Neurology Flashcards
You are caring for a 4 year male who presents with dysmetria, horizontal nystagmus, and ataxia. Her speech is slurred, but her mentation seems normal. Her strength is normal in her upper and lower extremities, and her patellar and achilles reflexes are 2+. If a brain MRI was obtained, what would it likely show?
A. Multiple T2 intense lesions in the white matter and deep grey matter with ill defined margins.
B. Relatively uniform T2 hyperintensity and T1 post-gadolinium enhancement throughout the cerebellar hemispheres.
C. Areas of diffusion restriction on diffusion weighted imaging.
D. Multiple lesions on T1, some gadolinium enhancing and some not.
B. Relatively uniform T2 hyperintensity and T1 post-gadolinium enhancement throughout the cerebellar hemispheres.
The patient has signs and symptoms consistent with acute cerebellar ataxia. While not always nessary, MRI imaging shows diffuse changes within the cerebellar hemispheres.
Option A would be seen with ADEM. Option C could be seen with ischemia or infarction. Option D would be characteristic of multiple sclerosis.
You are caring for a 4 year male who presents with dysmetria, horizontal nystagmus, and ataxia. Her speech is slurred, but her mentation seems normal. Her strength is normal in her upper and lower extremities, and her patellar and achilles reflexes are 2+. What is the treatment for her likely diagnosis?
The patient has signs and symptoms consistent with acute cerebellar ataxia. Treatment is supportive care.
What MRI imaging modality is represented by this image:
T2 FLAIR. The gray matter is bright relative to the white matter, indicating that this is a T2 weighted image. However, the CSF is black indicating is a fluid attenuated (FLAIR) image, ideal for identifying demyelinating lesions adjacent to the ventricles.
Image credit: Clarke L, et al.. MRI Patterns Distinguish AQP4 Antibody Positive Neuromyelitis Optica Spectrum Disorder From Multiple Sclerosis. Front Neurol. 2021 Sep 9;12:722237. Used under Creative Common Attribution License via Pubmed Central.
You are caring for a 10 year old female who presents with bilateral lower extremity weakness. On exam she has full passive range of motion of of the knee and hip, which are free of erythema or swelling. Her strength is 2+ to extension and flexion at the hip and 1+ to extension and flexion at the knee. Patellar and achilles reflexes are absent. Her mentation is normal and she is afebrile and otherwise well appearing. What life threatening complication is she at risk for?
The patient’s presentation is consistent with the diagnosis of acute inflammatory demyelinating polyneuropathy. She is at risk for ascending paralysis resulting in loss of respiratory muscle use.
You are caring for a 10 year old female who presents with bilateral lower extremity weakness. On exam she has full passive range of motion of of the knee and hip, which are free of erythema or swelling. Her strength is 2+ to extension and flexion at the hip and 1+ to extension and flexion at the knee. Patellar and achilles reflexes are absent. Her mentation is normal and she is afebrile and otherwise well appearing. What is the first line treatment for this patient?
The patient’s presentation is consistent with the diagnosis of acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome). First line therapy is IVIG.
What are the elements of the Kocher criteria for determining risk of septic arthritis?
- Non weight bearing
- Temperature > 38.5°C
- ESR > 40
- WBC > 12,000
You are caring for a 10 year old female who presents with bilateral lower extremity weakness and right arm weakness. Her symptoms developed over the last 18 hours. On exam she has a temperature of 38.9You are caring for a 10 year old female who presents with bilateral lower extremity weakness. On exam she has full passive range of motion of of the knee and hip, which are free of erythema or swelling. Her strength is 2+ to extension and flexion at the hip and 1+ to extension and flexion at the knee. Patellar and achilles reflexes are absent. Her mentation is normal and she is afebrile and otherwise well appearing. What is the first line treatment for this patient? full passive range of motion of of the knee and hip, which are free of erythema or swelling. Her strength is 3+ to extension and flexion at the right hip and 2+ to extension and flexion at left hip. Strength to extension and flexion at the knees is 2+. Hand grip strength is 3+ on the right, 5+ on the right. Although she will respond to commands sufficiently to complete your exam, she is quite sleepy and somewhat irritable. She is febrile. Patellar and achilles reflexes are absent. Her mentation is normal and she is afebrile and otherwise well appearing. What is the first line treatment for this patient?
