Practical Neurology recent issues

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Hypoglossal palsy from an atlanto-axial synovial cyst

A 63-year-old man had a 5-week history of a constant right-sided pulsating headache that was initially temporo-parietal but migrated to the suboccipital area. This was associated with new-onset of dysarthria. On examination, there was deviation of the tongue to the right, and the right-side had a wasted surface appearance, despite what he described as ‘wave-like’ prominence when looking at his tongue in the mirror (figure 1A). The remaining examination was normal. Routine laboratory tests and erythrocyte sedimentation rate were normal. MR scan of brain and internal auditory meatuses showed a cystic lesion in the right hypoglossal canal, appearing to abut the right 12th cranial nerve and to extend from the atlanto-axial joint (figure 2A and B). The right side of the tongue appeared enlarged and hyperintense on imaging (figure 2C and D), suggesting muscle fatty infiltration rather...

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Acute leukoencephalopathy in an adult

A 35-year-old unmarried man living alone was brought to the emergency department with acute abdominal pain mainly in the epigastric region. He could not provide any detailed history regarding his presenting complaint but had no fever or other constitutional symptoms. He was known to have diabetes mellitus, but we had no information on any other medical history. Following admission to hospital, his Glasgow Coma Scale score deteriorated rapidly over 24–48 hours from 14/15 to 4/15, and he was intubated and ventilated. His pupils were symmetrical at 4 mm in diameter and were slowly reactive. Deep tendon reflexes were globally diminished with reduced muscle tone, and plantar responses were down going. The remaining neurological examination including cranial nerve function and optic fundi was normal, and there was no neck stiffness. His blood pressure on admission was 190/100 mmHg with a pulse rate of 90 beats per minute. The random plasma...

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Highlights from this issue

The COVID-19 pandemic has changed so much that it is easy to forget what life was like before it began. We already experience a slight shock on seeing pictures of large groups of people standing close to or even touching one another, before realising it is an old photo. We are also becoming accustomed to being ignored by colleagues because of a low-level mask-induced prosopagnosia. Too much change; we can have too much change. However, we must change if we are to improve the care of patients with neurological disorders. Practical Neurology aims to publish papers to help us make those changes. During the pandemic, most neurologists are undertaking remote consultations and working out how best to do this. However, for neurologists working in isolated areas, this approach is not new; Callum Duncan and Angus MacLeod have provided a remote consultation service to the North of Scotland...

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Epileptic: David B

The Edinburgh Neurology Book Club recently read cartoonist David B’s Epileptic, a graphic novel and memoir about a childhood hijacked by his brother JC’s epilepsy and his parents’ search for an effective treatment. It is an odyssey through conventional and complementary medicine and a searing critique of both. The family repeatedly uproots as JC is subjected to a host of treatments, including novel drugs, macrobiotic communes and psychics. None provides more than brief relief from seizures. As the years pass he lapses into obesity, cognitive decline and scarring from his injuries, while David goes his own way. This novel is deliberately provocative, and the club’s discussion was heated. David’s childhood draws sharp parallels between the ‘real’ doctors and the quacks, with their cult-like devotees exalting their ineffective treatments with religious fervour. We felt the patients’ best interests seemed an afterthought; the motivation of these ‘healers’ being a blend of...

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Grand rounds: a precious resource to be nurtured

With the sad news of the death of neurologist Gerald Stern, it is a fitting time to recall his treatise in praise of Grand Rounds.1 This traditional style of learning has long been cherished by neurologists as the optimal way to teach both the clinical method and deductive reasoning.2 Medical education has significantly evolved, with the recognition of varying learning styles, availability of simulations and remote e-learning. Case presentations and the clinicopathological conference are among the most popular sessions in feedback from the Association of British Neurologists’ annual meeting, but the tradition of regional hospital-based Grand Rounds is no longer widespread. Attendance struggles with increasingly squeezed job plans, and the educational environment itself has been open to criticism. Grand Rounds evolved as a concept around the turn of the 20th century from the bedside teaching pioneered by Sir William Osler, but with exemplars...

