Motor neuron disease mimics in practice: a case series.

In the majority of cases of motor neuron disease (MND), diagnosis is clinical and unambiguous. However, given the gravity of the diagnosis, it is crucial that treatable mimics are differentiated accurately. We present three cases referred to our clinic with possible MND with unusual features that led to an alternative diagnosis.

Ruptured intracranial dermoid cysts: a pictorial review.

Intracranial dermoid cysts are rare, benign, congenital, slow-growing cystic lesions. They are composed of mature squamous epithelium and can contain apocrine, eccrine, and sebaceous glands as well as other exodermal structures. Rupture of intracranial dermoid cysts is a relatively uncommon phenomenon but can cause more serious complications such as chemical meningitis, vasospasm, and cerebral infarction.

Computer-aided diagnosis for (I)FP-CIT imaging: impact on clinical reporting.

Background: For (I)FP-CIT imaging, a number of algorithms have shown high performance in distinguishing normal patient images from those with disease, but none have yet been tested as part of reporting workflows. This study aims to evaluate the impact on reporters' performance of a computer-aided diagnosis (CADx) tool developed from established machine learning technology. Three experienced (I)FP-CIT reporters (two radiologists and one clinical scientist) were asked to visually score 155 reconstructed clinical and research images on a 5-point diagnostic confidence scale (read 1).

'Low grade glioma': an update for radiologists.

With the recent publication of a new World Health Organization brain tumour classification that reflects increased understanding of glioma tumour genetics, there is a need for radiologists to understand the changes and their implications for patient management. There has also been an increasing trend for adopting earlier, more aggressive surgical approaches to low-grade glioma (LGG) treatment. We will summarize these changes, give some context to the increased role of tumour genetics and discuss the associated implications of their adoption for radiologists.

Clinical Experience with Pedunculopontine Nucleus Stimulation in Conditions with Nigrostriatal Disconnection.

Background: The pedunculopontine nucleus (PPN) is a part of the mesencephalic locomotor region and, in recent years, it has been considered a new surgical target for deep brain stimulation (DBS) for movement disorders including atypical parkinsonian syndromes such as progressive supranuclear palsy (PSP) and multiple system atrophy. Involvement of the PPN may play an important role in gait impairment in these disorders and the development of PPN DBS could potentially provide treatment for this disabling problem. However, the role of the PPN and the specific pathways involved in gait control and other motor functions are poorly understood.

A review of pathologies associated with high T1W signal intensity in the basal ganglia on Magnetic Resonance Imaging.

With several functions and a fundamental influence over cognition and motor functions, the basal ganglia are the cohesive centre of the brain. There are several conditions which affect the basal ganglia and these have various clinical and radiological manifestations. Nevertheless, on magnetic resonance imaging there is a limited differential diagnosis for those conditions presenting with T1 weighted spin echo hyperintensity within the central nervous system in general and the basal ganglia in particular.

Preservation of passive constructions in a patient with primary progressive aphasia.

Research into agrammatic comprehension in English has described a pattern of impaired understanding of passives and retained ability on active constructions. Some accounts of this dissociation predict that patients who are unable to comprehend actives will also be impaired in the comprehension of passives. We report the case of a man with primary progressive aphasia (PPA) (WR), whose comprehension was at chance on active sentences, but at ceiling on passives.

Complete abolition of reading and writing ability with a third ventricle colloid cyst: implications for surgical intervention and proposed neural substrates of visual recognition and visual imaging ability.

We report a rare case of a patient unable to read (alexic) and write (agraphic) after a mild head injury. He had preserved speech and comprehension, could spell aloud, identify words spelt aloud and copy letter features. He was unable to visualise letters but showed no problems with digits.

Differentiating core and co-opted mechanisms in calculation: the neuroimaging of calculation in aphasia.

