A summary of recently published articles in the Neurology® Resident & Fellow Section
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February 2, 2021, Issue
This issue opens with a Training in Neurology report, which explores the role of psychiatry training in neurology residency. The Clinical Reasoning case details a 61-year-old man with signs of confusion, memory loss, and reduced speech, which progressed rapidly due to bithalamic abnormalities and a dural arteriovenous fistula. A Teaching NeuroImage reveals the case of a 35-year-old woman with a family history of adult-onset Alexander disease presenting with general stiffness and gait disturbance. The Teaching Video NeuroImage displays the co-occurrence of seesaw nystagmus with multifocal myoclonus following hypoxic encephalopathy, also referenced as Lance Adams syndrome.
Training in Neurology: Feedback From Graduates About the Psychiatry Component of Residency Training
This Training in Neurology article examines the role of psychiatry training in neurology residency. Four themes were identified for improving psychiatry training, including providing more teaching time in psychiatry, outpatient experiences, instruction in psychiatric treatments, and exposure to patients with conditions that overlap the 2 specialties.
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Clinical Reasoning: Rapidly Progressive Thalamic Dementia
A 61-year-old man exhibited signs of confusion, memory loss, and decreased speech. When managing patients with bilateral thalamic hyperintensities, this report encourages clinicians to conduct a timely cerebral angiogram to explore vascular etiologies.
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Teaching NeuroImages: Neuroimaging in Adult-Onset Alexander Disease
This case displays the imaging indicators of adult-onset Alexander disease in a 35-year-old woman with a family history of this diagnosis.
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Teaching Video NeuroImages: A Case of Lance Adams Syndrome With Seesaw Nystagmus
Seesaw nystagmus is noted in this case, in correlation with myoclonus following hypoxic encephalopathy.
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February 9, 2021, Issue
This issue highlights a Pearls & Oy-sters case that explores the rare occurrence of divergence nystagmus in correlation with lesions involving the dorsal pons and midline cerebellum in 2 patients. An Opinion & Special Articles article presents a novel occurrence of cerebellar degeneration in a young patient, stemming from CNS involvement in immunodysregulation, polyendocrinopathy, and enteropathy, X-linked (IPEX) syndrome. The Teaching NeuroImage depicts the co-occurrence of an adjacent cavernous malformation with a left-sided hemorrhage and a developmental venous anomaly emphasizing the evaluation of multiple vascular anomalies in patients with intracranial hemorrhage. In a Video Teaching NeuroImage, thrombosis and slow blood flow are demonstrated prior to basilar aneurysm rupture and depict the role of high-resolution and 4D-flow MRI in evaluating hemorrhage risk in intracranial aneurysms.
Pearls & Oy-sters: Divergence Nystagmus
Two adult women, ages 18 and 27 years, presented with divergence nystagmus during visual fixation with lesions involving the cerebellum and pons. In both patients, ataxia was also noted in the left or right extremities. This case emphasizes that lesions affecting the dorsal pons and midline cerebellum may be linked to divergence nystagmus on examination.
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Opinion and Special Articles: Cerebellar Ataxia and Liver Failure Complicating IPEX Syndrome
This Special Article explores the case of a 19-year-old man who developed progressive cerebellar ataxia from an early age, alongside insulin-dependent diabetes mellitus and liver failure. The resulting diagnosis of IPEX syndrome suggests that evaluating for immune, endocrine, and gastrointestinal dysfunction should be performed in patients with unexplained cerebellar degeneration.
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Teaching NeuroImages: The Venous System and Developmental Venous Anomalies: Drivers of Vascular Malformations?
A rare co-occurrence of developmental venous anomaly, arteriovenous malformation, and a cavernous malformation is featured in this case of a 57-year-old woman exhibiting acute confusion.
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Teaching Video NeuroImages: Wall Enhancement With Slow Blood Flow and Thrombosis Prior to Basilar Aneurysm Rupture
The case of a 31-year-old man indicates that slow blood flow and thrombosis may lead to vessel wall inflammation as evidenced by wall enhancement on imaging and contribute to basilar aneurysm rupture.
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February 16, 2021, Issue
This issue begins with a Clinical Reasoning case that explores an amyloid myopathy diagnosis for a 63-year-old woman, who reported symptoms of bilateral leg pain and muscle weakness. The Pearls & Oy-sters report describes an instance of systemic amyloidosis in a 61-year-old woman within 5 years of domino hAATR liver transplant. A Teaching NeuroImage shows the motor band sign on brain MRI, which depicts siderosis along the central sulcus in a patient with amyotrophic lateral sclerosis (figure). The Teaching Video NeuroImage illustrates a case of ictal unilateral eye blinking associated with frontotemporal seizure in a 6-year-old child.
Clinical Reasoning: A 63-Year-Old Woman Presenting With Bilateral Leg Pain
A 63-year-old woman presented with sudden bilateral leg pain and weakness, with no additional tingling or numbness. A timely muscle biopsy and the use of special stains such as Congo red are encouraged for patients with these symptoms.
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Pearls & Oy-sters: Number, Weaker, and Dizzier Due to Transthyretin Amyloidosis After 2 Liver Transplants
Domino liver transplant involves transplantation of a liver from an hATTR-positive donor for a person with liver failure. In this case, a 61-year-old woman reported numbness and weakness in the extremities 5 years after a liver transplant. A second liver transplant slowed but did not eliminate progressive symptoms of systemic amyloidosis.
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Teaching NeuroImages: The Motor Band Sign in Amyotrophic Lateral Sclerosis
The motor band sign is seen on brain MRI in this 71-year-old woman with amyotrophic lateral sclerosis, suggesting intracellular iron accumulation in the motor strip.
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Teaching Video NeuroImages: Ictal Unilateral Eye Blinking in Temporal Lobe Seizures: An Illustrative Video Case
A 6-year-old child exhibits unilateral eye blinking associated with right temporal lobe epilepsy.
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February 23, 2021, Issue
This issue starts with a Clinical Reasoning case, which details a 48-year-old man with binocular diplopia deriving from thalamo-mesencephalic infarct. The Pearls & Oy-sters case describes a 13-year-old child with a minor head injury, which uncovered a diagnosis of Sturge-Weber syndrome. Two Teaching Video NeuroImages are featured: the first explores a rare third ventriculostomy in a 6-year-old boy with chronic hydrocephalus. The second highlights the use of Bereitschaftspotential, or premotor capacity, to assist in the diagnosis of functional movement disorders.
Clinical Reasoning: A 48-Year-Old Man Presenting With Diplopia
A 48-year-old man displaying binocular diplopia is presented in this case, which investigates the location of a lesion at the thalamo-mesencephalic junction.
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Pearls & Oy-sters: Sturge-Weber Syndrome Unmasked by Traumatic Brain Injury
A minor head injury in a 13-year-old child provokes symptoms of a rare diagnosis of type III Sturge-Weber syndrome.
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Teaching Video NeuroImages: Spontaneous Third Ventriculostomy
Spontaneous third ventriculostomy can occur in patients with chronic hydrocephalus. The 4-year-old boy in this case presented 2 years after evaluation for macrocephaly with imaging evidence of spontaneous third ventriculostomy, as displayed in this video.
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Teaching Video NeuroImages: Bereitschaftspotential: A Neurophysiologic Test for Functional or Voluntary Jerks
This case describes the function of Bereitschaftspotential in evaluating and differentiating between movement disorders.
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