Neuro - Esper Flashcards
Clinical features and diagnostic tests for Creutzfeldt - Jakob disease
- CSF: 14-3-3 protein
- MRI: high signal intensity in the caudate nucleus and putamen bilaterally on T2
- EEG: characteristic bi- or triphasic spike wave complexes
- Patient presentation: rapidly progressing dementia and myoclonus
different peripheral nerve manifestations of diabetes
- Mononeuropathy (a single nerve) & Mononeuropathy Multiplex
- Symmetric Sensorimotor Neuropathy. The classic “glove and stocking” neuropathy
- Autonomic: GI and GU symptoms, impotence, and impaired sweating and vascular function
- Thoracoabdominal Radiculopathy
- Amyotrophy: Gradually progressive proximal leg weakness, Painful with NO sensory loss
Which nerves are lost first in diabetic neuropathy?
Small fibers (burning, tingling, numbness, pain) are lost first followed by large fibers (weakness, vibratory and position sense, numbness, areflexia)
Diagnostic tests for Polymyositis
Antinuclear antibody assay: positive in one third of patients with Polymyositis
MRI and ultrasonography: will show abnormalities due to inflammation of muscles
Biopsy: will show evidence of inflammation
Labs: serum creatinine and creatine kinase are commonly increased.
Signs and symptoms include: Symmetrical proximal muscle weakness, aches and cramping, morning stiffness, fatigue, anorexia, fever, weight loss, arrhythmias, dyspnea, dysphagia, aspiration, constipation, and reflux.
A manifestation that is due to the presence of a neoplasm or cancer, however, it is not caused by the local presence of the cancer. When the body mounts an immune response against a tumor, sometimes the antibodies that are formed recognize antigens that are similar to those present elsewhere in the body on healthy tissues. Because of this, these antibodies target the healthy tissues and cause damage to them.
Paraneoplastic syndromes
Lambert-Eaton Syndrome
most commonly due to small cell carcinoma of the lung, where antibodies are formed against presynaptic calcium channels of the neuromuscular junction
Proximal muscle weakness that improves with use. Dry mouth, pupils may not be reactive. No improvement of symptoms with use of anticholinesterase use. Often resolves with the resection of the tumor (commonly a thymoma)
Lambert-Eaton Syndrome clinical findings
Elevated intracranial pressure, headache, papilledema, and CN VI palsy. Spinal tap pressure (>250 mm H2O), MRI
Pseudotumor cerebri
Treatment: Self-limiting, diuretics, surgery (shunting)
- Straw colored/non-purulent CSF
- Increased lymphocytes
- Mildly elevated protein concentration
- Normal glucose concentration
Viral Infection
What signs do patients have to present with for Wernicke Encephalitis
Caine criteria for diagnosis in which patients must present with two of the following four signs: Thiamine dietary deficiency, oculomotor abnormalities, cerebellar dysfunction, either altered mental status or mild memory impairment. The Caine criteria are considered more sensitive for making a diagnosis.
Describe the diagnostic tests and signs and symptoms of Lyme disease
Localized: Bull’s eye rash (painless) lasting 1-30 days
Early Disseminated: meningitis, carditis, radiculoneuropathy, cranial neuropathy lasting weeks to months.
Late Disseminated: persistent arthritis and encephalopathy
- Explain common symptoms and signs of Myasthenia Gravis
Fluctuating weakness affecting facial and pharyngeal muscles, respiratory failure, aspiration, and muscle wasting in 10% of patients. Symptoms are better in the morning and get worse as the day progresses. The disease is usually associated with a thymoma. There are ocular and generalized forms (ocular symptoms in 40% initially and eventually appear in 85%).
Flu shots aggravate MG.
Treatment of Myasthenia Gravis
Pyridostigmine is used to treat MG.
Clinical presentation and treatment of cluster headaches
Cluster headaches are generally found more in men over 19 yo. There will be multiple attacks per day/week lasting 15mins - 3 hrs. Intensity is severe and unilateral in the supraorbital/temporal region. There is no aura but patients can have lacrimation and rhinorrhea. This often occurs at night and can lead to suicide.
Treament of cluster headaches
if symptomatic, Oxygen Dihydroergotamine Sumatriptan Sphenopalatine block Intranasal lidocaine Intranasal capsaicin Indomethacin Opioids
for prophylaxis. Verapamil Steroids Lithium Methysergide Valproic acid Neuroleptics Clonidine Daily triptans or ergots Daily opioids
Adrenoleukodystrophy
X-linked inherited disorder causing atrophy of the adrenal cortex and demyelination of the nervous system. This increases levels of very long chain fatty acids. Affects kids 4-8 years old.
