Why and how the work of Motor Neurone Disease Associations matters before and during bereavement: a consumer perspective.

Background: Studies on the experiences of consumers with Motor Neurone Disease Associations at end of life and bereavement are lacking, and their role and capability within the broader sectors of health and disability are unknown.

Objectives: To ascertain the experiences and views of bereaved motor neurone disease caregivers with Motor Neurone Disease Associations about service gaps and needed improvements before and during bereavement and to propose a model of care that fits with consumer preferences and where Motor Neurone Disease Associations are effective enablers of care.

Methods: A national bereavement survey was facilitated in 2019 by all Motor Neurone Disease Associations in Australia.

Family carers' experiences of receiving the news of a diagnosis of Motor Neurone Disease: A national survey.

Family carers have a central role in the care and support of people with MND and face the challenges of the disease from diagnosis to progression and the multiple losses of MND, but their support needs are often neglected. This study aimed to investigate the experiences of family carers at the time of diagnosis and their satisfaction with receiving the news. An anonymous postal survey was facilitated by all MND Associations in Australia (2014) and 190 family carers completed the questionnaire.

Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort.

Objective: To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia.

Methods: Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death.

Breaking the news of a diagnosis of motor neurone disease: A national survey of neurologists' perspectives.

Communication of the diagnosis of MND is daunting for patients and neurologists. This study aimed to establish a knowledge base of current Australian practice of breaking the news of an MND diagnosis, to assess the neurologists' educational and training needs and to compare the feedback obtained from neurologists and patients to international practice guidelines. An anonymous survey of neurologists was undertaken in Australia (2014).

Receiving the news of a diagnosis of motor neuron disease: What does it take to make it better?

Our objectives were to identify the experiences of people with MND in receiving the diagnosis and to determine which aspects of breaking this bad news were associated with greater satisfaction with the way the diagnosis was delivered to them. An anonymous postal survey was facilitated by all MND associations in Australia, in 2014, and centred on the SPIKES protocol for communicating bad news. Of the patients (n = 248, response rate 29%), 36% were dissatisfied with the delivery of the diagnosis and gave low ratings on the ability/skills of their neurologists to deliver the diagnosis.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing caused by a pituitary adenoma.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of the prolactinoma and we propose that this is a case of symptomatic SUNCT.

Modeling nitrate leaching and optimizing water and nitrogen management under irrigated maize in desert oases in Northwestern China.

Understanding water and N transport through the soil profile is important for efficient irrigation and nutrient management to minimize nitrate leaching to the groundwater, and to promote agricultural sustainable development in desert oases. In this study, a process-based water and nitrogen management model (WNMM) was used to simulate soil water movement, nitrate transport, and crop growth (maize [Zea mays L.]) under desert oasis conditions in northwestern China.

Intraneural perineurioma.

Intraneural perineurioma is a rare tumour that affects peripheral nerves and, based on its histological features, may be confused with hereditary motor and sensory neuropathies. Detailed neuropathology, including immunoperoxidase stains and electron microscopy, is vital to distinguish these conditions. We report two patients with intraneural perineurioma that demonstrate salient features of this tumour.

Primary Whipple's disease of the brain: characterization of the clinical syndrome and molecular diagnosis.

Background: Whipple's disease (WD) of the brain without evidence of systemic involvement is a rare illness that is difficult to recognize and potentially life-threatening.

Aim: To elucidate the clinical features and diagnosis of primary WD of the brain.

Design: A single case study, with review of published data.

Comparison of laboratory uranium sorption data with 'in situ distribution coefficients' at the Koongarra uranium deposit, Northern Australia.

Distribution coefficients derived from laboratory sorption experiments are commonly used to model the migration of long-lived radionuclides in the environment. However, it has been suggested that field measurements in natural systems ('in situ distribution coefficients') may provide a more accurate indication of 'true' partitioning coefficients than laboratory experiments. In this paper, the relationship between field and laboratory sorption data for uranium is evaluated, using data from the Koongarra uranium deposit in Northern Australia.

Enterovirus associated neurological disease in an HIV-1 infected man.

