Snapshot A 32-year-old man presents to the emergency department with worsening lower extremity weakness. The patient was in his usual state of health until approximately one week prior to presentation, where he developed increased difficulty climbing the stairs. Now he is unable to stand. A few weeks ago he developed bloody diarrhea, which he attributes to drinking unpasteurized milk. On physcal examination, he has bilateral lower extremity 1/5 strength with absent patellar reflexes. (Guillain-Barre syndrome) Guillain-Barré Syndrome Introduction acute inflammatory demyelinating polyradiculopathy (AIDP) most common presentation decreased axonal conduction speed inflammatory demyelination of peripheral nerves autoimmune attack of peripheral Schwann cells due to molecular mimicry previous Campylobacter jejuni and Mycoplasma pneumoniae may have an association with Zika virus infection Presentation symptoms symmetric weakness usually begins in distal limbs and moves proximally can begin proximally or a combination of proximally and distally physical exam decreased or absent deep tendon reflexes cranial nerve involvement common resulting in facial paralysis autonomic dysfunction resulting in cardiac irregularities and fluctuations in blood pressure Evaluation LP albuminocytologic dissociation ↑ CSF protein with normal cell count Treatment almost all patients survive and achieve complete recovery in several weeks respiratory support is critical until recovery pulmonary function testing plasmapheresis or IV immunoglobulin equal efficacy; no additive benefit Prognosis, prevention, and complications prognosis low mortality complications respiratory failure Progressive Multifocal Leukoencephalopathy (PML) Introduction widespread CNS demyelination due to destruction of oligodendrocytes associations latent JC virus reactivation ↑ risk with natalizumab ↑ risk with rituximab seen in the immunosuppressed HIV infection < 200 per mm3 organ transplant patients Presentation subacute neurological deficits e.g., hemiparesis, monoparesis, ataxia, altered mental status Evaluation clinical diagnosis with MRI imaging MRI symmetric or asymmetric lesions in multifocal areas PCR of CSF for JC virus brain biopsy Treatment patients with HIV antiretroviral therapy natalizumab-associated stop agent initiate plasma exchange Prognosis, prevention, and complications prognosis HAART therapy improves survival in patients with HIV prevention improving CD4+ count in patients with HIV complications death Acute Disseminated (Postinfectious) Encephalomyelitis Introduction autoimmune inflammatory demyelination of the CNS secondary to infection or vaccination via infection e.g., chickenpox and measles via vaccinations e.g., rabies and smallpox Presentation symptoms acute and rapidly progressive development multifocal neurological deficits altered mental status motor deficits cranial nerve neuropathy ataxia sensory deficit Evaluation clinical diagnosis Treatment high-dose corticosteroids IVIG or plasma exchange if poor response acyclovir Prognosis, prevention, and complications prevention no small pox vaccine before 12 months of age complications neurologic impairment Metachromic Leukodystrophy Introduction autosomal recessive lysosomal storage disease due to deficient arylsulfatase A inability to degrade sulfatides → sulfatide build up → myelin breakdown Presentation symptoms diverse neurological impairment depending on subtype e.g., motor skill regression in late infantile subtype Evaluation arylsulfatase enzyme activity in leukocytes decreased biopsy neural tissue metachromatic lipid deposits Treatment no curative treatment Charcot-Marie-Tooth Disease Introduction also known as hereditary motor and sensory neuropathy (HMSN) group of disorders negatively affecting peripheral nerves and/or myelin genetically distinct disorders Presentation symptoms peroneal nerve neuropathy lower leg atrophy "inverted bottle" appearance diminished or absent reflexes decrease vibration and proprioception sense pes cavus Treatment supportive Central Pontine Myelinolysis (CPM) Introduction pontine demylination loss of corticospinal and corticobulbar tracts due to rapid correction of hyponatremia can result in "locked-in syndrome" Presentation symptoms pseudobulbar palsy with spastic quadriparesis locked-in like presentation can move eyes and blink may also include cognitive function changes Prognosis often fatal
QUESTIONS 1 of 5 1 2 3 4 5 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.NE.17.4768) A 4-year-old previously healthy boy presents with 4 days of intermittent vomiting and 5-6 daily loose stools. His mother noted bloody stools and decreased oral intake of food and water over the last 24 hours. He is normally in daycare; however, he has been home for the past 3 days. On physical exam his temperature is 102.2°F (39°C), blood pressure is 140/90 mmHg, pulse is 120/min, respirations are 22/min and O2 saturation is 99% on room air. He has dry mucous membranes. On abdominal exam you note diffuse tenderness to palpation without rebound or guarding. There are no masses, hepatosplenomegaly, and bowel sounds are hyperactive. Ultrasound of the right lower quadrant is negative for appendicitis. Stool is guaiac positive. He receives 15mg/kg acetaminophen and fluids are started. The next day, he complains of lower extremity weakness and tingling. On repeat exam, lower extremity strength is 3/5 with diminished patellar deep tendon reflexes. Which of the following lab findings would most likely be seen in this patient? QID: 109064 Type & Select Correct Answer 1 Gram stain positive CSF 24% (93/380) 2 Peripheral eosinophilia 11% (42/380) 3 Xanthochromia on cerebrospinal fluid analysis 8% (32/380) 4 Increased cerebrospinal fluid protein with normal cell count 36% (137/380) 5 Oligoclonal bands on cerebrospinal fluid analysis 12% (44/380) M 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (M1.NE.14.77) A 26-year-old man presents to his primary doctor with one week of increasing weakness. He reports that he first noticed difficulty walking while attending his sister's graduation last week, and yesterday he had difficulty taking his coffee cup out of the microwave. He remembers having nausea and vomiting a few weeks prior, but other than that has no significant medical history. On exam, he has decreased reflexes in his bilateral upper and lower extremities, with intact sensation. If a lumbar puncture is performed, which of the following results are most likely? QID: 106891 Type & Select Correct Answer 1 High neutrophils, high protein, low glucose, high opening pressure 12% (30/245) 2 High lymphocytes, normal protein, normal glucose, normal opening pressure 17% (41/245) 3 High lymphocytes, high protein, low glucose, high opening pressure 16% (40/245) 4 Normal cell count, high protein, normal glucose, normal opening pressure 35% (86/245) 5 Normal cell count, normal protein, normal glucose, normal opening pressure 7% (17/245) M 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
All Videos (2) Login to View Community Videos Login to View Community Videos Symptoms of Charcot Marie Disease Keshav Mudgal Neurology - Diseases of Myelination E 10/23/2015 83 views 0.0 (0) Login to View Community Videos Login to View Community Videos Metachromic Leukodystophy Keshav Mudgal Neurology - Diseases of Myelination D 10/23/2015 28 views 5.0 (1) Neurology | Diseases of Myelination Neurology - Diseases of Myelination Listen Now 15:16 min 10/22/2021 65 plays 5.0 (1)