Nystagmus in a child with nephrotic syndrome.

We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram. A possibility of Wernicke encephalopathy (WE) was considered and the child was started on thiamine supplementation, following which he had significant improvement in his symptoms. His irritability reduced with significant improvement in the range of eye movements and vertical nystagmus. At 3-month follow-up, the child is asymptomatic with normal gait. Although WE is uncommon in children with nephrotic syndrome, the possibility has to be kept in mind when a child presents with atypical neurological symptoms.

Effects of transtympanic intermittent pressure therapy using a new tympanic membrane massage device for intractable Meniere's disease and delayed endolymphatic hydrops: a prospective study.

BACKGROUND: The retrospective study showed that the effect of the middle ear pressure treatment by the tramstympanic membrane massage (TMM) device was similar to that of the Meniett device. OBJECTIVES: The new TMM device named EFET device was prospectively evaluated in patients with Meniere's disease (MD) and delayed endolymphatic hydrops (DEH) and we compared the effects to the Meniett device. MATERIALS AND METHODS: 23 ears of 19 patients were treated with an EFET device, and 17 ears of 15 patients were treated with the Meniett device. All patients suffering from intractable MD and DEH were treated for 4 months. The insertion of a transtympanic ventilation tube was necessary for the Meniett device, but not the EFET device. RESULTS: In patients treated by the EFET and Meniett devices, the frequency of vertigo significantly improved after treatment. The distribution of vertigo outcomes at 4 months after treatment did not differ between patients treated with the both devices. CONCLUSIONS AND SIGNIFICANCE: Middle ear pressure treatment by the EFET device is effective and provides minimally invasive options for intractable MD and DEH like the Meniett device.

Seesaw nystagmus with internuclear ophthalmoplegia from bilateral dorsomedial pons and left thalamus infarction: a case report.

BACKGROUND: We describe for the first time the clinical features and mechanisms of a bilateral dorsomedial pons and left thalamus infarction with seesaw nystagmus and internuclear ophthalmoplegia. CASE PRESENTATION: A 62-year-old Chinese man was hospitalized for sudden-onset dizziness, diplopia, and gait disturbance. A neurological examination revealed seesaw nystagmus and internuclear ophthalmoplegia. Magnetic resonance imaging disclosed an acute infarction confined to the bilateral dorsomedial pons and left thalamus. Subsequently, 2 weeks of antithrombotic therapy led to an improvement in his symptoms. CONCLUSIONS: This case illustrates that the acute onset of seesaw nystagmus and internuclear ophthalmoplegia accompanied by risk factors for cerebrovascular diseases are highly suggestive of brainstem infarction.

Conversion of upbeat to downbeat nystagmus in Wernicke encephalopathy.

OBJECTIVE: To explain (1) why an initial upbeat nystagmus (UBN) converts to a permanent downbeat nystagmus (DBN) in Wernicke encephalopathy (WE) and (2) why convergence and certain vestibular provocative maneuvers may transiently switch UBN to DBN. METHODS: Following a literature review and study of our 2 patients, we develop hypotheses for the unusual patterns of vertical nystagmus in WE. RESULTS: Our overarching hypothesis is that there is a selective vulnerability and a selective recovery from thiamine deficiency of neurons within brainstem gaze-holding networks. Furthermore, since the circuits affected in WE are commonly paraventricular, especially medially, just under the floor of the fourth ventricle where lie structures important for control of vertical gaze, we suggest the patterns of involvement in WE also reflect a breakdown in vulnerable areas of the blood-brain barrier. Many of the initial deficits of our patients improved over time, but their DBN did not. Irreversible changes in paramedian tract neurons, which project to the cerebellar flocculus, may be the cause. Here we suggest that conversion of UBN to permanent DBN points to thiamine deficiency and may argue for a chronic, nonprogressive DBN/truncal ataxia syndrome. Finally, we posit that the transient switch of UBN to DBN reflects abnormal processing of otolith information about linear acceleration, and often points to a diagnosis of WE. CONCLUSION: Recognizing the unusual patterns of transient switching and then permanent conversion of UBN to DBN in WE is vital since long-term disability from WE may be prevented by timely, parenteral high-dose thiamine.

Long-term Visual Acuity, Strabismus, and Nystagmus Outcomes Following Multimodality Treatment in Group D Retinoblastoma Eyes.

