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.

Predictors of survival in frontotemporal lobar degeneration syndromes.

After decades of research, large-scale clinical trials in patients diagnosed with frontotemporal lobar degeneration (FTLD) are now underway across multiple centres worldwide. As such, refining the determinants of survival in FTLD represents a timely and important challenge. Specifically, disease outcome measures need greater clarity of definition to enable accurate tracking of therapeutic interventions in both clinical and research settings. Multiple factors potentially determine survival, including the clinical phenotype at presentation; radiological patterns of atrophy including markers on both structural and functional imaging; metabolic factors including eating behaviour and lipid metabolism; biomarkers including both serum and cerebrospinal fluid markers of underlying pathology; as well as genetic factors, including both dominantly inherited genes, but also genetic modifiers. The present review synthesises the effect of these factors on disease survival across the syndromes of frontotemporal dementia, with comparison to amyotrophic lateral sclerosis, progressive supranuclear palsy and corticobasal syndrome. A pathway is presented that outlines the utility of these varied survival factors for future clinical trials and drug development. Given the complexity of the FTLD spectrum, it seems unlikely that any single factor may predict overall survival in individual patients, further suggesting that a precision medicine approach will need to be developed in predicting disease survival in FTLD, to enhance drug target development and future clinical trial methodologies.

Current perspectives on neuroimaging techniques used to identify stroke mimics in clinical practice.

INTRODUCTION: Urgent clinical assessment and brain imaging are essential for differentiating stroke mimics from stroke and to avoid unnecessary initiation of reperfusion and other therapies in stroke mimic patients. AREAS COVERED: In this article, the authors will review acute stroke imaging and then the imaging patterns of the most common stroke mimics. The authors have focused our review on brain CT scan, and more specifically CT perfusion, as this is the most commonly available and emerging tool in emergency settings. The authors also provide information on acute brain MRI and MR perfusion. EXPERT OPINION: Imaging can contribute to the detection and diagnosis of acute stroke mimics. Knowledge of imaging findings in different stroke mimics can help distinguish these from patients with stroke who require timely reperfusion therapy. CT and MRI perfusion and diffusion-weighted imaging (DWI) MRI are useful imaging modalities for the assessment of acute stroke patients as they provide more accurate information than plain CT scan. Some of these modalities should be available in the emergency setting. The authors recommended CT perfusion as a useful tool for stroke management and differentiation with stroke mimics.

Chronic inflammatory demyelinating polyradiculoneuropathy presenting as predominantly sciatic monomelic neuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common yet underdiagnosed cause of potentially treatable chronic sensorimotor neuropathy. The classical form of the disease is characterised by symmetrical weakness in both distal and proximal muscle groups accompanied by sensory dysfunction and diminished tendon reflexes lasting more than 2 months. METHOD: The diagnosis of CIDP is supplemented by electrodiagnostic studies and biopsy findings confirming demyelination, in accordance with well-established diagnostic criteria. Atypical presentations of CIDP often pose a diagnostic challenge. RESULTS: In this paper, we present a case of isolated lower limb involvement due to CIDP to raise awareness of this focal lower limb variant. Of particular, significance is the use of lumbosacral plexus MRI to assist in the diagnosis. CONCLUSION: Focal CIDP is an atypical presentation that should be considered in patients presenting with chronic monomelic neuropathy and should be investigated with electrodiagnostic studies, lumbar puncture, nerve biopsy and MRI of the nerve roots and plexuses.

Progressive Camptocormia With Head Drop and Dysphagia.

A 72-year-old man had mild proximal weakness that developed into progressive camptocormia, head drop, numbness, and significant muscle wasting. What is your diagnosis?

Migration of Self-Introduced Acupuncture Needle into the Brainstem.

Acupuncture-related injuries to the central nervous system are a rare but well-documented occurrence. This report describes the case of a self-introduced acupuncture needle migrating into the brainstem following an initial failed attempt at surgical extraction. The patient displayed no neurological deficits, and the needle was eventually successfully removed under direct vision intraoperatively. We discuss the role of various imaging modalities in pre- and post-operative assessment of penetrating foreign bodies in the brainstem. We also discuss the options available for the management of such foreign bodies, including possible approaches for operative intervention, and the risks involved with both surgical and conservative management.

Grey and white matter correlates of recent and remote autobiographical memory retrieval--insights from the dementias.

The capacity to remember self-referential past events relies on the integrity of a distributed neural network. Controversy exists, however, regarding the involvement of specific brain structures for the retrieval of recently experienced versus more distant events. Here, we explored how characteristic patterns of atrophy in neurodegenerative disorders differentially disrupt remote versus recent autobiographical memory. Eleven behavioural-variant frontotemporal dementia, 10 semantic dementia, 15 Alzheimer's disease patients and 14 healthy older Controls completed the Autobiographical Interview. All patient groups displayed significant remote memory impairments relative to Controls. Similarly, recent period retrieval was significantly compromised in behavioural-variant frontotemporal dementia and Alzheimer's disease, yet semantic dementia patients scored in line with Controls. Voxel-based morphometry and diffusion tensor imaging analyses, for all participants combined, were conducted to investigate grey and white matter correlates of remote and recent autobiographical memory retrieval. Neural correlates common to both recent and remote time periods were identified, including the hippocampus, medial prefrontal, and frontopolar cortices, and the forceps minor and left hippocampal portion of the cingulum bundle. Regions exclusively implicated in each time period were also identified. The integrity of the anterior temporal cortices was related to the retrieval of remote memories, whereas the posterior cingulate cortex emerged as a structure significantly associated with recent autobiographical memory retrieval. This study represents the first investigation of the grey and white matter correlates of remote and recent autobiographical memory retrieval in neurodegenerative disorders. Our findings demonstrate the importance of core brain structures, including the medial prefrontal cortex and hippocampus, irrespective of time period, and point towards the contribution of discrete regions in mediating successful retrieval of distant versus recently experienced events.