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Carphology

GREEN LIGHT: STOP? What may be the societal implications of legalising cannabis? The state-by-state variation in the legal status of cannabis in the USA allows for comparison. Using the 2018 roadway fatality data, researchers were able to identify an increase 2.1 (95% CI 1.2 to 2.9; p<0.001) in traffic fatalities in the first four states to legalise the herb. Confounders may abound, but try as they might to correct for important factors, the team could not remove the suspicion that drug-driving may be partially to blame. JAMA Intern Med 2020;e201769. SOD1 OFF The rise of the antisense oligonucleotides continues—this time a phase 1–2 trial of intrathecal tofersen for SOD1-associated amyotrophic lateral sclerosis. Fifty patients were randomised in this safety and pharmacokinetics study, but cerebrospinal fluid concentrations of SOD1 were also assayed. The SOD1 concentration reduced in a dose-dependent manner, 33% lower at day 85 in patients...

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Simple visual hallucinations and epilepsy

Jasvir Virdee and Susan Mollan describe a patient presenting with photopsia who had an unusual ophthalmological diagnosis. Positive and/or negative visual phenomena may relate to pathology anywhere from the eye to the visual association cortex; thus, neurologists and ophthalmologists share responsibility for diagnosing and treating these symptoms. The authors describe how they arrived at an ophthalmological diagnosis for their patient’s photopsia; here I briefly discuss its neurological aspects, especially relating to epilepsy. The occipital lobes are the smallest lobes of the brain, approximately 13% of brain volume. Even so, occipital epilepsy still seems relatively uncommon, especially compared with that from the temporal lobes (20% of brain volume). Panayiotopoulos found occipital epilepsy to comprise only 4.6% of a large series of childhood epilepsy1 and case series of patients undergoing epilepsy surgery for refractory epilepsy have only very small numbers.2–4 The...

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ABN news

ABN RESPONSE TO CUMBERLEGE REPORT Following the July publication of the Cumberlege review, the ABN met with Dr June Raine (Chief Executive of the MHRA) and colleagues to discuss next steps including the possibility of developing a valproate register. The MHRA team were very well briefed on the topic. ABN explained the need for research to better understand the information given to women who become pregnant while taking valproate, and the basis for decisions made by clinicians and patients. It was agreed that the ABN would link with the MHRA team with recommended areas of research and database requirements to be discussed between MHRA and NIHR/NHS Digital. ABN: THE VIRTUAL EXPERIENCE The ABN Annual Meeting, rebranded for 2020 as ‘The Virtual Experience’ opened on 7 September and will run online until 16 November. To register, visit https://www.theabn.org/page/virtual_meeting_2020. Live sessions will be...

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Management of incidental unruptured intracranial aneurysms

The widespread use of MR has led to the increasingly frequent diagnosis of unruptured incidental intracranial aneurysms. Most are small (<7 mm diameter) and will never rupture. Yet, their recognition causes much anxiety, and their optimal management remains controversial. This review addresses the difficulties in managing incidental unruptured saccular intracranial aneurysms. Note that our conclusions and recommendations do not apply to symptomatic unruptured aneurysms or to fusiform, dissecting, mycotic, traumatic and paediatric aneurysms, each of which has a different natural history.

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Cerebral venous thrombosis: a practical guide

All neurologists need to be able to recognise and treat cerebral venous thrombosis (CVT). It is difficult to diagnose, partly due to its relative rarity, its multiple and various clinical manifestations (different from ‘conventional’ stroke, and often mimicking other acute neurological conditions), and because it is often challenging to obtain and interpret optimal and timely brain imaging. Although CVT can result in death or permanent disability, it generally has a favourable prognosis if diagnosed and treated early. Neurologists involved in stroke care therefore also need to be aware of the treatments for CVT (with varying degrees of supporting evidence): the mainstay is prompt anticoagulation but patients who deteriorate despite treatment can be considered for endovascular procedures (endovascular thrombolysis or thrombectomy) or neurosurgery (decompressive craniotomy). This review summarises current knowledge on the risk factors, diagnosis, treatment and prognosis of CVT in adults, and highlights some areas for future research.

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