PURPOSE: To analyze the long-term visual acuity, strabismus, and nystagmus outcomes in Group D retinoblastoma following multimodality treatments in a national retinoblastoma referral center. DESIGN: Retrospective interventional case series. METHODS: A 13-year retrospective chart review of Group D eyes treated initially with intravenous chemotherapy (IVC) and followed up for at least 1 year from last treatment. Risk factors for final visual acuity (VA) were analyzed, and rate of strabismus and nystagmus at last follow-up visit were calculated. RESULTS: One hundred and four Group D eyes (92 patients) presented to our center during the study period, of which 32 (27 patients) met the inclusion criteria. Following IVC (vincristine, etoposide, and carboplatin), adjuvant treatments included intraophthalmic artery chemotherapy in 5 (16%) eyes, plaque brachytherapy in 5 (16%), transpupillary thermotherapy (TTT) in 18 (56%), and cryotherapy in 24 (75%) eyes. On last examination, 64.41 ± 6.76 months from presentation, mean final VA was 20/283 (logMAR equivalent of 1.15 ± 0.15). On univariate analysis, presentation age, foveal retinoblastoma (at initial examination), use of TTT, and tumor-foveola distance (at last visit) were found to be significant risk factors for worse VA (P < .026). On multivariate analysis, however, only TTT was found to be significant (P = .010). At last visit, 6 of 27 (22%) patients had nystagmus and 12 of 20 (60%) bilaterally salvaged patients had strabismus (n = 10 exotropia and n = 2 esotropia). CONCLUSIONS: After multimodality treatments initiated with IVC, 50% of salvaged Group D retinoblastoma eyes had <20/200 vision, with TTT being a risk factor for worse vision; 60% had strabismus; and 22% had nystagmus.

Nystagmus: an uncommon neurological manifestation of thiamine deficiency as a serious complication of sleeve gastrectomy.

Wernicke encephalopathy--a debilitating acute or subacute neurological disorder-is caused by a deficiency in thiamine (vitamin B(1)). It is characterized by a classical clinical triad of symptoms: ocular impairment, cerebellar dysfunction, and confusion. Although bariatric surgery can certainly improve the overall health of an obese individual, it can also make him or her more susceptible to serious nutrition deficiencies. Following surgery, inadequate caloric intake, rapid and excessive weight loss, food intolerance, lack of adherence to nutrition supplementation, and/or the onset of prolonged vomiting can lead to severe nutrition deficiencies. It is generally believed that the more malabsorptive the surgery proves, the more likely is it that such a deficiency will occur. The case presented here shows that after sleeve gastrectomy (SG), a patient may also develop dangerous nutrition deficits that can negatively affect his or her life. In this particular case, a patient presented with a severe vitamin B(1) deficiency following SG for morbid obesity. Although patients may exhibit pathophysiologies similar to Wernicke encephalopathy after this surgery, only 2 cases of severe vitamin B(1) deficiency following sleeve gastrectomy have been reported. The grave consequences of thiamine deficiency observed in this patient underscore the importance of supplementation after SG.

Evolution of abnormal eye movements in Wernicke's encephalopathy: correlation with serial MRI findings.

A 33-year-old woman with Wernicke's encephalopathy (WE) due to poor oral intake after allogeneic stem cell transplantation for acute myeloid leukemia showed a sequential development of bilateral gaze-evoked nystagmus (GEN), rightward gaze palsy, and upbeat nystagmus. Initial MRIs obtained when she had GEN only showed a lesion involving the medullary tegmentum, and follow-up MRIs revealed additional lesions in the pontine and midbrain tegmentum along with development of rightward gaze palsy, and finally bilateral medial thalamus lesions in association with upbeat nystagmus. The evolution of abnormal ocular motor findings and serial MRI changes in our patient with WE provide imaging evidence on relative vulnerability of the neural structures, and on the progression of lesions and ocular motor findings in thiamine deficiency.

Neurocysticercosis presenting as a vertical one-and-a-half syndrome with associated contralesional horizontal gaze paresis.

We describe a patient presenting with vertical one-and-a-half syndrome and concomitant contralesional horizontal gaze paresis as the result of a solitary neurocysticercosis (NCC) lesion in the right midbrain extending into the thalamomesencephalic junction. The patient received an albendazole-dexamethasone course which resulted in resolution of his symptoms. The neuro-ophthalmological complications of NCC are reviewed and the clinical topography of the neuro-ophthalmological findings of this unusual observation are discussed.

Vitamin B1 in the treatment of Wernicke's encephalopathy due to hyperemesis after gastroplasty.

