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  •   Highlights from this issue
    Pract. Neurol. Pub Date : 2021-10-01
    Phil E M Smith, Geraint N Fuller

    There is a notion that progress in our knowledge is made gradually with the slow accretion of information, with bricks of information being added slowly to build the edifice of knowledge. Ideas are refined and improved, additional data, whether about the pathogenesis of a disorder or the result of treatment trials, add to the whole. But this is not the only way. Sometimes new ideas arrive and knock

  •   Home seizure detection: a maturing technology
    Pract. Neurol. Pub Date : 2021-10-01
    Mark Manford

    For many people with epilepsy and their carers, the unpredictability of seizures is especially stressful and debilitating, limiting normal social function, including employment. It is also a major cause of morbidity and mortality. Sudden unexpected death in epilepsy (SUDEP) is a particular risk; eliminating sleep-related tonic-clonic seizures and lone sleeping might remove 82% of SUDEP risk.1 Moreover

  •   Progressive supranuclear palsy: diagnosis and management
    Pract. Neurol. Pub Date : 2021-10-01
    James B Rowe, Negin Holland, Timothy Rittman

    Treating patients with progressive supranuclear palsy (PSP) is both effective and rewarding. This review aims to share our experience in the proactive management of PSP, considering the patient, the family and the medical context in which the illness unfolds. There are many opportunities to assist your patients, ameliorate their symptoms, reduce their risks and harm, and guide them through the complex

  •   Depression after stroke
    Pract. Neurol. Pub Date : 2021-10-01
    João Facucho-Oliveira, Daniel Esteves-Sousa, Pedro Espada-Santos, Nuno Moura, Margarida Albuquerque, Ana Margarida Fraga, Sandra Sousa, Pedro Cintra, Luis Mendonça, Fernando Pita

    Stroke treatment has dramatically improved in recent decades. However, although new treatments have reduced its mortality and the severity of its physical and cognitive sequelae, many people still have incapacitating disabilities following a stroke. Depression is the most common psychiatric disorder following stroke; it is important to recognise and treat as it limits motor and cognitive rehabilitation

  •   Trigeminal neuralgia: a practical guide
    Pract. Neurol. Pub Date : 2021-10-01
    Giorgio Lambru, Joanna Zakrzewska, Manjit Matharu

    Trigeminal neuralgia (TN) is a highly disabling disorder characterised by very severe, brief and electric shock like recurrent episodes of facial pain. New diagnostic criteria, which subclassify TN on the basis of presence of trigeminal neurovascular conflict or an underlying neurological disorder, should be used as they allow better characterisation of patients and help in decision-making regarding

  •   Syringomyelia: diagnosis and management
    Pract. Neurol. Pub Date : 2021-10-01
    Graham Flint

    In the pre-MR era syringomyelia often presented late, as a crippling neurological disorder. Today, most cases are diagnosed earlier, with less pronounced deficits. We are therefore presented with new challenges, including understanding the significance of various presenting symptoms, knowing when surgery might help and being aware of other treatments that could benefit someone living with the effects

  •   Autoimmune encephalitis: clinical spectrum and management
    Pract. Neurol. Pub Date : 2021-10-01
    Christopher E Uy, Sophie Binks, Sarosh R Irani

    Autoimmune encephalitis defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. It comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. The rapid and ongoing expansion of this field has been driven by the identification of several pathogenic autoantibodies that cause polysymptomatic

  •   Cryopyrin-associated periodic syndrome: a treatable genetic inflammatory condition
    Pract. Neurol. Pub Date : 2021-10-01
    Maja Christensen, Mathew Wallis, Peter Jessup, Irène Lemelle, Dean L Jones

    A 20-year-old man presented with recurrent subdural haemorrhages on a background of progressive sensorineural hearing loss, juvenile idiopathic arthritis and intracranial hypertension of unknown cause. His mother had a similar previous history. They both had a persistently mildly elevated serum C reactive protein. Repeat lumbar punctures identified persistently elevated intracranial pressure and mild

