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Biomedical subjects

Robert W Baloh

Publications and source records attributed to Robert W Baloh.

At least 19 recordsLinked to original sources
PubMed2006

Body lateropulsion as an isolated or predominant symptom of a pontine infarction.

BACKGROUND: Lateropulsion of the body--that is, falling to one side--is a well-known clinical feature of stroke in the posterior circulation. Body lateropulsion as an isolated or predominant manifestation of a pontine stroke has not been reported previously. OBJECTIVE: To elucidate the possible mechanisms of patients presenting with body lateropulsion as an isolated or predominant symptom of an isolated pontine infarction. METHODS: Between May 2004 and February 2006, out of 134 patients admitted with an isolated pontine stroke, we identified 8 (6%) consecutive patients in the Keimyung University Stroke Registry who had body lateropulsion as the main presenting symptom. RESULTS: All lesions were localised to the paramedian tegmentum just ventral to the fourth ventricle. All except one showed a uniform pattern of body lateropulsion, in which the direction of falling was away from the side of an infarct. In two patients body lateropulsion was the sole clinical manifestation, whereas the other patients had other neurological signs. All but one patient had contraversive tilting of the subjective visual vertical (SVV). In all cases, the direction of SVV tilt corresponded to the direction of body lateropulsion. The mean net tilt angle was 6.1 degrees. CONCLUSIONS: Based on the known anatomy of ascending vestibular pathways, SVV tilting and MRI findings, it is concluded that body lateropulsion probably results from damage to the graviceptive pathway ascending through the paramedian pontine tegmentum.

Accidental Falls↗
PubMed2006

Increased risk of death in community-dwelling older people with white matter hyperintensities on MRI.

BACKGROUND: Previous studies in subjects with a history of stroke have shown that white matter hyperintensities (WMH) on MRI are associated with increased risk of death. However, it has not been determined whether WMH are independently related to death in community-dwelling older people without stroke. METHODS: In a sample of community-dwelling people over 75 years with no history of stroke or other neurological diseases, WMH on brain MRI T2-weighted sequences were classified as grade 0, grade 1, or grade 2. Grade 2 WMH were identified in 36 subjects. Age- and sex-matched grade 0 and grade 1 WMH groups were selected for comparison to the grade 2 WMH group. All subjects underwent an initial clinical evaluation and were followed for a median of 11.8 years (interquartile range=10.7 to 12.2 years). Cox proportional-hazards analysis was used to determine the independent association between WMH and time to death from any cause. RESULTS: In an unadjusted analysis, grade 2 WMH was associated with death from any cause (hazard ratio=1.98; 95% confidence interval=1.06, 3.70). After adjustment for hypertension, high cholesterol, diabetes, and coronary artery disease, grade 2 WMH remained significantly associated with death (hazard ratio=2.31; 95% confidence interval=1.21, 4.40) in these age- and sex-matched groups. CONCLUSIONS: Severe WMH increase the risk of death, even in community-dwelling elderly without stroke or other neurological disease, independent of other covariates including hypertension, age, and coronary artery disease.

Age Distribution↗
PubMed2006

Cerebellar infarction in the territory of the medial branch of the superior cerebellar artery.

The authors studied 14 patients with an isolated cerebellar infarct in the territory of the medial branch of the superior cerebellar artery (MSCA). The most common clinical finding was severe gait ataxia with sudden falling (n = 9) or severe veering (n = 2). Cerebellar dysarthria was found in 8 patients. Eight patients had a mild unilateral limb ataxia. These findings emphasize that MSCA territory cerebellar infarction presented with the prominent gait ataxia and cerebellar dysarthria.

Adult↗
PubMed2006

Gentamicin ototoxicity: clinical features and the effect on the human vestibulo-ocular reflex.

CONCLUSIONS: Gentamicin ototoxicity presents with gait imbalance and oscillopsia, but only rarely with hearing loss and vertigo. Sinusoidal rotational stimuli with high accelerations such as the bedside head-thrust test or rotational step changes in velocity are useful to diagnose bilateral vestibulopathy. OBJECTIVE: To describe the salient clinical features and vestibular testing results in gentamicin ototoxicity. PATIENTS AND METHODS: A retrospective review of the quantitative vestibular function testing results for patients presenting to the UCLA Neurotology Clinic with gentamicin ototoxicity over the past 10 years (n=35). RESULTS: All patients presented with imbalance and 33 out of 35 had oscillopsia. Three patients reported a noticeable change in hearing and five reported vertigo. Of the 35 patients, 15 were in renal failure at the time of gentamicin administration. Patients with pre-existing peripheral neuropathy compensated poorly. Sinusoidal rotational testing demonstrated profoundly decreased gain and increased phase lead over the entire frequency range, with a subset of patients having relatively preserved gain at the intermediate frequencies (0.8-1.6 Hz) and low acceleration (<30 degrees/s). There was little or no response to high acceleration step changes in velocity. The time constant measured both by sinusoidal and step responses was ultra-low. All patients tested had a positive head-thrust test bilaterally.