Wernicke's encephalopathy (WE) is a severe brain disorder, first described in 1881, and is caused by a nutritional deficiency of thiamine (vitamin B1) found mostly in patients suffering from chronic alcoholism. In addition, WE can also complicate bariatric surgery if adequate vitamin supplementation is not insured. Without immediate treatment, the prognosis is poor and the mortality rate is high. Most patients present with atypical neurological symptoms, which hampers rapid diagnosis. We present a 40-year-old woman who underwent gastroplasty combined with gastric banding for severe obesity. She experienced repetitive vomiting and her diet was without vitamin supplementation. After three months she developed convergent strabismus, apathy and urinary incontinence, which was diagnosed as WE and treated as such. Six months later her recovery was incomplete, still showing gait difficulties and nystagmus. We aim to show that adequate vitamin supplementation in patients undergoing gastroplasty is necessary, especially considering the risk of permanent neurological deficits.

Effect of tongue stimulation on nystagmus eye movements in blind patients.

We have observed dramatic effects of tactile tongue stimulation on nystagmus eye movements in patients with acquired blindness, and we report these results. Six adult subjects (3 subjects with light perception or worse vision and 3 normal subjects) were included in this study. Causes of blindness included traumatic explosion, anterior ischemic optic neuropathy, and central retinal artery occlusion. Duration of blindness was 15, 3 and 1.5 years, respectively. A video eye tracking system (Eyelink 1000) was used to record eye movements. The eye movement recording (EMR) was repeated four times in a span of 20 min. Two of the EMRs were performed without tongue stimulation and two with tongue stimulation in randomized order. A tongue stimulus was applied to the surface of the tongue using a Brainport device that produces an electrical tactile stimulus. The nystagmus waveform characteristics and frequency were analyzed. We found that all blind subjects showed continuous jerk nystagmus with slow and quick phases, mainly in horizontal plane in their primary eye positions. The recorded nystagmus waveforms were jerk with linear velocity slow phases. When the tongue stimulus was applied, the frequency of nystagmus was significantly reduced by 47, 40, and 11%, and relative amplitude was reduced by 43, 45, and 6% for three blind subjects, respectively. In conclusion, we think our results that tongue stimulation influences nystagmus eye movements support a link between non-visual sensory input and ocular motor activity.

Neurological complications in hyperemesis gravidarum.

Hyperemesis gravidarum can impair correct absorption of an adequate amount of thiamine and can cause electrolyte imbalance. This study investigated the neurological complications in a pregnant woman with hyperemesis gravidarum. A 29-year-old pregnant woman was admitted for hyperemesis gravidarum. Besides undernutrition, a neurological examination disclosed weakness with hyporeflexia, ophthalmoparesis, multidirectional nystagmus and optic disks swelling; the patient became rapidly comatose. Brain MRI showed symmetric signal hyperintensity and swelling of periaqueductal area, hypothalamus and mammillary bodies, medial and posterior portions of the thalamus and columns of fornix, consistent with Wernicke encephalopathy (WE). Neurophysiological studies revealed an axonal sensory-motor polyneuropathy, likely due to thiamine deficiency or critical illness polyneuropathy. Sodium and potassium supplementation and parenteral thiamine were administered with improvement of consciousness state in a few days. WE evolved in Korsakoff syndrome. A repeat MRI showed a marked improvement of WE-related alterations and a new hyperintense lesion in the pons, suggestive of central pontine myelinolysis. No sign or symptom due to involvement of the pons was present.

The Tullio phenomenon: a neurologically neglected presentation.

The Tullio phenomenon refers to sound-induced disequilibrium or oscillopsia. Patients with this condition frequently present to neurologists, many of whom are unfamiliar with the condition and its diagnostic criteria. Indeed, due to the unusual nature of the symptoms patients are often misdiagnosed as having psychiatric disturbances. Tullio patients describe disequilibrium, auditory and visual symptoms, which are recurrent, brief, and often triggered by loud noises or middle ear pressure changes, e.g. the Valsalva manoeuvre. Many cases are associated with superior semicircular canal dehiscence (SCCD). Early work suggested that the presence of sound-induced torsional eye movements and visual field tilts were consequent upon a utricular-mediated ocular tilt reaction. However, more recent evidence from imaging and oculographic research, as well as data from our patient series indicates that these ocular abnormalities are usually the result of superior semicircular canal stimulation. The clinical history and a focussed examination are often sufficient to make the diagnosis, which can be confirmed with high resolution CT imaging of the temporal bones. In some patients, surgical occlusion or resurfacing of the affected canal can ameliorate symptoms and signs. The aim of this paper is two-fold: Firstly, to review the clinical features of the Tullio phenomenon, and secondly, to highlight our own observations in three cases with a new clinical syndrome consisting of Tullio's phenomenon with bilateral vestibular failure, a pure horizontal nystagmus in response to sound, and no evidence of canal dehiscence.

