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117 Cards in this Set
- Front
- Back
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Demyelinating (Myelinoclastic) Disorders
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1. MS
2. Perivenous Encephalomyelitis 3. Progressive Multifocal Leukoencephalopathy |
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Myelinoclastic lesions that do not involve PNS
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MS
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Lesions tend to be periventricular
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MS lesions
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Myelinoclastic lesions confined to spinal cord, optic chiasm and optic nerves
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Neuromyelitis Optica (Devic's Disease)
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Early MS plaques
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Poorly demarcated & soft
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Late MS plaques
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Well demarcated & firm
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MS plaques show demyelination without axonal damage
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Early plaques
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MS plaques with axonal damage and gliosis
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Late plaques
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MS demographics
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Females 3:1
Age of onset 20-40 years Survival 20 years High concordance among monozygotic twins |
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MS findings in CSF
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MBP
IgG oligoclonal bands (not in serum) |
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Infection precedes myelinoclastic attack, but no infection at time of demyelination.
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Perivenous encephalomyelitis (PEM)
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2 types of Perivenous encephalomyelitis
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1. Acute disseminating encephalomyelitis (ADEM)
2. Acute hemorrhagic leukoencephalomyelitis (AHLE) |
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Demyelinating disease caused by slow viral infection
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Progressive Multifocal Leukoencephalopathy (PML)
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Oligodendroglial nuclei with viral inclusions
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Progressive Multifocal Leukoencephalopathy (PML)
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Progressive Multifocal Leukoencephalopathy (PML) viral causative agents
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Polyomaviridae, specifically JC
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3 types of Dysmyelinating Disorders
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1. Metachromatic
Leukodystrophy (MLD) 2. Globoid Leukodystrophy (GLD) 3. Adrenal Leukodystrophy (ALD) |
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Basic difference between Leukoencephalopathy and Leukodystrophy
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Leukoencephalopathy affects CNS only, whereas Leukodystrophy affects CNS and PNS
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Metachromatic macrophages
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Metachromatic leukodystorphy (MLD)
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Lysosome disorder: Aryl sulfatase A deficiency
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Metachromatic leukodystorphy (MLD)
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Metachromatic leukodystorphy (MLD) genetics
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Autosomal recessive; chromosome 22
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Globoid Leukodystrophy AKA
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Krabbe's Disease
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Large, globoid macrophages
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Globoid Leukodystrophy (GLD)
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Lysosome disorder: Galactocerebroside-beta - galactosidase deficiency
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Globoid Leukodystrophy (GLD)
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Globoid Leukodystrophy (GLD) genetics
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Autosomal recessive; chromosome 14
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Storage of VLCFA
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Adrenal Leukodystrophy (ALD)
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Peroxisomal enzyme defect
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Adrenal Leukodystrophy (ALD)
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Adrenal Leukodystrophy (ALD) genetics
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X-linked
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Presents in infancy as seizures, deafness, and retinal detachment
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Adrenal Leukodystrophy (ALD)
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Fiber-type grouping
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Denervation atrophy
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Floppy baby
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Werdnig-Hoffman Disease
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Wednig-Hoffman Disease fatality
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3 years
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Spinal Muscular Neuron (SMN) gene mutation
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Werdnig-Hoffman Disease
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Angular atrophic muscle fibers
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Denervation atrophy
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Target fibers
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Denervation atrophy
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Lesion most associated with Myasthenia Gravis
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Thymic lesion
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CA most associated with Lamberton-Eaton Myasthenic Syndrome
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Small-cell lung carcinoma
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Antibodies toward presynaptic voltage-gated calcium-channels
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Lamberton-Eaton Myasthenic Syndrome
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Antibodies toward postsynaptic acetylcholine receptors
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Myasthenia gravis
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NMJ disorder which improves with repetitive stimulation
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Lamberton-Eaton Myasthenic Syndrome
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NMJ disorder which fatigues with repetitive stimulation
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Myasthenia Gravis
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DMD on Xp21 deletion
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Duchenne's MD
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Hypertrophic, split fibers
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Duchenne's MD
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DMD comorbidities
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Heart failure and arrhthymias
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CTG trinucleotide repeat expansion
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Myotonic dystrophy
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hatchet facies
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Myotonic dystrophy
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Ring fibers
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Myotonic dystrophy
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Adult risk associated with dermatomyositis
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(1/4) visceral cancer
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Heliotrope rash
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Dermatomyositis
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Childhood manifestation of dermatomyositis
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calcinosis with enteric vasculitis
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CD8+ T-cell invasion of myofibers
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Polymyositis
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Ascending paralysis status-post "flu-like" illness
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Guillain-Barre Syndrome
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Guillain-Barre Syndrome AKA
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Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
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Onion bulbs
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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
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Pes Cavus
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Charcot-Marie-Tooth disease (CMT)
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In advanced cases, nerve is hypertrophic and palpable
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Charcot-Marie-Tooth disease (CMT)
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Charcot-Marie-Tooth disease (CMT) onset
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childhood (hereditary)
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Defective PMP22 gene
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Charcot-Marie-Tooth disease (CMT)
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Abnormal transthyretin gene
