Persistent Nystagmus in Chronic Phase of Lateral Medullary Infarction

Background@#and PurposeWe aimed to determine the patterns and mechanisms of persistent nystagmus (PN) lasting >1 year in lateral medullary infarction (LMI). @*Methods@#We recruited 13 patients with PN due to LMI and another 13 with transient nystagmus (TN) (<1 year) as control. All patients underwent oculography, rotatory chair test, caloric test, bedside head impulse test, dizziness handicap inventory (DHI), and brain MRI. @*Results@#All patients had spontaneous, contralesional, horizontal-torsional nystagmus during the acute phase. Although two patients exhibited consistent contralesional torsional nystagmus, most patients (11/13, 85%) with PN evolved from the initial contralesional to ipsilesional nystagmus. During horizontal gaze, the patterns of ipsilesional PN were diverse; torsional (n=5), torsional-downbeat (n=2), horizontal (n=2), and horizontal nystagmus while looking at the lesion side, and torsional nystagmus while looking at the opposite side (n=2). During rotatory chair test, the gains of the vestibulo-ocular reflex in the PN group were lower than those in the TN group to the lesion side at 0.02 and 0.64 Hz. The caudal and ventrolateral parts of the vestibular nuclei were mostly involved in patients with PN. The DHI score did not differ between the groups. @*Conclusions@#PN patterns frequently change in LMI. Resultant vestibular asymmetry after vestibular afferents or cerebellar inhibitory pathway damage and/or inappropriate vestibular compensation may be responsible for PN in LMI. Impairment of the horizontal or vertical neural integrators may be another cause. The presence of PN does not necessarily indicate more severe dizziness in LMI.

Orbital Lymphoma Presenting with Inferior Rectus Palsy

No abstract available.

Vertical Diplopia as a Major Manifestation of Acute Peripheral Vestibulopathy

No abstract available.

Customized Vestibular Rehabilitation in the Patients with Bilateral Vestibulopathy: A Pilot Study in One Referred Center / 대한평형의학회지

OBJECTIVES: Bilateral vestibulopathy is characterized with unsteadiness and oscillopsia when walking or standing, worsening in darkness and/or on uneven ground. To establish the effect of customized vestibular rehabilitation in bilateral vestibulopathy, we analyzed the questionnaires and functional status before and after treatment. METHODS: Among 53 patients with customized vestibular rehabilitation from January 1st to November 30th in 2018, 6 patients (3 males; median age, 71 years; range, 54–75 years) who regularly exercised with good compliance were retrospectively enrolled. They were educated and trained the customized vestibular rehabilitation once a month or two by a supervisor during 40 minutes, and then exercised at home for 30 minutes over 5 days in a week. Dizziness handicap inventory (DHI), Korean vestibular disorders activities of daily living scale (vADL), Beck's depression index (BDI), test for dynamic visual acuity (DVA), and Timed Up and Go test (TUG) were performed before and after the customized vestibular rehabilitation. RESULTS: The patients exercised for median 5.5 months (range, 2–10 months) with the customized methods of vestibular rehabilitation, which included gaze and posture stabilization and gait control exercises. DHI score and TUG was improved after rehabilitation (DHI before vs. after rehabilitation=33 vs. 16, p=0.027, TUG before vs. after rehabilitation=12 vs. 10, p=0.026). BDI, DVA, and vADL scores did not differ between before and after treatment. CONCLUSIONS: Customized vestibular rehabilitation can improve dizziness and balance state in bilateral vestibulopathy. The steady exercises adapted individual peculiarities is the most important for vestibular rehabilitation.

Opsoclonus-Myoclonus Syndrome Associated with Scrub Typhus / 대한평형의학회지

Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus-myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15°–20° and a frequency of 6–8 Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and cerebrospinal fluid analysis revealed pleocytosis. Brain magnetic resonance imaging was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.

