A summary of recently published articles in the Neurology® Resident & Fellow Section
September 4, 2018, issue
This issue starts off with a pediatric Clinical Reasoning case that emphasizes that arthrogryposis (a child born with joint contractures) can be a presenting manifestation of an underlying neuromuscular disorder. The next Opinion and Special Article is a timely review of the state of neurology training in pain management. The Teaching NeuroImages cover multiple rare and more common eponymous brainstem syndromes.
Clinical Reasoning: A child with arthrogryposis: Congenital myasthenic syndrome: CHRNA1 mutation
A child presented at birth with arthrogryposis and respiratory and bulbar weakness and was subsequently diagnosed with congenital myasthenic syndrome. Clinicians should look for associated features that serve as diagnostic clues for myasthenic syndrome in patients with arthrogryposis.
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Opinion and Special Articles: Pain medicine: A case for neurologists
Neurologists are excellent diagnosticians and have abundant experience with medication management for chronic conditions. We provided practical advice to residency programs in hopes of generating pain management interest in their residents and incorporating more exposure to pain education during neurology residency.
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Teaching NeuroImages: Godtfredsen syndrome due to retroclival subdural hematoma
A 30-year-old woman presented with sudden onset headache of 2 days duration. Imaging showed a retroclival subdural hematoma extending from the posterior clinoid processes to the body of C2 vertebra, with compression of the brainstem.
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Teaching NeuroImages: Sleep-related periodic breathing in acquired central sleep apnea
A 68-year-old man complained of the subtle onset of postural instability. Imaging revealed a right upper medullary stroke that presented with Wallenberg syndrome and Ondine curse showing sleep-related Biot ataxic breathing.
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September 11, 2018, issue
Educational issues continue to be at the center of the mission of the Neurology® Resident & Fellow Section. The latest Education Research article explores methods of teaching about world health and related issues. The Clinical Reasoning case explores the presentation of an “adult” disease in a child. The Teaching Video NeuroImages shows the presentation of limb movement disorder and the Teaching NeuroImages discusses an atypical imaging presentation of a demyelinating disease.
Education Research: A framework for global health curricula for neurology trainees
As the global burden of neurologic disease is expanding, educating neurology trainees in the foundations of global health is becoming increasingly important. Educating neurology trainees and increasing awareness of the global epidemiology of neurologic diseases will affect how patients are diagnosed and managed worldwide. Thus, having standardized curricula that can be easily incorporated into existing didactic schedules is unique and essential.
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Clinical Reasoning: Young adult with dysphagia and severe weight loss
Juvenile amyotrophic lateral sclerosis, though rare, should be considered in the appropriate clinical context in a patient with dysphagia. A thorough neurologic examination may detect upper and lower motor neuron signs in clinically asymptomatic areas and will help in early diagnosis and appropriate management.
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Teaching Video NeuroImages: A treatable rare cause of chorea
A patient presented with hemichorea due to polycythemia vera but showed progressive choreic movements in the absence of abnormal hematocrit and hemoglobin levels at symptoms onset. The importance of this case resides on the fact that rare and treatable causes of chorea (or hemichorea) should be suspected, especially in elderly patients.
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Teaching NeuroImages: Cortical damage with leptomeningeal enhancement in neuromyelitis optica spectrum disorder
A 27-year-old woman presented with headache, weakness, and numbness in both lower extremities for 2 weeks. This patient's case is unusual, as neuromyelitis optica spectrum disorders rarely involve the cortex and pia mater.
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September 18, 2018, issue
Neurology Journal Club submissions are structured appraisals of recent Neurology research articles focused on the different types of methods used in clinical research. The Clinical Reasoning case explains the approach to a patient with transient speech issues. The Teaching NeuroImages reports an uncommon form of cysticercosis, while the Teaching Video NeuroImages demonstrates a particular dystonic posture.
Journal club: Florbetapir imaging in cerebral amyloid
This article critically appraises a recently accepted article in Neurology, focusing on amyloid-PET clinical relevance in cerebral amyloid angiopathy and intracerebral hemorrhage. The findings of this interesting and elegant study need to be considered in the context of potential limitations, some of which are inherent to amyloid-PET studies in the field, while others are more related to the selected methodology.
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Clinical Reasoning: Transient speech deficits in a patient with history of medulloblastoma
A 58-year-old left-handed man presented with transient speech difficulty, blurry vision, and right arm heaviness. The patient was initially diagnosed with a medulloblastoma after posterior headaches led to brain MRI showing a right cerebellar mass.
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Teaching NeuroImages: Spinal intramedullary cysticercosis: the pseudotumoral form
Cysticercosis is a parasitic disease caused by the larval stage of Taenia solium, which affects thousands of people worldwide. A 10-year-old boy was admitted to the hospital with decreased muscle strength in lower limbs. Physical examination revealed isolated spastic crural paraparesis.
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Teaching Video NeuroImages: The signs of dystonic tremor: Tremulous “escanciador”
A 58-year-old woman noticed right-hand jerky tremor exclusively while curling her hair. Her tremor-generating posture was reminiscent of the Asturian pourer of cider, or escanciador, who holds the bottle above the head when vertically pouring to aerate the liquid.
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September 25, 2018, issue
This issue begins with a Clinical Reasoning report of a rare neurologic case in the context of a systemic autoimmune disease. The Mystery Case is explained. Congratulations to all of our readers who selected the correct diagnosis. The second Clinical Reasoning case explores the issues dealt with by neurologists when performing prenatal consultations. Finally, the Teaching NeuroImages demonstrates findings in adult muscular dystrophy.
Clinical Reasoning: A 58-year-old woman with systemic scleroderma and progressive cervical cord compression
In this article, we introduce a rare neurologic case of systemic scleroderma affecting the cervical spinal cord through dystrophic calcinosis. The mass effect and the metabolic activity of the lesion were well demonstrated radiographically by means of MRT, CT angiogram, and PET/CT.
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Mystery Case: Spinal tuberculosis masquerading lung malignancy
A 32-year-old woman presented with backache of 4 months’ duration. She also complained of cough, anorexia, and some weight loss. She had a local tenderness at T6–T7 vertebrae.
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Clinical Reasoning: Ventriculomegaly detected on 20-week anatomic fetal ultrasound
A male fetus with severe hydrocephalus and multiple brain malformations was later found to have a pathogenic variant in the L1CAM gene on exome sequencing. We discuss a pathway of clinical reasoning from the perspective of both the fledgling field of fetal neurology and then into the realm of the neonatal neurologist managing a child with multiple congenital brain malformations and the differential diagnosis.
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Teaching NeuroImages: Myotonic dystrophy type 1
A 51-year-old woman presented with 2 years' history of progressive distal weakness and muscle atrophy. Clinical and electrophysiologic myotonia were found. Myotonic dystrophy type 1 (DM1) is the most common muscular dystrophy observed in adults. Though MRI in DM1, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, and mitochondrial myopathy may be similar, the clinical history for each condition is different.
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Footnotes
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