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Objective Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months. Methods Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters. Results In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months). Conclusion This Study Shows That Psp Patients Experience Falling More Than A Year And A Half An Average Before Receiving A Diagnosis And Although Msa Patients Also Tended To Fall, This Was Much Closer To The Time Of Diagnosis. Further Work With Larger Cohorts May Illustrate Whether These Preliminary Findings Can Be Generalised To Guide Diagnosis And Management.

Hermann Oppenheim described the 'Useless Hand' in 1911 as a classical but uncommon presentation of multiple sclerosis, in which a hand loses useful function due to proprioceptive loss, with relatively preserved motor function. Light touch perception may be subjectively altered or can be relatively intact. The lesion is (usually) a demyelinating plaque in the posterior columns of the cervical spinal cord. Depending on its location, it may affect one limb, or if more central, may produce a bilateral (if asymmetrical) picture. This article reviews a clinical case, historical background, pathophysiology as well as examination tips to aid its recognition.

Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are sporadic, neurodegenerative conditions and belong to a group known as the atypical Parkinsonian disorders. The atypical Parkinsonian (AP) disorders have some features of idiopathic Parkinson's disease (PD) but often with poor or transient levodopa response [1], distinct symptom profiles, more rapid progression and reduced survival. They have significant symptom burden [2]. It is beginning to be appreciated that AP disorders not only carry a heavy symptom burden equivalent to that of patients with advanced cancer but that AP patients experience significant morbidity for an extended period of time. PSP and MSA patients reach clinical milestones such as wheelchair-dependence or frequent falls early in the course of their disease and can live with these accumulating burdens for some years [3]. The acknowledgement of the benefits of emphasizing quality of life in progressive disorders and the growing evidence for palliative care need in non-malignant conditions suggests that MSA and PSP would benefit from an integrated palliative approach. Studies to determine palliative need and possibility of benefit from intervention are beginning to yield results in Parkinsonian conditions. This review aims to discuss the trajectory of disease in MSA and PSP, explore significant symptoms and summarize some evidence which exists for palliative care need and quality of life in these conditions. The challenges of a palliative approach with this group including advance care planning will also be discussed.

ObjectiveAlthough not typical of Parkinson’s disease (PD), caudate dopaminergic dysfunction can occur in early stages of the disease. However, its frequency and longitudinal implications in large cohorts of recently diagnosed patients remain to be established. We investigated the occurrence of caudate dopaminergic dysfunction in the very early phases of PD (123I-FP-CIT single photon emission CT and determined whether it was associated with the presence or subsequent development of cognitive impairment, depression, sleep and gait problems.MethodsPatients with PD and healthy controls were identified from the Parkinson’s Progression Markers Initiative (PPMI) database. We defined a clinically significant caudate dysfunction as 123I-FP-CIT binding ResultsAt baseline, 51.6% of 397 patients had normal caudate dopamine transporter binding, 26.0% had unilateral caudate involvement, 22.4% had bilaterally impaired caudate.Compared with those with a baseline normal caudate function, at the4-year follow-up patients with a baseline bilateral caudate involvement showed a higher frequency of cognitive impairment (pConclusionsEarly significant caudate dopaminergic denervation was found in half of the cases in the PPMI series. Baseline bilateral caudate involvement was associated with increased risk of developing cognitive impairment, depression and gait problems over the next 4 years.

Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are atypical Parkinsonian disorders with extended morbidity and reduced lifespan, known to have marked and early impact upon quality of life (QoL). This study aimed to address the lack of studies in the literature regarding personal perspectives on QoL in MSA and PSP in both patients and carers. Participants took part in qualitative, in-depth interviews in the North East of England, exploring what impacts their QoL and their experiences of living with these complex conditions. Connection to others was found to be a prevailing theme, encompassing difficulty communicating, social isolation, impact on personal relationships, and stigma. This work is helpful in that it emphasises the personal experiences of these patients and carers, which can provide insights into important areas for clinical service planning and best clinical management of individual patients as well as considerations for future research into QoL in these rare disorders.

Nitrous oxide is commonly used as an analgesic and anaesthetic agent. Nitrous oxide is also in use in industry as an aerosol propellant and is now recognised as a recreational drug whose use is growing, especially among the young. Nitrous oxide from whipped cream canisters is inhaled to produce a dissociative, intoxicated state. Nitrous oxide is known to inactivate vitamin B12 via oxidation, which can precipitate a demyelinating myelopathy akin to the classical B12 deficiency syndrome, subacute combined degeneration of the spinal cord. This case describes a young woman with chronic pain and a poor nutritional state who took regular nitrous oxide as an opiate-sparing agent. She developed a progressive subacute myelopathy with a sensory level, profoundly impaired joint position sense, extensor plantars and required a wheelchair. Once diagnosed, she responded well to a regime of nitrous oxide withdrawal, high-dose B12 replacement and physiotherapy. The case illustrates the need for clinical teams to be able to identify a nitrous oxide-precipitated myelopathy as its use as a drug of abuse increases; particularly in the case of malnourished patients who receive nitrous oxide surgically or obstetrically.