old male involving a coexistence of congenital fibrosis of the superior rectus muscle (SR) with Marcus Gunn Jaw-Winking phenomenon presented with an elevation of the upper eyelid along with a chronically open mouth that had been present since early infancy. There was extropia and hypertropia, a limitation of elevation and depression in the right eye, and an abnormal head posture (AHP) with a left head tilt, right face turn, and a chin-up head position. He also displayed an upper eyelid retraction and a 2-mm lagophthalos in the right eye. Results of the forced duction test indicated a constricted SR of the right eye. A dissection of the SR from the levator palpebrae superioris and a 5-mm SR recession was performed within the right eye. At 2 months post surgery, there was a 3 prism diopters (PD) hypertropia in the right eye when the left eye was fixated on the target and a 10 PD hypotropia in the left eye when the right eye was fixated on the target, along with an improvement in his AHP. In this rare case with a coexistence of congenital fibrosis of the SR and Marcus Gunn jaw-winking phenomenon, we found that the performance of an individualized surgical protocol proved effective in reducing the vertical deviation and improving AHP.

Case report: Acute audiovestibular presentation following hemi-pontine infarction

Posterior circulation stroke is an uncommon cause of sudden-onset sensorineural hearing loss (SSNHL). Anterior inferior cerebellar artery occlusion results in ipsilateral peripheral audiovestibular dysfunction. Few reports describe posterior circulation stroke presenting with contralateral SSNHL and acute vestibular and focal neurological signs. We present a case of contralateral SSNHL and isolated acute central vestibular dysfunction in the absence of other central focal neurological deficits. To the best of our knowledge, this has not been described to date. The patient was identified to have asymmetrical SSNHL, subtle skew deviation with left head tilt, and significant refixation saccades on video head impulse test despite bilaterally normal vestibulo-ocular reflex gains. Left pontine infarct was suspected and confirmed on magnetic resonance imaging. The patient was treated with an appropriate antiplatelet regimen. We highlight the importance of a thorough clinical diagnostic work-up as posterior circulation strokes with isolated audiovestibular deficits can be easily missed if other significant neurological deficits are absent.

Long‐term follow‐up of a diffuse cranial neurofibroma in a 4‐month‐old dog

AbstractDiffuse neurofibroma affects skin and subcutaneous tissues of the head and neck of children and young adults. Fourteen cases have been described in dogs, but none infiltrating a puppy's cranial cavity. A 4‐month‐old, female entire Pitbull was referred with a 2‐month history of left facial nerve paralysis, difficulty swallowing and left head tilt suggesting multifocal neurolocalisation. Computed tomography revealed an extra‐axial mass occupying the left nasal cavity, extending caudally along the skull base and following multiple cranial nerves (optic, oculomotor, trochlear, trigeminal, abducens, facial, glossopharyngeal, vagus and hypoglossal) exiting the skull through their respective foramina and continuing across their peripheral pathways. A superficial facial nerve biopsy was obtained leading to a diagnosis of neurofibroma. Treatment with corticosteroids showed lack of response, so no long‐term treatment was prescribed. Twenty‐one months after diagnosis, the dog remains stable without treatment, suggesting a very slow growth with acceptable long‐term quality of life.

Skew deviation and ocular tilt reaction as presenting feature of CACNA1A mutation

We report the case of a 9-year-old girl with aggravation of childhood left head tilt without diplopia. She had right hypertropia and right incyclotorsion, which was compatible with skew deviation and ocular tilt reaction (OTR). She had ataxia, epilepsy, and cerebellar atrophy. Her OTR and neurologic dysfunctions were secondary to a channelopathy caused by CACNA1A mutation.

