Neuromuscular flashcards

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jaz_walker
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270831
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Neuromuscular flashcards
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2014-04-16 17:46:15
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  1. 1. Historically, which 2 infections caused cerebral infarction through vasculitis?
    Tuberculosis, syphillis
  2. 2. What are the 3 types of brain herniation?
    Subfalcine, transtentorial and tonsillar
  3. 3. A 50 year old male presents with vomiting and confusion.. CT shows multiple lesions, which are well-demarcated, at the gray-white junction, with oedema.. What are the 5 most common sites of primary for brain metastases?
    Lung, breast, melanoma, kidney, GI tract.
  4. 4. A baby is born with microencephaly. What are the three most common causes?
    Chromosome abnomralities, fetal alcohol syndrome, HIV-1
  5. 5. What are the two types of cerebral oedema?
    Vasogenic oedema, cytotoxic oedema
  6. 6. A 36 year old male is in a road traffic accident and dies 24 hours afterwards.. On autopsy, there are wedge shaped areas with extravasation. There is nuclear pyknosis and cytoplasmic eosinophilia.. What are these areas called?
    Contusions
  7. 7. A CT of an unconscious patient shows transtentorial herniation.. You also note flame-shaped haemorrhages in the midbrain and pons.. What are these called?
    Duret haemorrhages.
  8. 8. A 17 year old man is diagnosed with a diffuse neurofibroma and is told that there are further tests to detect an underlying cause.. What are they looking for and what gene is involved?
    Neurofibromatosis type 1 - Neurofibromin
  9. 9. A 17 year old man is diagnosed with a plexiform neurofibroma and is told that there are further tests to detect an underlying cause.. What are they looking for?
    Neurofibromatosis type 1
  10. 10. A 17 year old man is diagnosed with multiple cutaneous neurofibromas and is told that there are further tests to detect an underlying cause.. What are they looking for?
    Neurofibromatosis type 1
  11. 11. A 51 year old female presents with weakness, particularly in lifting her hands over her head and rising from a seated position. She is Gottran's sign negative. CK is raised.. What are you concerned about and what is diagnostic?
    Polymyositis - Muscle biopsy shows endomysial inflammatory infiltrates and myofiber necrosis.
  12. 12. A 67 year old female presents with insiduous onset of weakness, and recurrent falls. She has difficulty turning doorknobs and you note footdrop.. CK is elevated. Disease does not respond to steroids. Muscle biopsy shows rimmed vacuoles.. What are you concerned about and what is prognosis?
    Inclusion body myositis - prognosis is poor, this is a progressive disease
  13. 13. A 62 year old man presents with weakness. On examination, this is worst in the proximal muscles and improves with repeated movements.. What are you concerned about and what malignancy would you now look for?
    Lambert-Eaton syndrome - Small cell carcinoma
  14. 14. A 48 year old woman presents with an 8 week history of fainting spells, symmetrical weakness and 'radicular pain' shooting from her neck downwards.. What are you concerned about and what would confirm your diagnosis?
    Chronic inflammatory demyelinating polyneuropathy - nerve conduction studies
  15. 15. A 29 year old woman presents with diplopia. You note ptosis and the patient comments that the weakness fluctuates dramatically.. Investigations show thymic hyperplasia.. What are you concerned about and what would confirm your diagnosis?
    Myasthenia gravis - Cholinesterase inhibitory drugs.
  16. 16. A 5 year old boy presents with clumsiness and difficulty in PE. Examination shows weakness in the pelvic girdle and some involvement of the shoulder girdle. There is pseudohypertrophy of the calf.. Muscle biopsy shows segmental necrosis and regeneration of individual myofibres. There are inflammatory cells present.. What are you concerned about and which gene is involved?
    Duchenne muscular dystrophy - Dystrophin gene
  17. 17. A 15 year old boy presents with clumsiness and difficulty in PE. Examination shows weakness in the pelvic girdle and some involvement of the shoulder girdle. There is pseudohypertrophy of the calf.. Muscle biopsy shows segmental necrosis and regeneration of individual myofibres. There are inflammatory cells present.. What are you concerned about and which gene is involved?