Episodic Ataxias: Clinical and Genetic Features

Episodic ataxia (EA) is a clinically heterogeneous group of disorders that are characterized by recurrent spells of truncal ataxia and incoordination lasting minutes to hours. Most have an autosomal dominant inheritance pattern. To date, 8 subtypes have been defined according to clinical and genetic characteristics, and five genes are known to be linked to EAs. Both EA1 and EA2, which are caused by mutations in KCNA1 and CACNA1A, account for the majority of EA, but many patients with no identified mutations still exhibit EA-like clinical features. Furthermore, genetically confirmed EAs have mostly been identified in Caucasian families. In this article, we review the current knowledge on the clinical and genetic characteristics of EAs. Additionally, we summarize the phenotypic features of the genetically confirmed EA2 families in Korea.

Multiple Cerebral Infarction in Cogan's Syndrome / 대한평형의학회지

Cogan's syndrome is a rare chronic inflammatory disease Characterized by non-syphilitic keratitis and vestibuloauditory dysfunction. Although the precise pathogenesis of Cogan's syndrome is unknown, it is thought to develop from vasculitis involving multiple organ system. It can be accompanied with various systemic diseases including arthritis, lymphadenopathy, splenomegaly, and aortitis with insufficiency. We report a case of typical Cogan's syndrome with multiple cerebral infarctions.

Saccadic Palsy after Cardiac Surgery: Serial Neuroimaging Findings during a 6-Year Follow-Up

BACKGROUND: Patients who develop horizontal and vertical saccadic palsy after cardiac surgery have rarely been described. Although most such patients exhibit distinct neurological deficits, their brain MRI findings are almost normal. In addition, functional neuroimaging of such patients has never been reported. CASE REPORT: A 43-year-old woman with dysarthria, dysphagia, and horizontal and vertical saccadic palsy after cardiac surgery was followed up for about 6 years; serial brain MRIs has been performed during this period, including susceptibility-weighted imaging (SWI) and [18F]-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET). Multiple microbleeds in the cerebral cortex, cerebellum, and brainstem, and glucose hypometabolism in the brainstem, cerebellum, and multiple cortical areas. CONCLUSIONS: To the best of our knowledge, this is the first reported case of saccadic palsy after cardiac surgery with serial SWI and [18F]-FDG-PET performed to explore the possible cerebral lesions.

Cerebellar Control of Saccades / 대한임상신경생리학회지

Saccades are rapid eye movements that shift the line of sight between successive points of fixation. The cerebellum calibrates saccadic amplitude (dorsal vermis and fastigial nucleus) and the saccadic pulse-step match (flocculus) for optimal visuo-ocular motor behavior. Based on electrophysiology and the pharmacological inactivation studies, early activity in one fastigial nucleus could be important for accelerating the eyes at the beginning of a saccade, and the later activity in the other fastigial nucleus could be critical for stopping the eye on target, which is controlled by inhibitory projection from the dorsal vermis. The cerebellum could monitor a corollary discharge of the saccadic command and terminate the eye movement when it is calculated to be on target. The fastigial nucleus and dorsal vermis also participate in the adaptive control of saccadic accuracy.

Recurrent Vertigo in Vestibular Schwannoma Responsive to Oxcarbazepine / 대한평형의학회지

Vestibular schwannoma (VS) are benign neoplasms that arise from Schwann cells of the eighth cranial nerve. Although progressive unilateral hearing loss with dizziness or disequilibrium provides a high suspicion index of VS, vertigo is the symptom causing the most pronounced negative effect on quality of life in patients with VS. We report a 55-year-old woman with recurrent paroxysmal vertigo and hyperventilation-induced nystagmus due to VS, which improved by oxcarbazepine treatment. We suggest that episodic vertigo in VS may be ascribed to the ectopic paroxysmal neuronal discharge from the partially demyelinated vestibular nerve due to tumor compression.