From WEMINO To WEBINO With Unilateral LMN Facial Palsy (P5-4.002)

<h3>Objective:</h3> To describe a case of progression from apparent trochlear nerve palsy, to wall-eyed monocular internuclear ophthalmoplegia (WEMINO), to wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) with unilateral lower motor neuron (LMN) facial palsy. <h3>Background:</h3> Various descriptions of INO with cranial nerve palsies are in the literature, but we report a patient with a remarkably instructive stepwise evolution. <h3>Design/Methods:</h3> Review of records of a patient with diplopia at our institution. <h3>Results:</h3> A 42-year-old woman presented acutely with oblique binocular diplopia worsening on right gaze and left head tilt, with left hypertropia. Trochlear nerve palsy was suspected but contrasted MRI brain with orbits, and initial CSF testing were unrevealing. On day 3, the hypertropia partly improved but she developed left eye adduction deficit. Given the fluctuation, myasthenia gravis was considered high in the differential diagnosis while test results were awaited. On day 7, she developed LMN left facial palsy and bilateral adduction deficit with abducting eye nystagmus, leading to the realization that a single progressing lesion in the pontine tegmentum could explain her entire sequence of events. Repeat MRI confirmed a minute enhancing lesion in the floor of the fourth ventricle corresponding to the dorsomedial surface of the left facial colliculus and bilateral medial longitudinal fasciculi (MLF), likely of demyelinating etiology. Her pending CSF tests returned with 7 oligoclonal bands. She received intravenous methylprednisolone and her symptoms nearly resolved by day 14. <h3>Conclusions:</h3> This patient presented with evolving symptoms that appeared to localize to the trochlear nerve initially, but lacked imaging correlate. It is clear in retrospect that her initial presentation represented a skew deviation from early INO, progressing to WEMINO and eventually WEBINO with unilateral fascicular facial palsy. The anatomical correlate was confirmed on repeated imaging. Her clinical course beautifully illustrates the importance of lesion localization for etiological diagnosis. <b>Disclosure:</b> Dr. Senthamarai Siddharthan has nothing to disclose. Dr. Witt has nothing to disclose. Dr. Kini has nothing to disclose. Dr. Vasireddy has nothing to disclose. The institution of Dr. Mathur Kumaraswamy has received research support from Woolsey Pharmaceuticals.

Otogenic intracranial abscessation secondary to an inflammatory polyp with chronic otitis media/interna in a cat

AbstractA 2‐year‐old spayed female domestic short haired cat presented for evaluation of chronic lethargy, inappetence and otitis media/interna that developed secondary to an inflammatory polyp, which had been removed via traction a month prior to presentation. Upon presentation, the cat displayed mild vestibular ataxia and left head tilt, in addition to a bulging left tympanic membrane. Therapy with broad‐spectrum antibiotic therapy and anti‐inflammatory corticosteroids was initiated, however the cat soon developed signs of central vestibular disease and obtunded mentation. Magnetic resonance imaging revealed left‐sided otitis media/interna and cerebellar abscessation. Surgical debridement of the abscess was indicated but declined by the owner in favour of euthanasia.

Choroid plexus carcinoma in a dog—Case report

AbstractObtaining a conclusive definitive diagnosis for an intra‐ventricular tumour in a dog can be challenging. In doing so, all differential diagnoses should be considered. Differentiation between choroid plexus tumours (CPTs) (a choroid plexus papilloma, atypical choroid plexus papilloma or choroid plexus carcinoma) and gliomas, including ependymoma, oligodendroglioma and astrocytoma should be considered. Histopathologic features and immunohistochemistry are essential in the differentiation between intra‐ventricular tumours. A 4‐year‐old Boerboel dog presented with a left head tilt and a wide range of nonspecific clinical abnormalities not limited to weight loss, depression and marked bradycardia. MRI proved useful in providing a diagnosis of an intra‐ventricular tumour and demonstrated the potential of choroid plexus carcinomas (CPCs) to metastasize. In this case, the histological features displayed malignancy with invasion into the neuroparenchyma, drop metastasis and immunohistochemistry with E‐cadherin‐positive result that assisted with differentiation of CPC from other tumours.