The case presentation is consistent with ADEM.
The first line treatment is steroids.
What are the 4 CNS regions defined by the Mcdonald criteria for the purpose of identifying symptoms that are “distant in space” when considering the diagnosis of multiple sclerosis?
- Spinal cord
- Infratentorial
- Cortical / justacortical
- Periventricular
You are caring for a 10 year old patient who was diagnosed with ADEM 6 months ago. Today she presents with weakness of her left arm that has developed over the past day. Her vital signs are normal. Grip strength and flexion at the left elbow are diminished, however her brachioradialis and biceps reflexes are brisk in that arm relative to her right arm. She has no cranial nerve deficits, and she has normal mental status. Her exam is otherwise reassuring. Diffusion weighted imaging shows no areas of restricted diffusion. Her MRI imaging reveals a ride sided area of gadolidium enhancement on T1 weighted images. This same area is hyperintense on T2 weighted images. She has a similar lesion in her cervical spinal cord, although this one does not enhance following injection of gadolidium. What is her most likely diagnosis?
A. Recurrent ADEM
B. Cerebral infarction
C. Lyme encephalitis
D. Multiple Sclerosis
D. Multiple sclerosis. With her initial presentation of ADEM with a second attack more than 3 months later associated with MRI findings in multiple CNS regions. (In addition, the non-enhancing lesion in her spinal cord suggests an older lesion that may have occurred some time ago).
You are caring for a 10 year old patient who was diagnosed with ADEM 6 months ago. Today she presents with weakness of her left arm that has developed over the past day. Her vital signs are normal. Grip strength and flexion at the left elbow are diminished, however her brachioradialis and biceps reflexes are brisk in that arm relative to her right arm. She has no cranial nerve deficits, and she has normal mental status. Her exam is otherwise reassuring. Diffusion weighted imaging shows no areas of restricted diffusion. Her MRI imaging reveals a right sided area of gadolidium enhancement on T1 weighted images. This same area is hyperintense on T2 weighted images. She has a similar lesion in her cervical spinal cord, although this one does not enhance following injection of gadolidium. What is the first line therapy for this patient?
Steroids are indicated for the acute attack.
For disease modification, a discussion should be had with the patient and family about rituximab (more effective) vs. older therapies such as glatiramer acetate or interferon beta-1a (fewer side effects).
You are caring for a 12 year old patient who presents with diminished visual acuity in her left eye developing over 2 days. She now feels that her vision in her right eye is also becoming worse. T1 post gadolinium weighted images of the optic nerves are shown. Here aquaporin-4 IgG is positive. What is the diagnosis and first line treatment?
The presence of bilateral demyelination (as evidenced by her symptoms and bilateral optic nerve enhancement on MRI imaging) combined with positive aquaporin-4 antibody are diagnostic for neuromyelitis optica spectrum disorders. Since she is aquaporin-4 antibody positive, no further clinical criteria are required. However, accompanying transvere myelitis and/or area postrema syndrome (intractable hiccups and/or vomiting) would further support the diagnosis.
First line treatment for attacks is steroids. Immunomodulator therapy may be employed for disease modification, but specific drug choices vary widely between providers and institutions.
Image credit: Sechi E, et al. Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management. Front Neurol. 2022 Jun 17;13:885218. Used under Creative Common Attribution License via Pubmed Central.
What are the relative intensities of gray matter and white matter on T1 weighted MRI imaging of the CNS?
Grey matter is darker, white matter is lighter.
What are the relative intensities of gray matter and white matter on T2 weighted MRI imaging of the CNS?
Grey matter is lighter, white matter is darker.
What is the most common age at which acute cerebellar ataxia is diagnosed?
1-3 years of age.
What is the most common age at which ADEM is diagnosed?
3-7 years of age.