Adult↗
PubMed2006

The personal and scientific feud between Ernst Brücke and Josef Hyrtl.

OBJECTIVE: To describe the events surrounding the personal and professional feud between Josef Hyrtl and Ernst Brücke and its impact on early investigations into the function of the semicircular canals of the inner ear. DATA SOURCES: Published data in scientific journals and news media, documents at the Vienna Institute for the History of Medicine, published personal letters, and an interview with Brücke's great-grandson, Dr. Thomas Brücke. CONCLUSION: Although Hyrtl was instrumental in recruiting Brücke to the University of Vienna, the two professors soon became embroiled in a feud that persisted throughout their academic careers. The difference in approach of these two giants in their field is well illustrated by their views on the function of the semicircular canals of the inner ear. Based on their shape, Hyrtl concluded that they were important for directional hearing, whereas based on animal experiments, Brücke concluded that they were sense organs for equilibrium.

Anatomy, Comparative↗
PubMed2005

A genome-wide linkage scan of familial benign recurrent vertigo: linkage to 22q12 with evidence of heterogeneity.

Benign recurrent vertigo (BRV) is a common disorder affecting up to 2% of the adult population and may be etiologically related to migraine because of similarities in the clinical spectrum of the phenotypes and a high co-morbidity within families. Many families have multiple-affected genetically related individuals suggesting familial transmission of the disorder with moderate to high penetrance. While clinically similar to episodic ataxias, there are currently no genes identified that contribute to BRV and no systematic linkage studies performed. In an initial effort to genetically define BRV, we have selected from our Neurology Clinic population a subset of 20 multigenerational families with apparent autosomal dominant transmission, and performed genetic linkage mapping using both parametric and non-parametric linkage (NPL) approaches. The Affymetrix 10K SNP Mapping Assay was used for the genotyping. Heterogeneity LOD (HLOD) analysis reveals the evidence of genetic heterogeneity for BRV and evidence of linkage in a subset of the families to 22q12 (HLOD = 4.02). An additional region was identified by NPL analysis at 5p15 (LOD = 2.63). As migraine is observed substantially more commonly both within the BRV-affected individuals and the related family members, it is possible that a form of migraine is allelic to the BRV locus at 22q12. However, testing linkage or the chromosome 22q12 region to a broader migraine/vertigo phenotype by defining affectation status as either migrainous headaches or BRV greatly weakened the linkage signal, and no significant other peaks were detected. Thus, BRV and migraine does not appear to be allelic disorders within these families. We conclude that BRV is a heterogeneous genetic disorder, appears genetically distinct from migraine with aura and is linked to 22q12. Additional family and population-based linkage and association studies will be needed to determine the causative alleles.

Chromosomes, Human, Pair 22↗
PubMed2005

Regional estimates of hair cells and supporting cells in the human crista ampullaris.

Regional estimates of type I and type II vestibular hair cells (HC) and supporting cell (SC) numbers were obtained from the horizontal crista ampullaris by using design-based stereology in human. Cristae were microdissected from temporal bones obtained post-mortem (N=16, age range 26-98 years). Three groups were made according to age: group 1, n=5, ages between 26 and 67 years, average age 51 years; group 2, n=4, average age 84 years; and group 3, n=7, average age 94 years. For group 1, the average total HC number was 8,005+/-214, corresponding to 4,119+/-107 type I HC, 3,886+/-117 type II HC, and 10,274+/-224 SC. The type I:type II HC ratio was 1.06+/-0.01, and HC density was 0.80 cells/100 microm2. For group 2, the average total HC number was 7,074+/-489, corresponding to 3,733+/-212 type I HC, 3,341+/-314 type II HC, and 9,321+/-858 SC. The type I:II HC ratio was 1.12+/-0.06, and HC density was 0.75 cells/100 microm2. For group 3, the average HC number was 6,009+/-327, corresponding to 3,380+/-223 type I HC, 2,628+/-235 type II HC, and 10,185+/-182 SC. The type I:II HC ratio was 1.34+/-0.10, and HC density was 0.63 cells/100 microm2. A significant decline in type I, type II, and total HC number and density was found in groups 2 and 3, with individuals exceeding the average human life span.

Adult↗
PubMed2005

Dejerine-Sottas syndrome and vestibular loss due to a point mutation in the PMP22 gene.