    Becker muscular dystrophy - Dystrophin gene
  18. 18. A 39 year old woman presents with a rapidly progressive weakness over the past 2 days. She is now complaining of shortness of breath.. What are you concerned about?
    Guillan Barre syndrome
  19. 19. A 61 year old man with a history of poorly controlled type II diabetes complains of balance problems.. What are you concerned about?
    Peripheral neuropathy
  20. 20. A 53 year old female presents with difficulty brushing her hair. She also has a symmetrical skin rash. She is Gottran's sign positive.. Muscle biopsy shows prominent perifascicular and paraseptal atrophy.. What are you concerned about?
    Dermatomyositis
  21. 21. A 37 year old man presents with difficulty hearing.. CT head shows bilateral schwannoma. What are you concerned about?
    Neurofibromatosis type 2
  22. 22. A 30 year old pregnancy woman has raised AFP. What are you concerned about?
    Neural tube defects
  23. 23. A 31 year old woman has not been taking folate during her pregnancy.. What disorder does folate protect against?
    Neural tube defects
  24. 24. A 49 year old man presents with jaundice. He also has asterixis.. What does this mean for the brain, and which metabolite causes it?
    Hepatic encephaly - Ammonia
  25. 25. A 69 year old female dies of unknown cause.. Autopsy shows that arteries supplying the basal ganglia have hyaline arteriolar sclerosis.. What is the likely cause of this?
    Longstanding hypertension
  26. 26. A 69 year old female dies of unknown cause.. Autopsy shows Charcot-Bouchard microaneurysms. What is the likely cause of this?
    Longstanding hypertension
  27. 27. A 69 year old female dies of unknown cause.. Autopsy shows a small cavitary infarct in the deep gray matter of the thalamus.. What is the likely cause of this?
    Longstanding hypertension
  28. 28. A 69 year old female dies of unknown cause.. Autopsy shows a slitlike cavity surrounded by brownish discolouration (slit haemorrhage). What is the likely cause of this?
    Longstanding hypertension
  29. 29. A 69 year old woman presents with headaches, confusion and vomiting. She has a seizure shortly afterwards and dies.. Postmortem shows brain oedema, with tonsillar herniation. There is fibrinoid necrosis of arterioles in the gray and white matter.. What is the likely cause of this?
    Acute hypertensive encephalopathy - Longstanding hypertension
  30. 30. A 48 year old man presents with a loss of movement and pain sensation in her legs, but preservation of vibration and touch sensation.. What is the likely cause of this?
    Syringomylia
  31. 31. A 42 year old woman presents with leg weakness. Unfortunately she dies in a RTA several weeks after her symptoms resolve.. Autopsy shows multiple well circumstribed, slightly depressed lesions which are glassy-appearing and gray-tan.. What is the likely cause of this?
    Multiple sclerosis
  32. 32. A 42 year old woman presents with leg weakness.. CSF shows oligoclonal bands.. What is the likely cause of this?
    Multiple sclerosis
  33. 33. A 42 year old woman presents with eyesight problems.. CSF shows no oligoclonal bands.. What is the likely cause of this?
    Neuromyelitis optica
  34. 34. A 79 year old woman with dementia dies of pneumonia.. Autopsy of the brain shows plaques and neurofibrillary tangles. Containing tau protein.. What is the most likely cause of this?
    Alzheimer's disease
  35. 35. A 30 year old man presents with forgetfulness and chorea. He commits suicide 3 years later.. On autopsy, the brain is small, with atrophy of the caudate nucleus, and some change in the putamen. There is severe loss of neurons from these areas.. What is this and how is it inherited?
    Huntington disease - autosomal dominant inheritance
  36. 36. A 71 year old woman undergoes cardiac arrest and is found dead in her house 2 days later.. There is acute neuronal cell change, with nuclear pyknosis and karyorrhexis. There is neutrophil invasion.. What is this and how long after her arrest did she die?
    Global cerebral ischaemia - 12-24 hours (early changes have occurred)
  37. 37. A 71 year old woman undergoes cardiac arrest and is found dead in her house 2 weeks later.. There is necrosis of brain tissue, influx of macrophages, vascular proliferation and ractive gliosis.. What is this and how long after her arrest did she die?
