Intracranial dural arteriovenous fistula (dAVF) is characterized by an abnormal connection between branches of arteries and veins in the dura mater. Clinical manifestations of dAVF vary depending on their location, feeder arterial supply, amount of shunting, and most importantly, their venous drainage pattern. Acute vertigo has been rarely reported as an initial presentation of dAVF due to venous congestion in the brainstem. We report a patient who presented with acute right vestibulopathy without any brainstem signs in dAVF involving the transversesigmoid sinus. The patient showed abnormal caloric response but normal head impulse in the affected ear. Without any treatment, the patient’s symptoms gradually improved with a normalization of right canal paresis. Follow-up cerebral angiography also revealed a spontaneous regression of the shunt flow and reduction of venous drainage at the right transverse-sigmoid sinus. Based on the results of vestibular function tests and cerebral angiography, acute vertigo in our patient may be ascribed to impaired reabsorption of endolymph by focal venous congestion.
Seesaw nystagmus (SSN) is characterized by conjugate torsional nystagmus with opposite vertical components in the two eyes. The waveform may be pendular or jerk (hemi-seesaw nystagmus, HSSN), in which the slow phase corresponds to one half-cycle and the quick phase to the other. Pendular SSN and HSSN have distinct clinical presentations and underlying causes. The pathophysiology of pendular SSN may be instability of visuovestibular interactions, while the underlying mechanism for HSSN may be related to the ocular tilt reaction or an imbalance in vestibular pathways. We report a patient with HSSN due to unilateral mesodiencephalic infarction that becomes apparent during visual fixation only.
Citations
Citations to this article as recorded by
Midbrain lesion-induced disconjugate gaze: a unifying circuit mechanism of ocular alignment? Maximilian U. Friedrich, Laurin Schappe, Sashank Prasad, Helen Friedrich, Michael D. Fox, Andreas Zwergal, David S. Zee, Klaus Faßbender, Klaus-Ulrich Dillmann Journal of Neurology.2024;[Epub] CrossRef
Objectives This study aimed to investigate clinical significance of a head-bending test in benign paroxysmal positional vertigo (BPPV) involving the posterior semicircular canal (PC-BPPV).
Methods We retrospectively recruited 256 patients with unilateral PC-BPPV between January 2016 and December 2021, and assessed the clinical characteristics of patients showing head-bending nystagmus (HBN).
Results Of 256 patients, 138 (53.9%) showed HBN. Most patients (n=136, 98.6%) had downbeat nystagmus with (n=38) or without (n=98) torsional component. The remaining two patients had pure upbeat and torsional nystagmus, respectively. The torsional component was directed to the contralesional side in all. Between patients with and without HBN, there were no significant differences in clinical characteristics such as age, lateralization, types of BPPV (canalolithiasis or cupulolithiasis), and success rate of repositioning maneuver.
Conclusions Head-bending test may be useful in predicting the diagnosis and lateralization of PC-BPPV.
Dissociated vertical-torsional nystagmus is a unique form of nystagmus characterized by conjugate torsional but disparate vertical components. It has been mainly reported in internuclear ophthalmoplegia or medial medullary lesion involving the medial longitudinal fasciculus (MLF). The patterns of the nystagmus may be explained by a disruption of vestibulo-ocular reflex pathways from vertical semicircular canal or utriculo-ocular reflex within the MLF, but it is debatable. We described a dissociated upbeat-torsional nystagmus in a patient with vestibular nucleus infarction without involvement of MLF.
The flocculus plays a crucial role in control of eye movements. Based on animal experiment, it is suggested that the flocculus is important for governing vestibuleocular reflexes. In humans, an isolated floccular lesion is extremely rare. We report oculomotor abnormalities in a patient with unilateral infarction of the flocculus, and compare our results with those of previously reported patients with floccular lesion.
The flocculus plays a crucial role in control of eye movements. Based on animal experiment, it is suggested that the flocculus is important for governing vestibule-ocular reflexes (VORs). In humans, an isolated floccular lesion is extremely rare. We report oculomotor abnormalities in a patient with unilateral infarction of the flocculus, and compare our results with those of previously reported patients with floccular lesion.
Spinocerebellar ataxia type 28 (SCA 28) is characterized by young-adult onset, very slowly progressive gait and limb ataxia, dysarthria, nystagmus, ptosis, and ophthalmoplegia. It is caused by a heterozygous pathogenic mutation in the AFG3L2. So far, approximately 80 cases with genetically-confirmed SCA 28 have been reported in the literature. We report a patient with mild gait ataxia and dysarthria who carried a known pathogenic mutation in the AFG3L2. This is the first report of genetically-confirmed SCA 28 in Korea.
Cogan syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly and diarrhea. We report a case of Cogan syndrome accompanied with meningitis.
Cogan’s syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan’s syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan’s syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly, diarrhea. We report a case of Cogan’s syndrome accompanied with meningitis.