  •   Iatrogenic cerebral radiation necrosis
    Pract. Neurol. Pub Date : 2021-10-01
    Carolina Maria Helena Hilton, Lena Specht, Eva Loebner Lund, Pernille Christina Martens, Grethe Schmidt, Lars-Henrik Krarup

    Cerebral radiation necrosis is the most serious late reaction to high doses of ionising radiation to the brain, and its treatment is generally unsatisfactory. We present a patient who developed cerebral radiation necrosis after protracted fluoroscopy during repeated embolisations of an extracranial arteriovenous malformation. Treatment with bevacizumab (a humanised murine monoclonal antibody against

  •   Bilateral trigeminal motor nucleus syndrome
    Pract. Neurol. Pub Date : 2021-10-01
    Chulika Makawita, Tharuka Herath, Medini Boteju, Nalin Karunasena, Sivanesan Pratheepa, Sunethra Senanayake

    A 48-year-old man was brought to our unit with deteriorating consciousness but no available history. His Glasgow Coma Scale score was 11/15 (E3, V4, M4). A limited neurological examination identified neck stiffness with normal upper and lower limbs except for extensor plantar responses. Cranial nerve examination was also limited, but with no obvious ophthalmoplegia, facial weakness, wasting of muscles

  •   Amoebic encephalitis mimicking acute disseminated encephalomyelitis
    Pract. Neurol. Pub Date : 2021-10-01
    Yuhei Otowa, Isamu Yamakawa, Nobuhiro Ogawa, Akihiro Kitamura, Hyou Kim, Mitsuru Sanada, Makoto Urushitani

    A 16-year-old Japanese girl presented with left hemiparesis and coma, following a 2-day history of headache and fever. She had been previously well and no history of a skin wound. MR scan of the brain showed a 6 cm lesion in the right frontal white matter with mass effect and slight edge enhancement (figure 1). A craniotomy was performed for external decompression and brain biopsy. The intraoperative

  •   Spinal dural arteriovenous fistula presenting as progressive thoracic myelopathy
    Pract. Neurol. Pub Date : 2021-10-01
    Nabeela Nathoo, Erin F Balcom, Stephen Joza, Thomas Yeo, Cian O'Kelly, Aakash Shetty

    A 51-year-old man had a 4-month history of back pain and progressive leg weakness. The back pain was on the left side, radiating down his left buttock and leg to the dorsum of the foot. He had gradually developed bilateral leg weakness with a right foot drop such that he required a four-wheeled walker. There was accompanying left anterior thigh numbness and saddle anaesthesia, together with urinary

  •   Paraneoplastic opsoclonus-myoclonus-ataxia syndrome secondary to ovarian cancer
    Pract. Neurol. Pub Date : 2021-10-01
    Andrew W McHattie, Diana Wei, Hena Ahmad, Niran Nirmalananthan

    A 63-year-old woman presented as a thrombolysis call with dysarthria, diplopia, vertigo and generalised weakness. She had a history of high-grade serous ovarian cancer, treated 3 years previously with surgery and chemotherapy and believed to be in remission. She also had osteoarthritis of the hips. On examination, there was mild dysarthria, abnormal eye movements in horizontal and vertical planes and

  •   Toluene-induced leukodystrophy from glue sniffing
    Pract. Neurol. Pub Date : 2021-10-01
    Yue Hui Lau, Ahmad Shahir Mawardi, Norzaini Rose Zain, Shanthi Viswanathan

    A 33-year-old man with a history of chronic toluene abuse through glue sniffing, developed tremors, cerebellar signs and cognitive decline. MR scan of the brain showed global cerebral and cerebellar atrophy with symmetrical T2-weighted hypointensities in the basal ganglia, thalami and midbrain. After stopping glue sniffing, his tremors, ataxia of gait, speech and cognition partially improved. Early

  •   Lemierre’s syndrome
    Pract. Neurol. Pub Date : 2021-10-01
    Eng Chuan Foo, Matthew Tanti, Helen Cliffe, Marc Randall

    Lemierre’s syndrome is a rare and potentially life-threatening condition that follows an oropharyngeal infection, typically from Fusobacterium necrophorum, and usually affects healthy adolescents or young adults. The characteristic features are septic thrombophlebitis of the internal jugular vein and septic embolism leading to multiorgan involvement, commonly the brain, lungs and bones. We report a