We describe a father and daughter with Dejerine-Sottas syndrome and bilateral vestibular loss due to an L71P missense mutation in the peripheral myelin protein 22 (PMP22). The combination of vestibular loss and peripheral neuropathy led to profound imbalance at a young age. It is important to recognize this combination of peripheral nerve and vestibular deficits since rehabilitation strategies and prognosis are different.

Adult↗
PubMed2005

A longitudinal study of oculomotor function in normal older people.

Cross-sectional studies have found declines in most measures of oculomotor function in older subjects compared to young controls, but no prior study has followed the same subjects over time. We measured saccade peak velocity, saccade delay time, smooth pursuit, optokinetic nystagmus (OKN), visual-vestibular-ocular-reflex (VVOR), fixation suppression of the vestibulo-ocular reflex (VOR-fix), and the vestibulo-ocular reflex in 53 subjects (older than 75 years) able to complete at least 9 yearly evaluations. In addition at each visit all patients underwent a complete history and examination, a gait and balance assessment, mini-mental status evaluation, and visual acuity testing. A subset of subjects completed 12 yearly evaluations (14 patients). Despite significant declines of most variables over time, smooth pursuit gain and saccade peak velocity remained stable during the duration of the study both in the 9-year group and the patients completing 12 years. Decline in OKN, VVOR, and VOR were significantly correlated (P<0.001) with decline in the Tinetti gait and balance score, even after controlling for age. In normal healthy older subjects, smooth pursuit and saccade peak velocity are relatively maintained while OKN, VVOR, and VOR function decline. The significant correlation between decline in oculomotor measures and gait and balance measures (even after controlling for age) suggests a common mechanism for the decline in both measures.

Aged↗
PubMed2005

Late-onset pure cerebellar ataxia: differentiating those with and without identifiable mutations.

Late onset cerebellar ataxia can be caused by several genetic mutations but a large percentage of patients remain undiagnosed. Thirty-eight patients with onset of slowly progressive, pure cerebellar ataxia >or=40 years-of-age were identified from a large ataxia database. Their clinical findings and quantitative oculomotor tests were reviewed; all were screened for SCA1, SCA2, SCA3, SCA6, SCA8, SCA14, and the Fragile X premutation (FMR1). All 47 exons of CACNA1A were screened for mutations. Genetic analysis uncovered a mutation in 11 patients. The SCA6 mutation was present in 8 patients (repeats 22-23). Three additional genetic mutations were found: SCA1 (42 repeats), SCA3 (66 repeats), and SCA8 (121 repeats). Patients without identified genetic mutations were characterized by 1) a later age of onset, 2) truncal without extremity ataxia, 3) and down beat nystagmus. Although only a third of these idiopathic late onset ataxia patients had a positive family history, this homogeneous syndrome probably represents a yet to be identified genetic disorder.

Adult↗
PubMed2005

Estimation of the number of nerve fibers in the human vestibular endorgans using unbiased stereology and immunohistochemistry.

The objective of this study was to obtain estimates of the number of nerve fibers in the human crista ampullaris and utricular macula from normal individuals using unbiased stereology and immunohistochemistry. Vestibular endorgans with the attached vestibular nerve stump were microdissected from the temporal bones. Specimens were divided into two groups. The first group (group 1, N = 8, age range, 68-98 years old, mean = 87 years) was fixed with paraformaldehyde and post-fixed with osmium tetroxide. The second group (group 2, N = 5, age range, 80-98 years old, mean = 86.6 years) was fixed with paraformaldehyde, immunoreacted with monoclonal antibodies against neurofilaments, and post-fixed with osmium tetroxide. The endorgans of both groups were embedded in resin and 2-mum thick sections were made. Estimates of the number of nerve fibers were obtained using an unbiased stereological method, the fractionator. The diameter distribution of nerve fibers was also obtained. The average number of fibers in the horizontal, posterior and superior cristae of individuals in group 1 (N = 14 cristae) was 1424+/-320 (CV = 0.22). The average percentage of small (less than 3 microm), medium (between 3 and 5 microm) and large (more than 5 microm) size fibers was 22.4%, 51.5% and 26.1%, respectively. In group 2 (N = 12), there was an average of 1792+/-99 (CV = 0.05) nerve fibers. The average percentage of small, medium and large size fibers was 22%, 51.2% and 26.8%. In the macula utricle from group 1, there was an average of 3026 nerve fibers (N = 2, ages 80 and 96 years old). There was an average 30.75% small, 56% medium and 13.2% large size fibers. In the utricular macula from group 2 (N = 3, ages 84, 92 and 96 years old), there was an average of 3715 nerve fibers. The average percentage of small, medium and large size fibers was 33.2%, 51.7% and 15.1%. The nerve fiber number in both groups is within the range of previous studies, however, the number of fibers in group 2 was significantly higher than that in group 1 (p = 0.01). This difference is likely due to increased sensitivity gained by the immunohistochemical staining of the axoplasm of nerve fibers in group 2. Results from the present study demonstrate the use of unbiased stereology and immunohistochemistry in human vestibular endorgans, as a reliable and efficient method to estimate the number of nerve fibers. These methods can be applied for studies of normal aging and pathological conditions of the vestibular periphery.

