Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects.

PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

Sharing the load: How a personally coloured calculator for grapheme-colour synaesthetes can reduce processing costs.

Synaesthesia refers to a diverse group of perceptions. These unusual perceptions are defined by the experience of concurrents; these are conscious experiences that are catalysed by attention to some normally unrelated stimulus, the inducer. In grapheme-colour synaesthesia numbers, letters, and words can all cause colour concurrents, and these are independent of the actual colour with which the graphemes are displayed. For example, when seeing the numeral '3' a person with synaesthesia might experience green as the concurrent irrespective of whether the numeral is printed in blue, black, or red. As a trait, synaesthesia has the potential to cause both positive and negative effects. However, regardless of the end effect, synaesthesia incurs an initial cost when compared with its equivalent example from normal perception; this is the additional processing cost needed to generate the information on the concurrent. We contend that this cost can be reduced by mirroring the concurrent in the environment. We designed the Digital-Colour Calculator (DCC) app, allowing each user to personalise and select the colours with which it displays its digits; it is the first reported example of a device/approach that leverages the concurrent. In this article we report on the reactions to the DCC for a sample of fifty-three synaesthetes and thirty-five non-synaesthetes. The synaesthetes showed a strong preference for the DCC over its normal counterpart. The non-synaesthetes showed no obvious preference. When using the DCC a subsample of the synaesthete group showed consistent improvement in task speed (around 8%) whereas no synaesthete showed a decrement in their speed.

The neuro-otology of Susac syndrome.

OBJECTIVE: We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS: The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. RESULTS: Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss. CONCLUSIONS: Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.

Substantiating synesthesia: a novel aid in a case of grapheme-colour synesthesia and concomitant dyscalculia.

Here we present the case of SP, a 21-year-old female with life-long dyscalculia. SP was subsequently diagnosed with grapheme-color synesthesia, a diagnosis that serendipitously catalyzed our development of a novel aid:The digit-color calculator (DCC). The DCC substantiates SP's color concurrents, dramatically ameliorating her difficulties with basic calculations. We envisage the DCC and its analogues may assist others in educational settings, particularly if they experience difficulties with the acquisition of literacy and numeracy. Further devices that leverage synesthesia may also have the potential to improve the quality of life for others with trait synesthesia regardless of concomitant disorder.

If Time Is Brain Where Is the Improvement in Prehospital Time after Stroke?

Despite the availability of thrombolytic and endovascular therapy for acute ischemic stroke, many patients are ineligible due to delayed hospital arrival. The identification of factors related to either early or delayed hospital arrival may reveal potential targets of intervention to reduce prehospital delay and improve access to time-critical thrombolysis and clot retrieval therapy. Here, we have reviewed studies reporting on factors associated with either early or delayed hospital arrival after stroke, together with an analysis of stroke onset to hospital arrival times. Much effort in the stroke treatment community has been devoted to reducing door-to-needle times with encouraging improvements. However, this review has revealed that the median onset-to-door times and the percentage of stroke patients arriving before the logistically critical 3 h have shown little improvement in the past two decades. Major factors affecting prehospital time were related to emergency medical pathways, stroke symptomatology, patient and bystander behavior, patient health characteristics, and stroke treatment awareness. Interventions addressing these factors may prove effective in reducing prehospital delay, allowing prompt diagnosis, which in turn may increase the rates and/or efficacy of acute treatments such as thrombolysis and clot retrieval therapy and thereby improve stroke outcomes.

Testing the importance of the Medial Temporal Lobes in human interoception: Does it matter if there is a memory component to the task?

Interoception is the ability to consciously perceive internal bodily states. Neuroimaging suggests that the insula (IC) and anterior cingulate cortex (ACC) mediate interoception, while studies involving patients/animals with brain lesions suggest the medial temporal lobe (MTL) is particularly important. One reason for these contrasting conclusions may lie in the types of interoceptive task used by these different approaches. Some tasks probably require integration of current physiological state with mnemonic information (e.g., how much one last ate), and these may be especially reliant upon MTL processing. We compared one task that probably requires integration - a water load task - with one that likely does not - a heart-rate tracking task - in two individuals with selective MTL damage (and with intact IC and ACC). A group of matched healthy individuals served as controls. The main finding was that individuals with MTL damage, relative to controls, were equally and significantly impaired on both types of interoception task. This suggests that MTL structures are involved in mediating interoception even when using a task (heart rate tracking) that does not seemingly require memory and that in neuroimaging studies activates the IC and ACC. The reasons for this apparent inconsistency with neuroimaging findings and the functional role of the MTL in interoception are discussed.