The role of language in exact calculation is the subject of debate. Some behavioral and functional neuroimaging investigations of healthy participants suggest that calculation requires language resources. However, there are also reports of individuals with severe aphasic language impairment who retain calculation ability.

Bilateral Homonymous Hemianopia with Sparing of the Vertical Meridian.

A 19-year-old woman, who was 16 weeks post partum, collapsed with loss of consciousness. Following awakening she suffered abrupt loss of vision. Partial recovery occurred, although she has been left with bilateral homonymous hemianopia with sparing of vision just to the left of the vertical meridian.

Restricted diffusion in Wallerian degeneration of the middle cerebellar peduncles following pontine infarction.

Background: Acute Wallerian degeneration following infarction has been show to result in areas of restricted diffusion within the brain. Very few reports describe this appearance in middle cerebellar peduncles.

Case Report: A 37 year old woman was admitted to hospital following sudden collapse and was subsequently found to have a pontine infarct.

Antibody-negative neuromyelitis optica with heavy B-cell infiltration.

There are several distinct clinical phenotypes of inflammatory demyelinating diseases of the central nervous system. In classical multiple sclerosis (MS) there are varied pathological patterns, possibly with differences in pathogenesis. Neuromyelitis optica (NMO) is often associated with a specific antibody, suggesting a distinct pathogenesis.

Effects of sex and age on regional prefrontal brain volume in two human cohorts.

This study examined interactive effects of sex and age on prefrontal brain anatomy in humans. It specifically targeted ranges of the adult life span and regions of cortex that previously showed male-female differences. Participants were 68 healthy human males and females aged 20-72 years.

Dexamethasone and enhancing solitary cerebral mass lesions: alterations in perfusion and blood-tumor barrier kinetics shown by magnetic resonance imaging.

Objective: Glucocorticoid analogues are often administered to patients with intracranial space-occupying lesions. Clinical response can be dramatic, but the neurophysiological response is not well documented. This study sought to investigate the blood-lesion barrier, blood-brain barrier, and cerebral perfusion characteristics of patients who have undergone such therapy using magnetic resonance imaging.

Agrammatic but numerate.

A central question in cognitive neuroscience concerns the extent to which language enables other higher cognitive functions. In the case of mathematics, the resources of the language faculty, both lexical and syntactic, have been claimed to be important for exact calculation, and some functional brain imaging studies have shown that calculation is associated with activation of a network of left-hemisphere language regions, such as the angular gyrus and the banks of the intraparietal sulcus. We investigate the integrity of mathematical calculations in three men with large left-hemisphere perisylvian lesions.

Wernicke encephalopathy: unusual findings in nonalcoholic patients.

We report the unique findings on magnetic resonance imaging (MRI) in two cases of diet-related Wernicke encephalopathy (WE). There was no relation to alcohol in either case. The first patient presented with seizures and showed changes of WE on diffusion-weighted MRI with involvement of the motor strip.

Contrast-enhanced fluid-attenuated inversion recovery imaging for leptomeningeal disease in children.

Background And Purpose: To develop an MR imaging method that improves detection of leptomeningeal disease when compared with the current reference standard, contrast-enhanced T1-weighted imaging.

Methods: We investigated the cases of 10 children who were at high risk of intracranial leptomeningeal disease (Sturge-Weber syndrome and medulloblastoma). The cases of Sturge-Weber syndrome were investigated by using one MR imaging examination, and the cases of medulloblastoma were investigated by using four MR imaging examinations performed over 18 months.

Dural arteriovenous fistulae: noninvasive diagnosis with dynamic MR digital subtraction angiography.

MR digital subtraction angiography (DSA) is a new diagnostic tool capable of producing dynamic images of the cerebral circulation with the injection of gadopentetate dimeglumine into a peripheral vein. Previous reports have concentrated on its potential as a noninvasive technique for the study of pial arteriovenous malformations. In this report, we present our early findings with MR DSA in the evaluation of intracranial dural arteriovenous fistulae.