Metachromatic leukodystrophy
Autosomal recessive caused by a defect in Arylsufatase A resulting in the accumulation of sulfatides causing myelin breakdown. Macrophages containing sulfatides bind to certain types of dyes, which change absorbance spectrum.
Alexander disease
Rare neurological disease in infants and kids which involves loss of myelin in the brain and accumulation of irregular extracellular fibers (AKA Rosenthal fibers)
Krabbe disease
Deficiency of Galactocerebroside Beta Galactosidase. Autosomal recessive
Demyelinating disease most commonly show white matter changes on MRI:
- CADASIL: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. Mutation of Notch 3 gene on Chromosome 19. It’s associated with migraines.
- Multiple Sclerosis
- Marchiafava-Bignami: affects corpus callosum and anterior commissure. Most common patients are men with alcoholism
- Wernicke-Korsakoff
- Mercury Poisoning
- Subacute Combined Degeneration
- Progressive Multifocal Dystrophy (from JC virus)
- Neuromyelitis Optica: variant of MS also called Devic disease. Worse than MS and happens more in the Asian population
- Subacute Sclerosing Panencephalitis
- Acute Disseminated Encephalomyelitis: follows viral infection or viral immunization. Headache, lethargy, and coma occur and are more common than focal findings
- Central Pontine Myelinolysis: due to rapid correction of hyponatremia. This causes rapidly developing quadriplegia. Associated with alcoholics.
etiology and diagnosis of an acute ischemic stroke versus a hemorrhagic stroke
On CT a hemorrhagic stroke would show immediately. However, CT scanning is insensitive for small or early ischemic infarcts. Diffuse Weighted Imaging MRI can more accurately identify areas of ischemia within minutes of onset.
clinical and laboratory findings in B12 deficiency
B12 deficiency will cause pernicious anemia and sometimes Subacute Combined Degeneration. Signs and symptoms include:
• Myelin loss in the posterior columns causing sensory ataxia, paresthesias in the feet, impaired vibration and position sense, spasticity, hyperreflexia, and Babinski sign.
• Increased levels of Homocysteine and Methylmalonic acid levels
clinical features of and diagnostic tests used in HSV Encephalitis
HSV encephalitis is caused by HSV-1 that enters the brain via the trigeminal ganglion or olfactory bulbs hence predilection for the temporal and frontal lobes.
Signs and symptoms include: fever, headache, focal seizures, focal signs (aphasia, personality changes, agitation, hallucinations), meningeal signs, and déjà vu phenomenon.
Diagnosis: CSF (increased white cells, red cells and protein; decreased or normal glucose), CSF PCR for HSV, EEG, imaging, and brain biopsy.
Differentiate Guillain-Barré and chronic inflammatory demyelinating polyradiculoneuropathy
CIDP produces manifestations similar to GBS but symptoms tend to come on more slowly and progress for a longer period of time (namely, CIPD lasts longer than 8 weeks).
clinical findings, diagnostic tests and treatment for multiple sclerosis
- Motor: weakness, spasticity, UMN (hyperreflexia, Babinski), fatigue
- Sensory: Vibratory, pain, temperature, and crude touch loss
- Lhermitte Sign: electrical sensation down the back after flexion of the neck
- Optic neuritis: painful loss of vision
- CN problems: facial pain, numbness for any of the CN
- Internuclear Ophthalmoplegia
- Cerebellar ataxia (gait, tremor)
- Autonomic Dysfunction: Bowel (constipation), Bladder problems, Sexual Dysfunction
- Psychiatric: depression, bipolar disorder, cognitive disorder, memory trouble
- Symptoms may be worsened or precipitated by Uhtoff’s phenomenon (exposure to heat)
Diagnosis:
• MRI of brain & spinal cord: look for abnormal lesions of the white matter (look for 2 or more lesions at 1 period of time)
• CSF evaluation looking for Oligoclonal Bodies
• Evoked potentials: visual (optic nerve), brainstem auditory (pontine lesions), and somatosensory (spinal cord)
• History & Physical
Treatment:
• Solumedrol: steroid that is used to treat relapses.
• Usually treated with disease modifying therapies and symptomatic therapies:
• Interferon Drugs, Glatiramer acetate, Natalizumab, Fingolimod, Mitoxantrone, Teriflunomide, and Dimethyl fumarate
- Describe the clinical presentations, diagnosis and treatment of Alzheimer Disease
Clinical findings: most common dementia over age 65. Progressive, degenerative brain disease characterized by memory impairment/dementia early on, and changes in behavior, personality, judgment and ADLs. Risk factors include Down syndrome, family history, and advanced age. Geographic or temporal disorientation, day-night disorientation, language deterioration, wandering, irritability, depression, hallucinations, delusions, agitation, incontinence, and total dependence on caregivers are also common.