  •   Pulse-synchronous torsional nystagmus
    Pract. Neurol. Pub Date : 2021-10-01
    Ivan Milenkovic, Thomas Sycha, Evelyn Berger-Sieczkowski, Paulus Rommer, Christian Czerny, Gerald Wiest

    Purely torsional spontaneous nystagmus almost always has a central vestibular cause. We describe a man with spontaneous pulse-synchronous torsional nystagmus in which the clockwise component corresponded to his pulse upswing, in keeping with a peripheral vestibular cause; following imaging we diagnosed left-sided superior canal dehiscence syndrome. Identifying pulse synchronicity of spontaneous nystagmus

  •   Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL)
    Pract. Neurol. Pub Date : 2021-10-01
    Rhea YY Tan, Anna M Drazyk, Kathryn Urankar, Clare Bailey, Stefan Gräf, Hugh Markus, Nicola J Giffin

    A 44-year-old Caucasian man presented with seizures and cognitive impairment. He had marked retinal drusen, and MR brain scan showed features of cerebral small vessel disease; he was diagnosed with a leukoencephalopathy of uncertain cause. He died at the age of 46 years and postmortem brain examination showed widespread small vessel changes described as a vasculopathy of unknown cause. Seven years

  •   Leucoencephalopathy with progressive cerebral atrophy
    Pract. Neurol. Pub Date : 2021-10-01
    Nina Xie, Qiying Sun, Mengchuan Luo, Yafang Zhou

    A 38-year-old man presented to our centre with subacute cognitive decline and right limb weakness. His memory had been normal until 5 months before, initially having difficulty recalling friends’ names. Over the next 3 months, he became sleepy and lacked interest in social activities. Gradually his right limbs became inflexible, interfering with tooth brushing and walking. An MR scan of brain at another

  •   Visual misperceptions
    Pract. Neurol. Pub Date : 2021-10-01
    Ryan Keh, Sarah Al-Bachari, Mohanned Mustafa, Somenath Chatterjee, Neil Watson, Suvankar Pal, Rejith Dayanandan

    A 74-year-old retired male scientist reported visual misperceptions over 3 weeks. These included seeing objects as if through a camera lens, fluctuating brightness despite no change in ambient light, photopsia, light sensitivity, objects appearing closer than they were, horizontal diplopia, altitudinal field loss and ‘looking from the inside outward’. When walking, he felt the floor curved upwards

  •   The Last Act of Love
    Pract. Neurol. Pub Date : 2021-10-01
    Christopher Hutchcroft, Matthew Jones, Tim Lavin, James Lilleker, Jane Molloy, David McKee, Anna Richardson, Rajiv Mohanraj

    The Last Act of Love , Cathy Rentzenbrink’s account of her brother Matty’s traumatic brain injury and consequent permanent vegetative state, was the first non-fiction book read by our neurology book club. This acclaimed memoir charts the family’s 8-year journey, starting with the initial shock of Matty’s accident and hopes for recovery, through a growing acceptance of his irreversible neurological

  •   Carphology
    Pract. Neurol. Pub Date : 2021-10-01
    A Fo Ben

    A Cambridge team have captured the zeitgeist, with a charming paper that leads with a strong pun. Their classifications of videoconferencing-related illnesses are of course, Zoomnotic diseases. This is a rich and well-written paper that AFB urges you to discover for yourself, before a colleague quotes it to you. As a flavour, they describe and define ‘Zychosis’ ‘believing you actually are in the spot

  •   ABN news
    Pract. Neurol. Pub Date : 2021-10-01
    Joanne Lawrence, Jon Sussman

    ![Graphic][1] The ABN will hold its first face-to-face meeting for 2 years on 4 November at the Holiday Inn, Bloomsbury. This will be a hybrid meeting, delegates may register to attend in person or virtually, and recorded sessions will be available to all delegates for 3 months after the event. The theme of the meeting is ‘Facing’ and plenary sessions, include: 1. Facing the issues in 2021 2. Faced