Isolated Body Lateropulsion as a Presenting Symptom of Lateral Medullary Infarction / 대한평형의학회지

Body lateropulsion is a common manifestation of lateral medullary infarction (LMI), and usually associated with vertigo, limb ataxia, sensory disturbance, and Horner's syndrome. However, isolated body lateropulsion as a presenting symptom of LMI is rare, and the responsible lesion for lateropulsion remains uncertain. We report a 71-year-old woman who showed isolated body lateropulsion as a presenting symptom of LMI. Ipsilateral body lateropulsion in our patient may be ascribed to the involvement of the ascending dorsal spinocerebellar tract rather than the descending lateral vestibulospinal tract, which runs more ventromedially.

Moyamoya Disease Associated with Optic Disc Coloboma

Moyamoya disease is a cerebrovascular disorder characterized by progressive stenosis of the distal internal carotid arteries, and in rare cases can occur in association with optic disc coloboma. We describe a 31-year-old man with transient left-sided weakness caused by steno-occlusion of the bilateral internal carotid arteries. A fundoscopic examination revealed a coloboma involving the optic disc of the right eye. Clinicians should be aware of the possibility of moyamoya disease in patients with optic disc coloboma, even when other classic symptoms of this disease are absent.

The Clinical Characteristics and Endovascular Management Outcomes of Dural Carotid Cavernous Fistulas

PURPOSE: To describe the clinical characteristics and endovascular management outcomes of Dural carotid cavernous sinus fistula (Dural CCF). METHODS: Ocular symptoms, signs, and complications of 15 eyes from 14 patients confirmed with Dural CCF by angiography were examined. The medical records of patients who underwent endovascular management were analyzed retrospectively. RESULTS: The mean age of the patients was 50.6 +/- 14.2 years; 4 were men and 10 were women. The eye consisted of 7 right eyes and 8 left eyes. Two eyes were of hypertension patients. The chief clinical symptoms on the first visit were diplopia, injection, ocular pain, proptosis, ptosis, and tinnitus. On cerebral angiography, Barrow Type D was the most common consisting of 12 cases (80%). Endovascular embolization was performed in 13 eyes and the average number of times received was 1. Signs and symptoms improved over a mean of 4.5 months of clinical follow-up in 12 eyes. There were no ocular complications related to endovascular embolization. CONCLUSIONS: Dural CCF should be suspected in a middle-aged woman with injection or diplopia. Endovascular embolization is considered minimally invasive and highly successful for the treatment of Dural CCF.

A Case of Bilateral Horizontal Semicircular Canal Dysplasia with Normal Cochlea: Three Dimensional Volume Rendering Image Using MR CISS Sequence / 대한평형의학회지

Congenital inner ear malformations are frequently found in patients with sensorineural hearing loss, but isolated vestibular anomalies with normal cochlear development have been rarely reported. We report a 20-year-old man with recurrent dizziness and disequlibrium without hearing impairment. Neuro-otological evaluations showed a left peripheral vestibulopathy with normal hearing function. Three dimensional volume rendering image using magnetic resonance constructive interference in steady state sequence demonstrated isolated vestibular anomalies involving bilateral horizontal semicircular canals. Isolated vestibular anomalies might not be as rare as previously thought among patients with recurrent dizziness, and should be carefully evaluated through various imaging techniques.

Unilateral Sensorineural Hearing Loss in Cerebral Venous Thrombosis

A 24-year-old woman presented with headache and left-sided tinnitus. Evaluations demonstrated left sensorineural hearing loss and cerebral venous thrombosis (CVT) with normal intracranial pressure. Unilateral hearing loss has rarely been reported in patients with CVT. It is thought that the unilateral hearing loss in this case was caused by impaired drainage of the cochlear veins into the lateral sinus.

Hereditary Episodic Vertigo / 대한평형의학회지

No abstract available.