Unilateral Isolated Inferior Rectus Palsy: A Rare Presentation of Ocular Myasthenia Gravis

Ocular Myasthenia Gravis with unilateral isolated Inferior Rectus palsy and no ptosis is rarely encountered. We present a case of 25-year-old male with sudden onset binocular diplopia and no features of ptosis. Deviations in primary gaze were 3-8 Prism Dioptre (PD) with left hypertropia. Right and left head tilt deviations were 5PD and 3PD left hypertropia respectively with increase in hypertropia in left gazes. Acetylcholine receptor binding antibodies were found to be raised. Repetitive Nerve Stimulation showed decreased amplitude. Patient was managed with Oral Pyridostigmine with excellent symptomatic relief and drug tolerance. No features of Myasthenia Gravis (Systemic or Ocular) were noted on follow-up.

Subjective visual vertical imprecision during lateral head tilt in patients with chronic dizziness.

Most prior studies of the subjective visual vertical (SVV) focus on inaccuracy of subjects' SVV responses with the head in an upright position. Here we investigated SVV imprecision during lateral head tilt in patients with chronic dizziness compared to healthy controls. Forty-five dizzy patients and 45 healthy controls underwent SVV testing wearing virtual reality (VR) goggles, sitting upright (0°) and during head tilt in the roll plane (± 30°). Ten trials were completed in each of three static head positions. The SVV inaccuracy and SVV imprecision were analyzed and compared between groups, along with systematic errors during head tilt, i.e., A-effect and E-effect (E-effect is a typical SVV response during head tilts of ± 30°). The SVV imprecision was found to be affected by head position (upright/right head tilt/left head tilt, p < 0.001) and underlying dizziness (dizzy patients/healthy controls, p = 0.005). The SVV imprecision during left head tilt was greater in dizzy patients compared to healthy controls (p = 0.04). With right head tilt, there was a trend towards greater SVV imprecision in dizzy patients (p = 0.08). Dizzy patients were more likely to have bilateral (6.7%) or unilateral (22.2%) A-effect during lateral head tilt than healthy controls (bilateral (0%) or unilateral (6.7%) A-effect, p < 0.01). Greater SVV imprecision in chronically dizzy patients during head tilts may be attributable to increased noise of vestibular sensory afferents or disturbances of multisensory integration. Our findings suggest that SVV imprecision may be a useful clinical parameter of underlying dizziness measurable with bedside SVV testing in VR.

Head Roll-Tilt Subjective Visual Vertical Test in the Diagnosis of Persistent Postural-Perceptual Dizziness.

Objective:To examine the validity of head roll-tilt subjective visual vertical (HT-SVV) in diagnosing persistent postural-perceptual dizziness (PPPD).Study Design:Retrospective review.Setting:Tertiary referral center.Patients:Sixty-one patients with PPPD, 10 with unilateral vestibular hypofunction (UVH), and 11 with psychogenic dizziness (PD), showing chronic vestibular symptoms for >3 months.Interventions:Head-tilt perception gain (HTPG, i.e., mean perceptual gain [perceived/actual tilt angle]) during right or left head tilt of approximately 30° (HT-SVV) and conventional head-upright SVV (UP-SVV) were measured. Bithermal caloric testing, cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP), and posturography were conducted.Main Outcome Measures:Multiple comparisons were performed for the HT-SVV and other vestibular tests among the disease groups. A receiver operating characteristic curve was created to predict PPPD using HTPG.Results:HTPG was significantly greater in the PPPD group than in the UVH and PD groups. There were no significant differences in UP-SVV, cVEMP, oVEMP, and posturography (foam ratio and Romberg ratio on foam) among the disease groups, while the UVH group had the highest canal paresis compared to the other two groups. The area under the curve of the receiver operating characteristic curve for predicting PPPD was 0.764, and the HTPG value of 1.202 had a specificity of 95.2% for diagnosing PPPD.Conclusions:While conventional vestibular tests including UP-SVV, VEMPs, and posturography did not show abnormalities in PPPD, high HTPG in the HT-SVV test, an excessive perception of head tilt, can be a specific marker for discriminating PPPD from other chronic vestibular diseases.