    Global cerebral ischaemia - 24 hours - 1 week (subacute changes)
  38. 38. A 70 year old female dies in hospital of unknown cause.. Autopsy of the brain shows brain tissue of the frontal lobe is pale. Soft and swollen.. What is this and how long ago was it?
    Non-haemorrhagic infarct - 48 hours
  39. 39. A 70 year old female dies in hospital of unknown cause.. Autopsy of the brain shows brain tissue of the frontal lobe is gelatinous and friable, with a clear demarcation between normal and abnormal tissue. What is this and how long ago was it?
    Non-haemorrhagic infarct - 2 - 10 days
  40. 40. A 70 year old female dies in hospital of unknown cause.. Autopsy of the brain shows brain tissue of the frontal lobe which has neutrofilic migration and mononuclear phagocytic cells. What is this and how long ago was it?
    Non-haemorrhagic infarct - 2 days up to 2-3 weeks
  41. 41. A 70 year old female dies in hospital of unknown cause.. Autopsy of the brain shows brain tissue of the frontal lobe which has swollen glial cells and 'red neurons'. There is blood extravasation and resorption. What is this and how long ago was it?
    haemorrhagic infarct - 12-48 hours
  42. 42. A 70 year old female dies in hospital of unknown cause.. Autopsy of the brain shows brain tissue of the frontal lobe which has swollen glial cells and 'red neurons'. What is this and how long ago was it?
    Non-haemorrhagic infarct - 14 hours
  43. 43. A 46 year old female presents with eyesight problems.. CT shows a dura-based mass which is on the midline and has a dural tail.. What is this and what are multiple such lesions associated with?
    Meningioma - Neurofibromatosis type 2
  44. 44. A 81 year old woman with a history of alcoholism presents with confusion.. CT shows haemorrhage and necrosis in the mammillary bodies and adjacent to the third and fourth ventricles.. What is this and what causes it?
    Wernicke's encephalopathy - Thiamine deficiency
  45. 45. A 26 year old female presents with a large, poorly defined soft tissue mass on their arm.. Histology shows a tumour which is highly cellular and has features of overt malignancy. It is described as 'marble-like'.. What is this and what condition is associated with it?
    Malignant peripheral nerve sheath tumour - Neurofibromatosis type 1
  46. 46. A 82 year old man dies at home of unknown cause.. At autopsy there are wedge shaped areas of brain infarct uccurring at the most distal portions of arterial territories.. What is this and what does it tell you?
    Watershed infarcts - A hypotensive episode occurred e.g. cardiac arrest.
  47. 47. A 7 year old boy presents with seizures.. CT shows a lesion in the midline of the cerebellum. It is well circumscribed, gray and friable, on surgery. On biopsy, there are small blue cells, with little cytoplasm and hyperchromacity.. What is this and what is the best treatment?
    Medulloblastoma - radiotherapy
  48. 48. A 28 year old female presents with seizures.. she has hemangioblastomas in the cerebellar hemispheres. There are also cysts in the pancreas and kidneys.. What is this and what non-head tumour is associated?
    von Hippel-Linau Disease - renal cell carcinoma
  49. 49. A 29 year old male presents with ataxia.. CT shows a cerebellar lesion. On biopsy it is a mural nodule with a large, fluid-filled cyst. On microscopy, there are numeros capillary-sized vessels.. What is this and what non-head tumour is associated?
    von Hippel-Linau Disease - renal cell carcinoma
  50. 50. A 39 year old male is found, dead, on the roadside.. Autopsy shows a depressed, retracted, yellowish brown patch on the crest of a gyrus of the brain.. What is this and when was it?
    Contusions - This is an old injury.
  51. 51. A 69 year old male presents with a sudden, terrible headache which started whilst straining at stool. He loses consciousness and dies soon after.. What is this and which genetic disease gives a high risk for this?
    Subarachnoid haemorrhage (berry aneurysm) - ADPCK disease
  52. 52. A 19 year old male presents with seizures.. CT head shows a tangled network of vascular channels.. What is this likely to be?