  •   Cerebral lipiodol embolisation
    Pract. Neurol. Pub Date : 2021-09-11
    Lisa Batcheller, Mark Thaller, Ben Wright

    Cerebral lipiodol embolisation is a rare but serious complication of lymphangiography. A man in his seventies had undergone lymphangiography for a refractory chyle leak following oesophagectomy. The day after lymphangiography, his conscious level dropped with bilaterally miotic pupils, increased muscle tone and double incontinence. CT scan of the head showed patchy high density throughout basal ganglia

  •   Paraneoplastic neurological syndromes: a practical approach to diagnosis and management
    Pract. Neurol. Pub Date : 2021-09-11
    Sophie Binks, Christopher Uy, Jerome Honnorat, Sarosh R Irani

    Paraneoplastic neurological syndromes (PNS) are the immune-mediated effects of a remote cancer and are characterised by an autoantibody response against antigens expressed by the tumour. Classically, well-characterised ‘onconeuronal’ antibodies target intracellular antigens and hence cannot access their antigens across intact cell membranes. The pathogenic mediators are likely to be neuronal-specific

  •   HTLV-1 encephalitis
    Pract. Neurol. Pub Date : 2021-08-30
    Josh King-Robson, Timothy Hampton, Carolina Rosadas, Graham P Taylor, Biba Stanton

    A 53-year-old woman developed subacute onset of upper limb weakness, sensory loss and cerebellar dysfunction. She was known to have human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy. MR scan of the brain showed extensive T2 hyperintensity within the deep and subcortical white matter, with punctate contrast enhancement. Cerebrospinal fluid (CSF) was lymphocytic with very high levels of

  •   Bilateral loss of taste from a unilateral thalamic infarct
    Pract. Neurol. Pub Date : 2021-08-25
    Fionn Mag Uidhir, Aravinth Sivagnanaratnam

    Loss of sense of taste (hypogeusia) involving a part of the tongue can follow acute stroke. We describe a woman with a small right thalamic acute infarct causing bilateral (mainly left-sided) hypogeusia. Her problem remains sufficiently severe to cause distress and nutritional deficit. The anatomical distribution of her problem—cheiro-oral syndrome with concurrent hypogeusia—suggested involvement of

  •   Dorsal midbrain (Parinaud) syndrome
    Pract. Neurol. Pub Date : 2021-08-25
    Esha Prakash, Helen J Kuht, Sreemathi Harieaswar, Mervyn G Thomas

    A 52-year-old man presented with sudden onset of vertical diplopia, limited vertical gaze (figure 1), vertical oscillopsia, taste changes and lip numbness. His family had noted left-sided facial changes, with flattening of the nasolabial fold (figure 2). He had no significant medical or family history. He worked as a bus driver, drank 21 units of alcohol per week and had a smoking history of 9 pack-year

  •   What about hypnosis, Doctor?
    Pract. Neurol. Pub Date : 2021-08-20
    Mark Edwards

    ‘What about hypnosis Doctor, do you think that might help?’ Picture the clinical encounter within which this question has arisen, and I predict the following. First, you have reached a point where you, as a health professional, feel that you have nothing else to offer the patient in front of you. And second, the word ‘hypnosis’ could have been replaced by any of the following: osteopathy, acupuncture

  •   Neurolisteriosis presenting with multiple intracerebral haemorrhages
    Pract. Neurol. Pub Date : 2021-08-20
    Aayesha Soni, Lawrence Maskew Tucker

    A 49-year-old man had acute-onset dizziness, vomiting and diplopia. He had taken treatment for HIV infection since 2017, with a CD4 count over 300×106/L (430–1690) and suppressed HIV viral load. His blood pressure was 170/110 mm Hg on presentation though without history of hypertension. On examination, there were cerebellar signs, including bilateral horizontal gaze-evoked nystagmus, dysdiadochokinesia

  •   Longitudinally extensive transverse myelitis: don’t forget syphilis
    Pract. Neurol. Pub Date : 2021-08-19
    Chumpol Anamnart, Jiraporn Jitprapaikulsan