Bilateral Internuclear Ophthalmoplegia Following Spontaneous Subarachnoid Hemorrhage

A 44-year-old woman presented with horizontal diplopia 2 days after spontaneous subarachnoid hemorrhage. Neurological examination disclosed bilateral internuclear ophthalmoplegia and neck stiffness. Neurovascular imagings documented subarachnoid and intraventriclar hemorrhage with an aneurysm arising from the left superior cerebellar artery, and vasogenic edema in the bilateral pontomesencephalic junction. Parenchymal vasogenic edema due to microvascular damage may give rise to bilateral internuclear ophthalmoplegia during the acute stage of spontaneous subarachnoid hemorrhage.

Bilateral Spontaneous Carotid Artery Dissection With Uncontrolled Graves' Disease

Spontaneous carotid artery dissection is an important cause of ischemic stroke in young patients. There are some genetic or environmental factors associated with risks for spontaneous arterial dissection. Although thyroid hormone may affect the vascular system, the predisposition for arterial dissection remains unknown in thyrotoxicosis. We present a patient with uncontrolled Graves' disease, who developed bilateral internal carotid artery dissections.

Vestibular Neuritis and Bilateral Vestibulopathy

Vestibular neuritis is the second most common cause of peripheral vestibular vertigo. The key signs and symptoms are the acute onset of sustained rotatory vertigo without hearing loss, postural imbalance with Romberg's sign, and peripheral type nystagmus. Head thrust and caloric tests show ipsilateral hyporesponsiveness, but hearing test shows normal. Either an inflammation of the vestibular nerve or labyrinthine ischemia was proposed as a cause of vestibular neuritis. Recovery after vestibular neuritis is usually incomplete. Despite the assumed viral cause, the effects of corticosteroids, antiviral agents, or the two in combination are uncertain. Bilateral vestibulopathy is a rare disorder of the peripheral labyrinth or the eighth nerve. The most frequent etiologies include ototoxicity, autoimmune disorders, meningitis, neuropathies, sequential vestibular neuritis, cerebellar degeneration, tumors, and miscellaneous otological diseases. The two key symptoms are unsteadiness of gait and oscillopsia associated with head movements or when walking. The diagnosis is made with the simple bedside tests for defective vestibulo-ocular reflex (head thrust and dynamic visual acuity tests). Bilateral vestibulopathy is confirmed by the absence of nystagmus reaction to both caloric and rotatory chair tests. The spontaneous recovery is relatively rare and incomplete. Vestibular rehabilitation is supportive of the improvement, but the efficacy of physical therapy is limited.

Tilt Suppression of the Post-rotatory Nystagmus in Cerebellar Nodular Lesions

BACKGROUND AND OBJECTIVES: Head tilt at the end of step rotation about a vertical axis decreases the time constant (TC) of the post-rotatory nystagmus, which is known as tilt-suppression of the vestibulo-ocular reflex (VOR). Tilt suppression of the VOR is mediated by the cerebellar nodulus and ventral uvula and is eliminated after surgical ablation of those structures. However, studies on the tilt suppression of the VOR have been sparse in humans with cerebellar lesions. MATERIALS AND METHODS:Five patients with circumscribed cerebellar lesions involving the nodulusor ventral uvula underwent recording of spontaneous and positional nystagmus, and the VOR. To evaluate tilt suppression of the VOR, the participants pitched their head forward at the end of step rotation about a vertical axis both in the clockwise and counter-clockwise directions. RESULTS: The VOR gain was increased in a patient with infarction in the territory of the medial posterior inferior cerebellar artery while the gain of visually enhanced VOR was normal in all the patients. The time constants of per- and post-rotatory nystagmus was increased in a patient with increased VOR gain and the tilt suppression of the post-rotatory nystagmus was impaired in two patients, either uni- or bilaterally. Spontaneous downbeat and central positional nystagmus were frequently accompanied. CONCLUSIONS: Nodular lesion may impair tilt suppression of the VOR. Measurement of tilt suppressive effect of the VOR may provide a valuable tool for evaluating the nodular dysfunction.