A Case of Idiopathic Orbital Inflammation Presenting as Isolated Myositis of the Inferior Oblique Muscle

Purpose: To report a case of idiopathic orbital inflammation presenting with isolated myositis of the inferior oblique muscle. Case summary: A 54‐year‐old man presented with swelling on the left lower lid, pain on superonasal and inferonasal gaze, and binocular diplopia for 2 months. His head was tilted to the right by about 5° and mild conjunctival injection and 3 mm narrowing of palpebral fissure of the left eye compared to the other eye were observed. Eight prism diopter (PD) left hypertropia and 4 PD intermittent esotropia were noted on primary gaze, which worsened on leftward gaze, downward gaze, and left head tilt. Orbital magnetic resonance imaging (MRI) showed enhanced hypertrophy of the left inferior oblique muscle. Systemic work‐up for possible inflammatory diseases yielded negative results. Therefore, a presumptive diagnosis of idiopathic isolated myositis of the left inferior oblique muscle was made. The patient was treated with 60 mg of oral corticosteroid per day for the first week, and the dose was tapered for 1 month as the symptoms reduced. Two months later, the patient became free from any symptoms and follow-up orbital MRI showed a significant decrease in size of the left inferior oblique muscle. There have been no signs of recurrence for 7 months. Conclusions: A presumptive diagnosis of idiopathic isolated myositis of the inferior oblique muscle was made in a patient with swelling of the left lower lid and binocular diplopia based on orbital MRI and systemic work‐up. Good results were achieved with oral corticosteroid therapy.

Nuclear Fourth Nerve Palsy

A 71-year-old woman reported vertical diplopia immediately following cardiac catheterization. On examination, she had a right hypertropia that increased on left gaze and right head tilt, with 8 degrees of excyclotorsion of the right eye in primary gaze. She had no other neurologic findings. Postcontrast axial computed tomography (Fig A) and sagittal T1 magnetic resonance imaging (Fig B) demonstrate enhancement of the left fourth nerve nucleus (arrows). Companion anatomic illustrations show the location of the fourth nerve nucleus in the caudal midbrain and its decussation to innervate the contralateral superior oblique muscle.

Case 292.

History A 24-year-old right-handed woman presented to a neuro-ophthalmology clinic in Massachusetts in the summer with acute binocular diplopia when looking down and to the left, which started about 1 month earlier. Her medical history was notable for Raynaud syndrome, recurrent streptococcal pharyngitis, and an allergy to amoxicillin. Three days prior to developing diplopia, she presented to an outside emergency department due to fever, chills, and back pain. She received ciprofloxacin for presumed urinary tract infection based on urinalysis, which demonstrated few bacteria and was negative for leukocyte esterase, nitrites, and white blood cells. She then presented again to an outside emergency department for diplopia evaluation. Initial MRI and MR angiography of the brain at that time did not demonstrate any relevant findings, and the patient was referred to our department for neuro-ophthalmic evaluation, where she was seen 4 weeks later. Neuro-ophthalmic examination revealed 20/20 visual acuity in both eyes, and a right hypertropia in left gaze, downgaze and right head tilt, with right eye excyclotorsion. There were no ocular signs of myasthenia gravis or thyroid eye disease, nor did the patient report ocular or systemic symptoms. She denied recent travel. High-spatial-resolution MRI of the brain and orbit were performed (Figs 1, 2).