    A 31-year-old man presented with a 2-week history of progressive paraparesis, leg numbness and urinary retention. He had no fever. Neurological examination identified paraplegia with hyper-reflexia in both lower limbs. Pain and proprioceptive sensation were decreased below the T7 level. Magnetic resonance (MR) scan of the spine showed a T2-hyperintense lesion extending from the fourth cervical vertebra

  •   Huntington’s disease: diagnosis and management
    Pract. Neurol. Pub Date : 2021-08-19
    Thomas B Stoker, Sarah L Mason, Julia C Greenland, Simon T Holden, Helen Santini, Roger A Barker

    Huntington’s disease (HD) is an inherited neurodegenerative disease characterised by neuropsychiatric symptoms, a movement disorder (most commonly choreiform) and progressive cognitive impairment. The diagnosis is usually confirmed through identification of an increased CAG repeat length in the huntingtin gene in a patient with clinical features of the condition. Though diagnosis is usually straightforward

  •   Localisation in focal epilepsy: a practical guide
    Pract. Neurol. Pub Date : 2021-08-17
    Fahmida A Chowdhury, Rui Silva, Benjamin Whatley, Matthew C Walker

    The semiology of epileptic seizures reflects activation, or dysfunction, of areas of brain (often termed the symptomatogenic zone) as a seizure begins and evolves. Specific semiologies in focal epilepsies provide an insight into the location of the seizure onset zone, which is particularly important for presurgical epilepsy assessment. The correct diagnosis of paroxysmal events also depends on the

  •   ‘Scan-negative’ cauda equina syndrome: what to do when there is no neurosurgical cause
    Pract. Neurol. Pub Date : 2021-08-13
    Ingrid Hoeritzauer, Biba Stanton, Alan Carson, Jon Stone

    Suspected cauda equina syndrome is a common presentation in emergency departments, but most patients (≥70%) have no cauda equina compression on imaging. As neurologists become more involved with ‘front door’ neurology, referral rates of patients with these symptoms are increasing. A small proportion of patients without structural pathology have other neurological causes: we discuss the differential

  •   Eagle syndrome
    Pract. Neurol. Pub Date : 2021-08-13
    Erika Melissa Báez-Martínez, Lucia Maure Blesa, Beatriz Sobrino Guijarro, Cristina Ordoñez Gonzalez, Inmaculada Navas Vinagre, Maria Araceli García Torres

    A 36-year-old man reported 4 weeks of left-sided headache and neck pain, with left miosis and ptosis. This had started during vigorous exercise, including abrupt cervical movements, and had been constant since. Six years before he had developed three episodes of numbness in the right arm and leg, each lasting around 15 min, thought to anxiety related. He had no cardiovascular risk factors or history

  •   Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS): genetic and clinical aspects
    Pract. Neurol. Pub Date : 2021-08-13
    Andrea Cortese, Riccardo Curro', Elisa Vegezzi, Wai Yan Yau, Henry Houlden, Mary M Reilly

    Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) typically presents in middle life with a combination of neuropathy, ataxia and vestibular disease, with patients reporting progressive imbalance, oscillopsia, sensory disturbance and a dry cough. Examination identifies a sensory neuropathy or neuronopathy and bilaterally impaired vestibulo-ocular reflex. The underlying genetic

  •   Hypnosis
    Pract. Neurol. Pub Date : 2021-08-13
    Wendy Phillips, Jason Price, Paul D Molyneux, Quinton Deeley

    Clinical hypnosis is an important therapeutic tool with an increasingly understood cognitive and neurobiological basis, and evidence for efficacy. Hypnosis involves controlled modulation of components of cognition—such as awareness, volition, perception and belief—by an external agent (the hypnotist) or by oneself (self-hypnosis) employing suggestion. In this article, we describe what hypnosis is,

  •   Visual loss with chronic meningeal and systemic inflammation
    Pract. Neurol. Pub Date : 2021-08-13
    Peter M Fernandes, Lucy Kinton, Stephen Madill, Frederick R Burgess, Euan McRorie, Neeraj Dhaun, Desmond Kidd, Helen J Lachmann, Richard J Davenport, David Hunt