    AV malformation
  53. 53. A 69 year old man presents with confusion. He dies soon afterwards.. The parenchymal and subarachnoid vessels have chronic inflammation, multinucleate giant cells and the vessel walls are damaged. A diffuse encephalopathy is present.. What is this, and what would treatment have been?
    Primary angiitis of the CNS - immunosuppressants
  54. 54. A 6 year old female presents with confusion and epileptic seizures.. CT shows a portion of the ventricle is enlarged, but a portion is not.. What is this?
    Non-communicating hydrocephalus
  55. 55. A 6 year old female presents with confusion and epileptic seizures.. CT shows the entire ventricular system is enlarged. What is this?
    Communicating hydrocephalus
  56. 56. A 71 year old woman undergoes cardiac arrest and dies.. On autopsy, the brain is swollen, with wide gyri and narrowed sulci. The cut surface shows poor demarcation between gray and white matter.. What is this?
    Global cerebral ischaemia
  57. 57. A 61 year old man with a history of hypertension dies suddenly.. Autopsy shows haemorrhage in the basal ganglia, with extravasated blood. What is this?
    Subarachnoid haemorrhage
  58. 58. A 73 year old female dies suddenly.. Autopsy shows a lobqr haemorrhage. Congo red stain shows amyloid in vessel walls, causing wall rigidity.. What is this?
    Cerebral amyloid angiopathy
  59. 59. A 27 year old female is in a RTA and is briefly conscious afterwards, but then falls into a coma and eventually dies.. There is no evidence of damage on CT. On autopsy, there are asymmetrical injuries, which are near the angles of the lateral ventricles and brain stain. It is best seen with silver stain.. What is this?
    Diffuse axonal injury
  60. 60. A 1 year old female presents with developmental delay. She has displayed hand-wringing, breath-holding.. Genetic testing shows an X linked abnormality of MECP2. What is this?
    Rett syndrome
  61. 61. A 30 week pregnant mother has a stillborn baby.. Autopsy shows the brain has absent gyration, with a thickened cortex with only 4 layers.. What is this?
    Lissencephaly
  62. 62. A 30 week pregnant mother has a stillborn baby.. The brain has a bumpy, cobble-stone like surface.. What is this?
    Polymicrogyria
  63. 63. A 30 week pregnant mother has a stillborn baby.. The brain is not divided into hempispheres or lobes. There is cyclopia.. What is this?
    Holoprosencephaly
  64. 64. A newborn baby is seen to have a lumbar myelomeningocele on newborn baby check.. CT shows a small posterior fossa with a mishapen midline cerebellum. The vermis is extended through the foramen magnum and there is hydrocephalus. What is this?
    Arnold-Chiari malformation
  65. 65. A newborn baby was noted on prenatal scans to have an enlarged 4th ventricle. CT shows enlarged posterior fossa, absence of the cerebellar vermis and a large midline cyst. What is this?
    Dandy-Walker malformation
  66. 66. A 47 year old patient with AIDS presents with weakness and eyesight problems.. CSF shows JC virus. What is this?
    Progressive multifocal leukoencephalopathy.
  67. 67. A 3 year old male presents with developmental delay. He has spasticity and ataxia.. After death the autopsy shows the white matter is gray and translucent, and decreased volume of white matter. The ventricles are enlarged.. What is this?
    Leukodystrophy
  68. 68. A 79 year old man presents with personality changes and depression.. postmortem shows brain oedema, with tonsillar herniation. There is fibrinoid necrosis of arterioles in the gray and white matter.. What is this?
    Pick disease, a type of trontotemporal lobar degeneration
  69. 69. A 71 year old woman presents with a tremor. She dies 15 years later.. At autopsy there is pallor of the substantia nigra/ Lewy bodies are present.. What is this?
    Parkinson disease.
  70. 70. A 48 year old man presents with foot weakness. You note some fasciculations. He dies 18 years later. On autopsy you note anterior roots of the spinal cord are thin and gray. The precentral gyrus is mildly atrophic.. What is this?
    Amyotrophic lateral sclerosis
  71. 71. A 40 year old male presents with seizures. He also comments on a headache which is worst in the morning.. On CT there is a mass in the right cerebral hemisphere. It is invasive. On surgery, the cut surface is firm, with cystic degeneration. It is heterogeneous.. What is this?