Test-retest reliability of subjective visual vertical measurements with lateral head tilt in virtual reality goggles

Objective:The objective is to investigate the test-retest reliability of subjective visual vertical (SVV) in the upright position and with lateral head tilts through a computerized SVV measuring system using virtual reality (VR) goggles.Materials and Methods:Thirty healthy controls underwent SVV test in upright position, with the head tilted to the right 30°, and with the head tilted to the left 30°. Subjects wore SVV VR goggles, which contained a gyroscope for monitoring the angle of head tilt. Each subject completed 10 adjustments in each head position. The mean value of SVV deviations and SVV imprecision (the intra-individual variability of SVV deviations from the 10 adjustments) were recorded and compared across different head positions. The participants then repeated the same SVV protocol at least 1 week later. The test-retest reliability of SVV deviation and SVV imprecision were analyzed.Results:The SVV deviation (mean ± standard deviation) was 0.22° ± 1.56° in upright position, −9.64° ± 5.91° in right head tilt, and 7.20° ± 6.36° in left head tilt. The test-retest reliability of SVV deviation was excellent in upright position (intra-class correlation coefficient [ICC] = 0.77, P < 0.001), right head tilt (ICC = 0.83, P < 0.001) and left head tilt (ICC = 0.84, P < 0.001). The SVV values from the 10 adjustments made during right and left head tilts were less precise than when measured at upright (P < 0.001). The test-retest reliability of SVV imprecision was poor at upright (ICC = 0.21, P = 0.26) but fair-to-good in right head tilt (ICC = 0.72, P < 0.001) and left head tilt (ICC = 0.44, P = 0.04).Conclusion:The test-retest reliability of SVV deviation during lateral head tilts via VR goggles is excellent, which supports further research into the diagnostic value of head-tilt SVV in various vestibular disorders. In addition, the degree of SVV imprecision during head tilt has fair-to-good test-retest reliability, which suggests SVV imprecision may have clinical applicability.

Cranial nerve IV palsy after mild head trauma: a rare image.

Mild traumatic brain injury is characterized by a Glasgow coma scale GCS between 13 and 15. It is a frequent pathology in our country because of the increasing number of circulation accidents. Trauma-induced superior oblique palsy usually results from contusion or avulsion of the trochlear nerve. Severe craniocerebral trauma is often associated with the former mechanism, whereas more minor closed-head injuries can decompensate a congenital phoria. We report a patient who developed an isolated trochlear nerve palsy following minor head trauma. Our 53 years-old patient without significant past medical history was victim of a traffic accident. He presented with a complaint of diplopia following head trauma the week prior. He lost consciousness for less than 1 minute. He noticed diplopia immediately upon awakening, but had no other neurologic symptoms. He presented to our emergency department for evaluation. Computed brain tomography (CT) performed did not show any orbital fractures. Ocular exam revealed mild to moderate infraduction deficit and supraduction excess in adduction of the left eye. Left head tilt test was negative. We finally concluded that our patient had a trochlear nerve palsy following the brain trauma. Further Magnetic Resonance Imaging (MRI) revealed an unsuspected tentorial vascular malformation that was compressing the trochlear nerve in its subarachnoid course. In the absence of other features that support decompensation of a congenital trochlear nerve palsy, compressive lesions should be sought in case of fourth cranial nerve palsy following head trauma.

Vertical Strabismus and Positive Bielschowsky Head-Tilt Test: Atypical Presentation of a Maxillary Mucocele

AbstractTest of skew has become a cornerstone in the approach of a patient with vestibular symptoms but a detected vertical misalignment may be caused by an oculomotor disturbance and not a skew deviation. We report the case of an elderly patient with a 1-month history of dizziness and visual disturbance that revealed on bedside examination a spontaneous left head-tilt and a pathologic alternate cover test, with right eye hypertropia and excyclotorsion, worse with right head-tilt. Dizziness was assumed to have a visual origin with unrecognized binocular diplopia, caused by an acquired right eye superior oblique muscle palsy. However, imaging revealed a right maxillary mucocele that eroded the orbit floor into the orbit. The change of the intraorbital component of the maxillary fluid-filled mass with head-tilt through a dehiscent orbital floor may explain the findings of vertical strabismus and positive Bielschowsky head-tilt test in this case. Endoscopic treatment improved symptoms and findings.