    A 46-year-old man presented with worsening vision in the context of long-standing optic disc changes, bilateral sensorineural hearing loss, chronic aseptic meningitis, rash, arthralgia and raised inflammatory markers. The differential diagnoses of neoplasia, infection and inflammation are discussed. We highlight a condition that can present to adult neurologists, with successful diagnosis leading to

  •   Wall-eyed bilateral internuclear ophthalmoplegia associated with etanercept
    Pract. Neurol. Pub Date : 2021-08-05
    Tychicus Chen

    A 46-year-old man presented with horizontal diplopia over 5 days. He had psoriatic arthritis that had been stable on etanercept for several years but he had interrupted treatment for 3 months for fear of immunosuppression during the coronavirus pandemic. He had restarted therapy 3 months before presentation after worsening arthritis. On examination, he had bilateral exotropia with impaired adduction

  •   Autonomic dysreflexia in spinal cord injury
    Pract. Neurol. Pub Date : 2021-08-05
    Celine Lakra, Orlando Swayne, Gerry Christofi, Manishkumar Desai

    Autonomic dysreflexia is a relatively common condition in people who have a spinal cord injury above the level of T6. It is a potentially life-threatening; without timely and effective treatment, it can have deleterious cardiophysiological and systemic consequences. It is therefore imperative for medical professionals to have a clear understanding of its acute management, and be prepared to provide

  •   Hypertension with recurrent focal deficits
    Pract. Neurol. Pub Date : 2021-08-05
    Apurva Sharma, Ayush Agarwal, Padma Srivastava, Ajay Garg, Roopa Rajan, Anu Gupta, Rohit Bhatia, Mamta Bhushan Singh, MC Sharma, Venugopalan Vishnu

    A 42-year-old businessman had a 27-year history of multiple complaints. He had been born of a non-consanguineous marriage, but his siblings had young-onset hypertension. When aged 15 years, he developed an ulcerated skin lesion on the medial right thigh with right foot drop. This was presumptively treated as tuberculosis with corticosteroids and antituberculous therapy; the skin lesion resolved in

  •   Sensory ataxia with cranial nerve palsies
    Pract. Neurol. Pub Date : 2021-08-05
    Alexander Grundmann, Marc Hardwick, David Ledingham, James Miller

    A previously independent 64-year-old man presented to his local hospital with new-onset diplopia and dysarthria while watching television. Over the following days, he developed painless perioral and upper limb sensory disturbance, gait unsteadiness and limb ataxia, most pronounced in the upper limbs. He was initially admitted and treated for a cerebrovascular event, followed by a period of failed rehabilitation

  •   Bilateral optic neuritis as a first presentation of lymph node tuberculosis
    Pract. Neurol. Pub Date : 2021-07-28
    Ashvini Keshavan, Lisa McAnena, James F Acheson, Helen Booth, Gordon T Plant, Zhaleh Khaleeli

    Tuberculosis (TB) may affect the nervous system in many ways. We describe an immunocompetent teenage girl with lymph node TB who had first presented with bilateral optic neuritis. Detailed history identified features inconsistent with immune-mediated optic neuritis. Several unusual features prompted further investigation, including transient visual obscurations without raised intracranial pressure

  •   Progressive encephalomyelitis with rigidity and myoclonus (PERM)
    Pract. Neurol. Pub Date : 2021-07-28
    Neil Grech, John Paul Caruana Galizia, Adrian Pace

    Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a subtype of stiff-person syndrome (formerly stiff-man syndrome). It is rare and disabling, and characterised by brainstem symptoms, muscle stiffness, breathing issues and autonomic dysfunction. We describe a 65-year-old man who presented with odynophagia together with tongue and neck swelling, followed by multiple cranial nerve palsies

  •   Highlights from this issue
    Pract. Neurol. Pub Date : 2021-08-01
    Phil E M Smith, Geraint N Fuller

    Language provides us with names to categorise the world and becomes more sophisticated with use. Classification of everyday things comes naturally but we have a variable appreciation of our blind spots. If a neurologist were to try to distinguish between insects, most would probably apply a limited classification of flies (flies or bluebottles) not appreciating the diversity of species. However, when

  •   Neurology and clinical neurophysiology: an artificial divide
    Pract. Neurol. Pub Date : 2021-08-01
    Matthew C Kiernan