    Astrocytoma
  72. 72. A 16 year old girl presents with seizures.. CT shows a cerebellar lesion. It is cystic. On resection, there are rosenthal fibres, eosinophilic granular bodies and microcysts. What is this?
    pilocytic astrocytoma
  73. 73. A 38 year old man presents with a 2 year history of worsening early-morning headache and has recently had a fit.. CT shows a frontal lesion on the LHS. It is resected, and is a gelatinous, gray mass, with areas of focal haemorrhage. On microscopy, there are regular cells with spherical nuclei, with finely granular-appearing chromatin.. What is this?
    Oligodenroglioma
  74. 74. A 16 year old boy presents with early morning headaches.. CT shows a lesion by the ventricular system. On resection, it is a papillary mass, with regular cells. There are rosettes and canals.. What is this?
    Ependymoma
  75. 75. An immunosuppressed 71 year old female presents with seizures.. CT shows a lesion in the thalmus which is well defined, with extensive necrosis.. What is this?
    Primary B cell lymphoma. - This is the most common CNS neoplasm in immunosuppressed people.
  76. 76. A 8 year old boy presents with trouble in school. He also has learning difficulties.. CT shows CNS hamartomas. There are cysts in the liver, kidneys and pancreas.. What is this?
    Tuberous sclerosis
  77. 77. A 87 year old woman with Alzheimer's dies of unknown cause. There is a collection of clotted blood apposed to the contour of the brain without extending into the depths of the sulci. There is growth of granulation tissue from the dural surface into the hematoma. What kind of hematoma are you concerned about and how long ago was it?
    Subdural hematoma - 2 weeks
  78. 78. A 87 year old woman with Alzheimer's dies of unknown cause. There is a collection of clotted blood apposed to the contour of the brain without extending into the depths of the sulci. There is fibrosis from the dural surface into the hematoma. What kind of hematoma are you concerned about and how long ago was it?
    Subdural hematoma - 1-3 months
  79. 79. A father accidentally drops an infant.. What kind of hematoma are you concerned about and which vessel does this involve?
    Epidural hematoma - Middle meningeal artery
  80. 80. A 35 year old male presents with unconsciousness which only onsets several hours after falling a metre onto his head.. What kind of hematoma are you concerned about and which vessel does this involve?
    Epidural hematoma - Middle meningeal artery
  81. 81. A 87 year old woman with Alzheimer's presents with headache and more confusion than normal.. What kind of hematoma are you concerned about and which vessel does this involve?
    Subdural hematoma - Bridging veins
  82. 82. A glioma in the parietal region causes displacement of the cingulate gyrus.. What kind of herniation is likely and which vessel is at risk?
    Subfalcine herniation - anterior cerebral artery is at risk
  83. 83. A 67 year old male has a MRI head for screening.. A aneurysm is visible on the circle of Willis.. What kind of stroke does this put him at risk for?
    Subarachnoid haemorrhage
  84. 84. A 6 year old boy presents with gait disturbance and hand clumsiness.. What may this be and what diseases are there high risk of?
    Freidrich ataxia - cardiac diseae and diabetes.
  85. 85. A 71 year old woman undergoes cardiac arrest and is found dead in her house 2 weeks later.. What might you expect in her brain at autopsy?
    pseudolaminar necrosis, repair.
  86. 86. A 75 year old man with a tremor dies of unrelated causes.. Autopsy shows accumulation of a-Synuclein in the brain. Which 2 diseases are associated with this?
    Parkinson disease and multiple system atrophy
  87. 87. Which 3 opportunistic infections can cause cerebral infarction through vasculitis?
    Aspergillosis, herpes zoster, CMV
  88. 88. A CT of an unconscious patient shows transtentorial herniation.. Which artery and which nerve is most at risk?
    Third cranial nerve (causing a 'blown' pupil) - Posterior cerebral artery
  89. 89. A 59 year old woman presents with confabulation.. Which part of the brain correlates with her memory problems and what disease is most classically associated?
    Korsakoff's syndrome - Medial dorsal nucleus
  90. 90. A CT of an unconscious patient shows tonsillar herniation.. Which part of the brain is at risk?
    Medulla

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