Diagnosis: clinical criteria, mental status exam, and physical and neurological exams. Upon death, histopathological study shows extracellular deposition of Amyloid Beta Protein, intracellular Neurofibrillary Tangles, and loss of neurons, especially in the hippocampus.
Treatment:
Cholinesterase Inhibitors: Donepezil, Rivastigmine, Galantamine, and Tacrine.
NMDA receptor antagonist: Memantine
Patient education: drugs won’t cure; only reduce symptoms, give patients simple instructions, react calmly, and avoid confrontation.
CSF: 14-3-3 protein
Creutzfeldt - Jakob disease
EEG: characteristic bi- or triphasic spike wave complexes
Creutzfeldt - Jakob disease
Patient presentation: rapidly progressing dementia and myoclonus
Creutzfeldt - Jakob disease
Patient presents with Symmetrical proximal muscle weakness, aches and cramping, morning stiffness, fatigue, anorexia, fever, weight loss, arrhythmias, dyspnea, dysphagia, aspiration, constipation, and reflux. Labs: serum creatinine and creatine kinase are commonly increased.
Polymyositis
Treatment for pseudotumor cerebri
Treatment: Self-limiting, diuretics, surgery (shunting)
- Cloudy/purulent CSF
- Increased neutrophils/PMNs
- Increased protein concentration
- Decreased glucose concentration
Bacterial infection
Demyelination due to B12 deficiency commonly affects the posterior columns. Signs include ataxia, muscle weakness, and incontinence.
Subacute combined demyelination Syndrome
Degeneration of peripheral nerve axons due to malnourishment and/or excessive ethanol levels. It’s usually symmetric and predominantly distal. Signs include paresthesia, muscle weakness, and ataxia.
Alcoholic polyneuropathy
Demyelination of corpus callosum and adjacent structures due to chronic alcohol use. Signs include cognitive impairment (dementia), ataxia, apraxia, urinary incontinence, and seizures (possibly).
Marchiafava-Bignami
Due to thiamine deficiency. Wernicke: encephalopathy, oculomotor dysfunction, gait ataxia. Korsakoff: late sequelae of Wernicke with anterograde and retrograde amnesia with confabulation
Wernicke-Korsakoff syndrome
Encephalopathy, oculomotor dysfunction, gait ataxia
Wernicke Encephalitis
Treatment for Wernicke Encephalitis
Treatment: thiamine (before glucose), stop drinking alcohol, improve diet
Differentiate between folate and B12 deficiency using homocysteine and Methylmalonic acid
Vitamin B12 deficiency causes both hyperhomocysteinemia and increase Methylmalonic acid.
A folate deficiency will just be homocysteine
CSF increased white cells, red cells and protein; decreased or normal glucose,
HSV Encephalitis
How quickly is the onset of Guillaine-Barre?
Very rapid
Motor weakness, spasticity, UMN signs(hyperreflexia, Babinski), fatigue. Vibratory, pain, temperature, and crude touch loss. Painful loss of vision. Internuclear Ophthalmoplegia. Bowel (constipation), Bladder problems, Sexual Dysfunction .
Multiple sclerosis
MRI shows lesions of brain & spinal cord along the ventricles. CSF evaluation looking for Oligoclonal Bodies
Multiple sclerosis
Treatment for Multiple sclerosis
“So interferon, Glad Natalie forgot Mitch’s Terimusu, he’s Dad’s Fumming”
Solumedrol: steroid that is used to treat relapses.
• Usually treated with disease modifying therapies and symptomatic therapies:
• Interferon Drugs, Glatiramer acetate, Natalizumab, Fingolimod, Mitoxantrone, Teriflunomide, and Dimethyl fumarate
Patient presents with painful side around the left side of his umbilicus. He notices it seem to wrap around towards the back side and is in just one area. On physical examination you notice there are no vesicles of eruptions. Patient mentions recent weight loss and is diabetic. What is the Dx?
Diabetic Thoracoabdominal Radiculopathy. Damage to the root
Features of Gullaine Barre
Ascending paralysis Areflexia Loss of propioception No affect on EOM Pain
Features of Chronic Inflammatory Demyelinating polyneuropathy
Duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
Weakness
Areflexia
Pain
numbness, tingling
When do you treat prophylactically for headaches
Twice weekly or less - acute
Daily or every other day - prophylaxis