    The editors of Practical Neurology ask a reasonable question: who is best placed to oversee the neurophysiological investigation of patients presenting with neurological symptoms? Before considering the complexities of this seemingly straightforward question, it is accepted at the outset that any physician or technician could be trained to follow a testing proforma based on a symptom, or constellation

  •   Corticobasal syndrome: a practical guide
    Pract. Neurol. Pub Date : 2021-08-01
    Duncan Wilson, Campbell Le Heron, Tim Anderson

    Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. It presents insidiously and is slowly progressive. Clinicians should consider the diagnosis in people presenting with any combination of extrapyramidal features (with poor response to levodopa), apraxia or other parietal signs, aphasia and alien-limb

  •   Nodal and paranodal antibody-associated neuropathies
    Pract. Neurol. Pub Date : 2021-08-01
    Janev Fehmi, Tom Vale, Stephen Keddie, Simon Rinaldi

    Within the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo-paranodopathies’. Crucially, these patients often present with additional clinical features not usually seen with the most common immune-mediated neuropathies, Guillain-Barré syndrome and chronic

  •   Management of pineal and colloid cysts
    Pract. Neurol. Pub Date : 2021-08-01
    Michael D Jenkinson, Samantha Mills, Conor L Mallucci, Thomas Santarius

    The widespread use of MRI has led to the increasingly frequent diagnosis of pineal and colloid cysts. While most are small and incidental, do not require long-term monitoring and will never need treatment, they are a cause of patient anxiety and clinician uncertainty regarding the optimal management—particularly for larger cysts or those with an atypical appearance. Occasionally pineal cysts, and more

  •   Suspecting dementia: canaries, chameleons and zebras
    Pract. Neurol. Pub Date : 2021-08-01
    Jeremy C S Johnson, Laura McWhirter, Chris J D Hardy, Sebastian J Crutch, Charles R Marshall, Catherine J Mummery, Jonathan D Rohrer, Martin N Rossor, Jonathan M Schott, Rimona S Weil, Nick C Fox, Jason D Warren

    The early and accurate diagnosis of dementia is more important than ever before but remains challenging. Dementia is increasingly the business of neurologists and, with ageing populations worldwide, will become even more so in future. Here we outline a practical, symptom-led, bedside approach to suspecting dementia and its likely diagnosis, inspired by clinical experience and based on recognition of

  •   Optical coherence tomography: a window to the brain?
    Pract. Neurol. Pub Date : 2021-08-01
    Clare Fraser, Christian J Lueck

    First described in 1991 and introduced into clinical practice in 1996, optical coherence tomography (OCT) now has a very extensive role in many different areas of ophthalmological practice. It is non-invasive, cheap, highly reproducible, widely available and easy to perform. OCT also has a role in managing patients with neurological disorders, particularly idiopathic intracranial hypertension. This

  •   Whole-genome sequencing
    Pract. Neurol. Pub Date : 2021-08-01
    Huw R Morris, Henry Houlden, James Polke

    The costs of whole-genome sequencing have rapidly decreased, and it is being increasingly deployed in large-scale clinical research projects and introduced into routine clinical care. This will lead to rapid diagnoses for patients with genetic disease but also introduces uncertainty because of the diversity of human genomes and the potential difficulties in annotating new genetic variants for individual

  •   Visual hallucinations
    Pract. Neurol. Pub Date : 2021-08-01
    Rimona S Weil, A J Lees

    Visual hallucinations have intrigued neurologists and physicians for generations due to patients’ vivid and fascinating descriptions. They are most commonly associated with Parkinson’s disease and dementia with Lewy bodies, but also occur in people with visual loss, where they are known as Charles Bonnet syndrome. More rarely, they can develop in other neurological conditions, such as thalamic or midbrain

  •   Suspecting unwitnessed hypoglycaemia
    Pract. Neurol. Pub Date : 2021-08-01
    Danielle Lux, Mark Edwards, Liqun Zhang