We report a case of relapsing multifocal neurological disease associated with CNS echovirus 6 infection in an HIV-1-infected individual with no evidence of immunoglobulin deficiency. The illness was initially characterized by optic and cranial neuropathies and myelopathy; concurrent granulomatous hepatitis suggested disseminated viral infection. Treatment with combination nucleoside analogues led to partial remission, but a demyelinating polyneuropathy subsequently developed.

Familial neuronal intranuclear inclusion disease with ubiquitin positive inclusions.

Female monozygotic twins developed upper and lower limb neurogenic weakness in their thirties, followed by cerebellar ataxia, dysarthria and death after an illness duration of about 20 years. Autopsy revealed pathological features typical of neuronal intranuclear inclusion disease (NIID) and positive ubiquitin immunostaining of the inclusions. Two adult sons of one of the twins have now developed an identical illness.

Complete external ophthalmoplegia and asterixis with carbamazepine toxicity.

Ophthalmoplegia is a rarely observed sign in carbamazepine and other anticonvulsant overdoses. We present a patient who developed transient complete external ophthalmoplegia and asterixis with relative preservation of consciousness, in association with carbamazepine toxicity. Previously reported cases and proposed mechanisms are reviewed.

Phenelzine associated peripheral neuropathy--clinical and electrophysiologic findings.

Phenelzine associated sensorimotor peripheral neuropathy is reported in two patients. Symptoms were predominantly sensory, and improvement occurred after withdrawal of phenelzine. Electrophysiologic findings were consistent with an axonal process.

Benign vascular sexual headache and exertional headache: interrelationships and long term prognosis.

There is a definite relationship between the vascular type of benign sexual headache and benign exertional headache. Forty five patients with benign vascular sexual headache were reviewed. Twenty seven (60%) experienced benign vascular sexual headache alone and eighteen (40%) had experienced both benign vascular sexual headache and benign exertional headache on at least one occasion.

Bicycling induced pudendal nerve pressure neuropathy.

Pudendal neuropathies are well recognised as part of more generalised peripheral neuropathies; however, focal abnormalities of the pudendal nerve due to cycling-related injuries have been infrequently reported. We describe two patients who developed pudendal neuropathies secondary to pressure effects on the perineum from racing-bicycle saddles. Both were male competitive athletes, one of whom developed recurrent numbness of the penis and scrotum after prolonged cycling; the other developed numbness of the penis, an altered sensation of ejaculation, with disturbance of micturition and reduced awareness of defecation.

Pituitary apoplexy presenting as aseptic meningitis without visual loss or ophthalmoplegia.

Clinical and spinal fluid evidence of aseptic meningitis were the sole features at presentation in a patient with pituitary apoplexy. Visual impairment and bitemporal field defects developed later. Necrosis within a chromophobe adenoma was found at surgical decompression of the sella.

Loss of facial sweating and flushing in Holmes-Adie syndrome.

We investigated pupillary responses to parasympathetic (pilocarpine) and sympathetic agents (tyramine, cocaine, and phenylephrine) in a 51-year-old woman with tonic pupils, loss of muscle stretch reflexes in the limbs, and hemifacial loss of sweating and flushing (Ross' syndrome). A smaller pupillary response to tyramine and cocaine eyedrops on the symptomatic side indicated that outflow was disrupted in the postganglionic section of the ocular sympathetic pathway. A greater response to phenylephrine eyedrops on this side was consistent with denervation supersensitivity to adrenergic agents.

The utility of testing tactile perception of direction of scratch as a sensitive clinical sign of posterior column dysfunction in spinal cord disorders.

Classical beliefs about the functions of the dorsal columns of the spinal cord have been attacked following recent evidence that position and vibration sensations may be carried in the dorsal spinocerebellar tracts. There is evidence that the one specific function of the dorsal columns is for the transmission of information concerning the direction of tactile cutaneous movement. Thirty normal controls, 43 patients with spinal cord disorders and 10 patients with functional disorders were examined prospectively using an easily administered "direction of scratch" protocol.