Acute neurological presentation due to copper deficiency in a hemodialysis patient following gastric bypass surgery.

Acquired copper deficiency has been recently recognized as a cause of myelopathy, and has been reported to occur many years after gastric bypass surgery performed to aid weight reduction in morbidly obese patients. We report a case of a young woman treated by hemodialysis who presented with acute neurological symptoms 5 months after gastric bypass surgery for severe obesity. She had symptoms and signs of cerebellar, spinal cord and peripheral nerve disease, which improved following parenteral copper supplementation. Now that gastric bypass surgery is being offered to morbidly obese hemodialysis patients, this case highlights the importance of monitoring copper levels in hemodialysis patients following gastric bypass surgery.

Upbeat nystagmus changes to downbeat nystagmus with upward gaze in a patient with Wernicke's encephalopathy.

We describe a patient with Wernicke's encephalopathy who showed spontaneous upbeat nystagmus with decelerating slow phases that changed to downbeat nystagmus during upward gaze and increased during downward gaze. He also showed horizontal gaze-evoked nystagmus and impaired upward smooth pursuit. Magnetic resonance imaging demonstrated symmetric lesions involving the bilateral medial thalami, periaqueductal gray matters and inferior cerebellar peduncles. In this patient, the decelerating slow phases and disobedience to Alexander's law of upbeat nystagmus suggest both deficient (leaky) and unstable neural integrators subserving vertical eye motion. Dysfunction of the interstitial nucleus of Cajal or its descending pathway to the vestibulocerebellum via the paramedian tract cell groups may be responsible for the upbeat nystagmus and its modulation by gazes in our patient with Wernicke's encephalopathy.

[Neurootological symptoms in Bruns' syndrome]. / Objawy neurootologiczne w zespole Brunsa.

The aim of the study was to present clinical neurootological symptoms in the case of early stage of the fourth ventricle tumor diagnosed finally on the base of MRI. Visual-oculomotor, vestibular-oculomotor, vestibulo-spinal and other electrophysiological signs of the vestibular damage of the brainstem were: paresis of the sixth nerve unilaterally, prolonged latency of III-V waves on brain stem audiological responses (BERA), asymmetry of the fusion limit of optokinetic nystagmus, bilateral areflexia of the caloric and kinetic labyrinth reaction, abnormal Unterberger's test in craniocorpography, the presence of gaze nystagmus. The study stressed the value of the widen and careful neurootological examination in such cases.

[Computer-aided techniques for the treatment and rehabilitation of patients suffering vertigo and balance disorders].

A new computerized method is proposed for the correction and elimination of undesirable illusory sensations (dizziness), vestibulo-oculomotor (nystagmus), and vestibulo-postural (imbalance) reactions. The method allows to teach the subject about how to avoid generalization of afferent signals over the effector mechanisms in the central nervous system by developing a fixational reflex employing delayed biological feedback for the assessment of efforts being exerted (self-control of training results). Three variants of application of this technique were evaluated depending on the type of stimulation software intended to induce illusory and oculomotor reactions of a defined sensory modality (visual, vestibular or combined). The study involved 30 subjects divided into three groups. They had been taught using the visual (group 1), vestibular (group 2) or combined (group 3) methods. Each group was comprised of an approximately equal number of subjects with vestibulopathies of either peripheral or central origin. The study demonstrated that the proposed approach allows to invoke, with the use of a computer stimulation software, abnormal illusory and vestibulo-oculomotor responses and inhibit them by developing the adequate fixational reflex. Comparative analysis of the results obtained by teaching the patients with the help of the three different methods revealed the dependence of their effectiveness on the level of disturbances in the vestibular system. The visual method of correction proved to be especially efficacious for the patients with peripheral vestibulopathy, and the vestibular methods for the patients with central vestibulopathy. Patients with combined peripheral and central vestibulopathy required the choice of training modalities (either visual or vestibular) on an individual basis.

[Ocular tilt reaction in thalamic infarct]. / Reacción ocular de inclinación en infarto talámico.

Ocular tilt reaction (OTR) includes skew deviation, eye torsion and head tilt. It is usually accompanied by a tilt in the subjective visual vertical. OTR seems to reflect an otolithic dysfunction. This case report shows an OTR of central origin as a result of simultaneous paramedial thalamic and mesencephalon rostral infarcts.