Teaching NeuroImages: Trochlear nerve schwannoma

A 54-year-old woman presented for evaluation of binocular vertical diplopia worse in downgaze. She had limited infraduction of the right eye in adduction and a right hypertropia worse in left gaze and right head tilt, consistent with a right trochlear nerve palsy. MRI brain revealed enlargement and enhancement of the right trochlear nerve in the subarachnoid space (figure). Laboratory testing and CSF analysis were normal. The imaging findings were stable over several years, suggesting the diagnosis of a trochlear nerve schwannoma.

Paradoxical Vestibular Syndrome Secondary to Temporal Bone Osteomyelitis and Chronic Otitis Interna in a Guinea Pig (Cavia porcellus)

A 3-year-old female intact guinea pig (Cavia porcellus) was presented for torticollis and ocular discharge. On examination, the guinea pig had a left head tilt with corneal ulceration and absent palpebral response of the right eye. Computed tomography showed a soft tissue attenuating mass with multifocal mineralization compressing the caudal cerebellum with moderate lysis of the calvarium. On recheck, the disease had progressed to include ataxia and proprioceptive deficits. Systemic antibiotic therapy was initiated but the guinea pig only exhibited a marginal treatment response. Due to poor quality of life, the guinea pig was euthanized. A necropsy was performed and a bony lesion was found within the skull compressing the right cerebellum and temporal lobe. Histopathology revealed suppurative osteomyelitis consistent with severe chronic bacterial otitis interna. Vestibular signs in small exotic companion mammals are a common sequela to otitis interna, often noted ipsilateral to an affected inner ear, however, in this case rare paradoxical vestibular signs were observed. Severe chronic otitis interna may result in bony lysis and proliferation capable of expanding from the inner ear resulting in neurological deficits.

Ocular Counter Rolling in Astronauts After Short- and Long-Duration Spaceflight

Ocular counter-rolling (OCR) is a reflex generated by the activation of the gravity sensors in the inner ear that stabilizes gaze and posture during head tilt. We compared the OCR measures that were obtained in 6 astronauts before, during, and after a spaceflight lasting 4–6 days with the OCR measures obtained from 6 astronauts before and after a spaceflight lasting 4–9 months. OCR in the short-duration fliers was measured using the afterimage method during head tilt at 15°, 30°, and 45°. OCR in the long-duration fliers was measured using video-oculography during whole body tilt at 25°. A control group of 7 subjects was used to compare OCR measures during head tilt and whole body tilt. No OCR occurred during head tilt in microgravity, and the response returned to normal within 2 hours of return from short-duration spaceflight. However, the amplitude of OCR was reduced for several days after return from long-duration spaceflight. This decrease in amplitude was not accompanied by changes in the asymmetry of OCR between right and left head tilt. These results indicate that the adaptation of otolith-driven reflexes to microgravity is a long-duration process.

Acute intraparenchymal cerebral haemorrhage in an Iberian golden eagle \u2013 a case report

BackgroundIn birds there are reports of intracranial lesions but not of the clinical, computed tomographic and histopathologic features of acute intraparenchymal cerebral haemorrhage in Iberian golden eagle.Case presentationThe following report describes a case of a 30-year-old Iberian golden eagle (Aquila chrysaetos homeyeri) with no history of trauma, presented with acute opisthotonus, left head tilt and circling, anisocoria, positional nystagmus, and ataxia. The main differential diagnosis were hypovitaminosis B or E and intracranial disease due to trauma, infection, toxins or masses. A computed tomography (CT) of the head was performed with an 8-slices scanner and evidenced a hyperdense (63–65 HU) non-enhancing homogeneous well delineated round area in the midbrain, with 6 mm in its highest diameter. The attenuation values and the non-enhancing nature of the lesion strongly suggested the diagnosis of acute intraparenchymal haemorrhage, which was histologically confirmed after necropsy.ConclusionsIn birds with a central neurological dysfunction, the diagnosis of acute brain haemorrhage should be considered when the CT evidences a non-enhancing, homogeneous, well circumscribed hyperattenuated round area.