    Hypoglycaemic coma can present with acute neurological dysfunction mimicking stroke. It may be masked by normoglycaemia due to physiological compensatory mechanisms (Somogyi phenomenon). Specific MR brain scan changes should alert the clinician to consider this alternative to stroke in the acute setting in patients with a history of recurrent hypoglycaemia, even when the blood glucose is not low at

  •   Don’t forget the fornix
    Pract. Neurol. Pub Date : 2021-08-01
    Maurice Sidra, Louise Hattingh, Hawraman Ramadan

    A 52-year-old man presented with acute onset of primarily anterograde amnesia. He had a history of diabetes mellitus, coronary artery disease and smoking. We found no other focal neurological deficits. The amnesia improved slowly over the subsequent weeks. MR scan of the brain showed moderate chronic microangiopathic white matter changes and acute infarction in both fornices (figure 1). This most likely

  •   Orbital myositis with herpes zoster ophthalmicus
    Pract. Neurol. Pub Date : 2021-08-01
    Tychicus Chen

    A 56-year-old man attended the emergency department with 2 days of right-sided frontal and supraorbital headache, with a dull throbbing and intermittent lancinating quality. He had photophobia but no phonophobia, nausea, vomiting or visual impairment. There was no diplopia but he did have discomfort on right lateral gaze. He was immunocompetent; his only relevant medical history was of familial hypercholesterolaemia

  •   Syphilis: keeping an eye out for the great mimic
    Pract. Neurol. Pub Date : 2021-08-01
    Sean Lance, Annemarei Ranta, Evan Jolliffe

    A 27-year-old woman presented to her optometrist with a 2-week history of improving vision, such that she no longer required corrective lenses. She also had chronic daily headache and pulsatile tinnitus that worsened when lying flat. Direct funduscopy identified bilateral severe optic disc oedema (Frisen grade 4–5) (figure 1). Uncorrected visual acuity was normal bilaterally (prescription 3 years before:

  •   After discharges following compound muscle action potential in CASPR2 antibody-related disease
    Pract. Neurol. Pub Date : 2021-08-01
    Dimitrios Parissis, Panagiotis Ioannidis

    A 75-year-old man had a 3-month history of shooting and burning pain in all four limbs, with painless muscle twitches, fatigue and moderate weight loss. During this time, he had suffered four episodes of syncope. He had longstanding stable epilepsy, controlled with carbamazepine and lamotrigine. On examination, there was frequent fasciculation and myokymia in the limb and trunk muscles. Muscle tone

  •   Amyloidoma mimicking multiple sclerosis
    Pract. Neurol. Pub Date : 2021-08-01
    Ide Smets, Silvia Marino, Gavin Giovannoni

    A 56-year-old woman developed optic neuritis in 2001 and progressive right-sided weakness dating from 2015. MR scan of the brain showed a solitary contrast-enhancing lesion in the left periventricular region and her cerebrospinal fluid (CSF) contained unmatched oligoclonal IgG bands. In 2017, she was diagnosed with multiple sclerosis (MS) and started disease-modifying treatment with glatiramer acetate

  •   Adult-onset subacute sclerosing panencephalitis
    Pract. Neurol. Pub Date : 2021-08-01
    Sruthi S Nair, K V Vysakha, Ramshekhar N Menon, Soumya Sundaram

    Subacute sclerosing panencephalitis (SSPE) is a lethal slow viral disease of the central nervous system caused by a defective measles virus. The onset is mostly in childhood, manifesting clinically as decline in academic performance, behavioural changes, motor dysfunction and myoclonus. Adult-onset SSPE is rare and can present as rapidly progressive dementia. We present a young man of Indian origin

  •   Cushing’s disease presenting with psychosis
    Pract. Neurol. Pub Date : 2021-08-01
    Ahmed Saeed Mubarak Mohamed, Ahmed Iqbal, Suveera Prasad, Nigel Hoggard, Daniel Blackburn

    Cushing’s disease is a rare endocrine condition in which a pituitary corticotroph adenoma drives excess adrenal cortisol production, and is one cause of endogenous Cushing’s syndrome. We present a young woman with 3 weeks of headaches and cognitive disturbance who subsequently developed florid psychosis requiring multiple admissions under neurology and psychiatry. Her clinical stigmata of hypercortisolism

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