Observer-independent analysis of high-resolution MR images of the human cerebral cortex: in vivo delineation of cortical areas.

Using high-resolution MRI, it is now possible to examine the living human cortex down to a resolution of less than 300 mum. Thus, in vivo imaging is now approaching the resolution that has been successfully used in histological analysis of the cerebral cortex for many years, e.g., low-magnification light microscopy. This allows unprecedented views of cortical microstructure that reflect defined histological features, specifically, individual cortical layers. As in histological brain mapping, it is possible to use the changes in the cortical lamination patterns to define individual cortical areas. This allows in vivo neuroanatomical maps to be generated for individual subjects and precise correlation of the results from functional imaging studies in these subjects with their own microanatomical information. To this end, we adapted the well-established observer-independent cytoarchitectonic mapping techniques for defining cortical borders based on changes in cortical lamination for in vivo parcellation of high-resolution structural MR images.

Real and imaginary rotary motion processing: functional parcellation of the human parietal lobe revealed by fMRI.

Functional magnetic resonance imaging was performed in 16 healthy subjects while they undertook orientation discrimination tasks of real rotating and mentally rotating alphanumeric characters. Perception of rotating and stationary abstract characters was also performed. Mental rotation and the perception of alphanumeric characters undergoing real rotation activated equivalent cortical areas, in keeping with the analogue hypothesis of mental rotation. In addition, areas along the dorsal stream, including the V5/middle temporal complex and the intraparietal sulcus (IPS), were activated during both the real and imaginary rotary conditions. Within the parietal lobe there were areas of convergence (i.e., recruited by all three motion conditions) and areas of divergence (i.e., selectively activated by a particular condition). Tasks requiring canonical-mirror orientation discrimination revealed involvement of neural substrates localized to the ventrolateral bank of the IPS. Tasks in which this judgment was not performed and during which the subject viewed rotary motion of abstract stimuli recruited activity in the medial bank of the IPS. These results indicate subspecialization of the human posterior parietal lobe according to function.

High-resolution MRI reflects myeloarchitecture and cytoarchitecture of human cerebral cortex.

Maps of cytoarchitectonically defined cortical areas have proven to be a valuable tool for anatomic localization of activated brain regions revealed by functional imaging studies. However, architectonic data require observations in a sample of postmortem brains. They can only be used reliably for comparison with functional data as probabilistic maps after spatial normalization to a common reference space. The complete architectonic analysis of an individual living brain has not been achievable to date, because the relationship remains unclear between laminar gray value changes of cerebral cortex in magnetic resonance (MR) images and those of cyto- and myeloarchitectonic histologic sections. We examined intensity profiles through the cortex in five imaging modalities: in vivo T1 and postmortem T2 MRI, one cell body stain, and two myelin stains. After visualizing the dissimilarities in the shapes of these profiles using a canonical analysis, differences between the profiles from the different image modalities were compared quantitatively. Subsequently, the profiles extracted from the in vivo T1-weighted images were estimated from profiles extracted from cyto- and myeloarchitectonic sections using linear combinations. We could verify statistically the mixed nature of the cortical T1 signal obtained in vivo: The MR intensity profiles were significantly more similar to myeloarchitectonic than to cytoarchitectonic profiles, but a weighted sum of both fitted the T1 profiles best.

In vivo identification of human cortical areas using high-resolution MRI: an approach to cerebral structure-function correlation.