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Familial Amyloid Polyneuropathy
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3 most common gliomas
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Astrocytoma
Oligodenroglioma Ependymoma |
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Glial fibrillary acidic protein (GFAP) positive
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Astrocytoma
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Most common post-childhood astrocytoma
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Diffuse astrocytoma
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Astrocytoma grades
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I & II
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Anaplastic astrocytoma grade
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III; present mitoses
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Glioblastoma grade
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IV; present mitoses, necrosis, and vascular proliferation
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Astrocytoma location in adults
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2/3 in cerebrum
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Astrocytoma location in children
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2/3 in cerebellum
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Low grade astrocytoma symptoms (when present)
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ICH; seizures
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Histological "jigsaw" appearance
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Necrosis, as seen in glioblastoma
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Two types of astrocytomas
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Diffuse and pilocytic
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Rosenthal fibers
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Pilocytic astrocytoma
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Bipolar astrocytes with 2 long hair-like processes
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Pilocytic astrocytoma
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Pilocytic astrocytoma location
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Cerebellum
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Honeycomb cellular pattern with chicken-wire capillaries
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Oligodendrogliomas
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Complications associated with oligodendrogliomas
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Calcifications and spontaneous bleeding
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Histological rosettes
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Ependymoma
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Complications associated with ependymoma
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Non-communicating hydrocephalus
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Cerebellar tumor with small blue cells with little cytoplasm
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Medulloblastoma
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Medulloblastoma area of metastases
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SAS along spinal cord
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Hypersecretion of CSF
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Choroid plexus papilloma
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benign cyst situated in lateral ventricle near foramen of monroe that is position-dependant
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Colloid cyst
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Basic difference between schwannoma and neurofibroma
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Schwannoma affects schwann cells (peri-nerve fiber)
Neurofibroma affects schwann cells & fibroblasts (intra-nerve fiber) |
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Antoni A pattern
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densely packed spindle cells with nuclear palisading; associated with schwannoma
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Antoni B pattern
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cellular degeneration; associated with schwannoma
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Cafe-au-lait spots
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Neurofibromatosis
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Neurofibroma CA risk, especially post-radiation
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Neurofibrosarcoma
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Psammoma bodies
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Meningioma
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Histological whorls
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Meningioma
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Meningioma origin
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arachnoid granulations
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Lymphoma that has predilection for meninges
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Metastatic lymphoma
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Lymphoma that has predilection for white matter
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Primary CNS lymphoma
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Primary CNS lymphoma cell-type
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B-cell
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Cancers that often metastasize to brain
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Lung
Breast GI |
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Routes of brain metastasis
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Blood
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Most common cause of brain ischemia
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Atherosclerosis
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Thrombotic ischemia infarct color
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pale
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Embolic ischemia infarct color
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red
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Increases risk of CVA 5 times
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HTN
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Percentage of CO that goes to brain
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15%
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Hypotensive stroke area of vulnerability
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Watershed area
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Fat emobilism on gross exam
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white matter petechiae
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small emboli (air / thrombocytopenia) on gross exam
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gray matter petechiae
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subdural hematoma on gross exam
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blood in between sulci (on top of arachnoid membrane)
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berry aneurysm etiology
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congenital weakness of the blood vessel wall
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Segmental fibrinoid degeneration of small vessels in basal ganglia
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Lacunar infarct
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(10 hours - 5 days) post cerebral infarct
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Infiltration and digestion by neutrophils
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(24 hours - 3 weeks) post cerebral infarct
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Resorption by macrophages
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(7 days- 3 weeks) post cerebral infarct
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Vascular proliferation
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(3 days - months) post cerebral infarct
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Reactive astrogliosis, forming a glial scar. A large infarct results in cavitation.
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Penumbra
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2 zones of infarct, target zone and peripheral zone. Target zone neurons have died, and those in the peripheral zone are at risk.
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Most common cerebral infarct distribution
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Basal ganglia (70%)
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Cerebral infarct distribution which is a neurosurgical emergency
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Cerebellum (10%)
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Aneurysm most common in anterior part of the circle of Willis
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Berry (or saccular) aneurysm
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Berry aneurysm bleeds into this area
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subarachnoid space
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Aneurysm associated with HTN
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Charcot-Bouchard aneurysms
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Vertebro-basilar arteries most susceptible to this kind of aneurysm
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Atherosclerotic aneurysms
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Causes spontaneous CNS hemorrhage, which can cause seizures
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Arteriovenous malformation
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Gitter cells (lipid ladened macrophages) found in CSF is associated with...
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embolism and thrombosis
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