Aged↗
PubMed2005

Drop attacks secondary to superior canal dehiscence syndrome.

Two patients with unprovoked drop attacks were found to have dehiscence of the superior semicircular canal on CT of the temporal bone. Both had conductive hearing loss, preservation of stapedius reflex, and abnormal vestibular evoked myogenic potentials. Neither had sound- or pressure-induced nystagmus. Repair of the dehiscence in one case stopped the drop attacks, supporting a causal relationship between the dehiscence and the drop attacks.

Aged↗
PubMed2005

Nonconsensus intronic mutations cause episodic ataxia.

We discovered intronic mutations in two episodic ataxia type 2 (EA2) families: a four-nucleotide GAGT deletion at IVS41+(3-6) and a single nucleotide insertion (insT) at IVS24+3. We expressed minigenes harboring the mutations in cell lines to demonstrate exon skipping from the deletion mutation and the activation of a cryptic splice donor site from the insertion mutation. The identification of these disease-causing mutations expands the spectrum of EA2 mutations and emphasizes the importance of intronic sequences in regulating gene expression.

Adolescent↗
PubMed2005

Three brothers with a very-late-onset writer's cramp.

Writer's cramp (WC) is a form of focal task-specific dystonia, which is brought on by writing. Although most cases are sporadic, a positive family history is present in 5% to 20% of cases. To date, WC has been reported in several families with primary torsion dystonia, including DYT7, a pure focal dystonia, and in the mixed dystonias, DYT1, DYT6, and DYT13. We describe a family of Bulgarian descent with three brothers presenting with a very-late-onset dystonic WC, compatible with linkage to chromosome 18p.

Age Factors↗
PubMed2005

Migraine-associated vertigo.

CONCLUSIONS: It is probably not wise to demand a temporal relationship between migraine symptoms and vertigo for the definition of migrainous vertigo. When recurrent vertigo attacks begin at an early age in a patient with normal hearing and migraine, there are few diagnoses other than migraine that need to be considered. OBJECTIVE: The clinical association between migraine and vestibular symptoms, such as dizziness, motion intolerance and spontaneous attacks of vertigo, is well documented. Recently, investigators have attempted to develop diagnostic criteria for this association. We hypothesized that there are multiple migraine-associated vestibular syndromes and studied a more homogenous subset of them (benign recurrent vertigo). MATERIAL AND METHODS: A structured interview was conducted over the telephone with 40 patients who presented to our neurotology clinic with benign recurrent vertigo and met the International Headache Society criteria for migraine. The structured interview was also conducted with 40 relatives of the patients who reported the same symptoms. RESULTS: A marked female predominance was found. Most of the patients had vertigo attacks lasting minutes or hours and most were completely free of dizziness between attacks. Imbalance and nausea typically accompanied the vertigo. However, in half of the cases, vertigo occurred without an association with headache.

Adult↗
PubMed2005

The Dix-Hallpike test and the canalith repositioning maneuver.

The Dix-Hallpike test and the canalith repositioning maneuver (CRM) are used to diagnose and treat benign positional vertigo (BPV). Dix-Hallpike is the standard procedure for diagnosis of BPV, but if the horizontal canal is not tested for BPV and the Dix-Hallpike is only carried out once, the condition may not be diagnosed and appropriately treated. We describe our method of testing for BPV and treating it with CRM. The Dix-Hallpike test involves rapidly moving the patient from a sitting position to "head hanging," where the patient's head is at least 10 degrees below horizontal. This is performed initially for the posterior semicircular canals. If these movements fail to elicit vertigo and nystagmus, tests of the horizontal semicircular canals are performed by laying the patient on each side. Importantly, if there is no vertigo or nystagmus elicited by testing the horizontal semi-circular canals, the posterior semicircular canals are tested again. It appears that being held in the head hanging positions and then left and right lateral positions will often allow the canaliths to collect such that the Dix-Hallpike test will become positive. Failure to repeat the tests of the posterior semicircular canals may result in a falsely negative test. Testing the horizontal canals and repeating the Dix-Hallpike test will reduce the likelihood of patients undergoing extra testing or other consequences of misdiagnosis. If, during any of this testing, a movement elicits vertigo or nystagmus, the appropriate CRM is then carried out.

Diagnostic Techniques, Otological↗