Understanding the relationship between the structural and functional organization of the human brain is one of the most important goals of neuroscience. Individual variability in brain structure means that it is essential to obtain this information from the same subject. To date, this has been almost impossible. Even though noninvasive functional imaging techniques such as functional MRI (fMRI) are now commonplace, there is no complementary noninvasive structural technique. We present an in vivo method of examining the detailed neuroanatomy of any individual, which can then be correlated with that individual's own functional results. This method utilizes high-resolution structural MRI to identify distinct cortical regions based on cortical lamination structure. We demonstrate that the observed MR lamination patterns relate to myeloarchitecture through a correlation of histology with MRI. In vivo high-resolution MRI studies identify striate cortex, as well as visual area V5, in four individuals, as defined by using fMRI. The anatomical identification of a cortical area (V5MT) outside of striate cortex is a significant advance, proving it possible to identify extra-striate cortical areas and demonstrating that in vivo structural mapping of the human cerebral cortex is possible.

A role for the thalamus in memory for unique entities.

Recent case studies of amnesic patients have yielded patterns of impairment that challenge Tulving's (1972) division of retrograde memory into two categories (namely, episodic and semantic memory). Here we describe a patient (JG) who developed partially blocked access to previously stored knowledge following the onset of a medial thalamic infarction. Having previously demonstrated that JG has a dense, pervasive amnesia for autobiographical material, famous people and famous events (Miller et al. , 2001), in this study, we show that he is unable to access information about unique entities across a range of domains. In contrast, his memory for more general semantic knowledge (such as word meanings) is intact. JG's memory impairment is discussed in the context of a model of neural functioning put forward by Damasio (1990), in which it was proposed that recall of unique entities depends on many cortical regions being activated in synchrony. This activity, thought to be triggered and coordinated by anterior temporal and frontal lobe regions is, in turn, modulated by projections from the thalamus. We propose that JG's thalamic lesion has disrupted the coordination of the diffuse cortical networks necessary to generate highly specific memories from the past, be they episodic or semantic.

Widespread dorsal stream activation during a parametric mental rotation task, revealed with functional magnetic resonance imaging.

Following a parametrically modulated mental rotation task, in which 10 healthy subjects were instructed to determine whether alphanumeric characters were normal or mirror-reversed, bilateral dorsal stream activations culminating in the intraparietal region were revealed with functional magnetic resonance imaging. Although the parietal activations were bilateral, we observed a right hemispheric dominance for the task, consistent with our previous findings (I. M. Harris et al., 2000). By studying individual activation maps in response to the paradigm, we discerned parcellation of the intraparietal region into discrete subdivisions. In this paper, we address the involvement of structures surrounding the intraparietal sulcus in mental rotation, as well as describing a wider visuospatial attentional network, encompassing neural substrates within the dorsal stream.

Neuropsychological and psychiatric outcomes following coronary surgery or angioplasty: a comparative study.

BACKGROUND: Medical outcomes following coronary artery bypass grafting (CABG) or percutaneous transluminal coronary angioplasty (PTCA) are similar, but few studies have compared neuropsychological outcomes after these procedures. METHODS: A retrospective study compared detailed neurocognitive and psychosocial functioning in 32 patients (CABG, n = 16; PTCA, n = 16) aged 61 +/- 6 years, 9-15 months after coronary revascularisation. Subjects were tested for executive functioning, speed of processing/attention and learning/memory, significant psychopathology (General Health Questionnaire, GHQ) and psychosocial functioning (Short Form (SF)-36 health survey). In the prospective study, 55 patients completed GHQ and SF-36 surveys, the day prior to and 6 months following PTCA. RESULTS: There were no significant differences between the CABG and PTCA groups for neuropsychological or psychosocial end-points (P > 0.20). Executive functioning in both groups, however, was worse than for healthy population controls (P < 0.01). The PTCA patients were significantly more likely than CABG patients to have psychiatric abnormality (GHQ Score >4; P < 0.01). After PTCA, however, there was a significant improvement in the GHQ and SF-36 scores (P < 0.05). CONCLUSIONS: Although executive function is often impaired after coronary revascularisation, neuropsychological status appears equivalent after CABG or PTCA. Psychiatric pathology is common in patients undergoing PTCA, but improves after this intervention.