Nutritional 1.

The Wernicke-Korsakoff syndrome affects all of the following except The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. The hippocampus B. The mammillary bodies C. The thalamus D. The third nerve nucleus 2. Subacute combined degeneration is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. A genetic metabolic disorder B. A demyelinative disease C. A nutritional deficiency disorder D. A form of motor neuron disease E. A paraneoplastic syndrome 3. Korsakoff's amnesia can result from: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. HIE-stroke. B. Seizures. C. Both. D. Neither. 4. A 49 year old man had resection of the terminal ileum and part of the colon for Crohn's disease. Seven years later, tingling, weakness, and spasticity of the legs develops. He should be treated with: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Vitamin B12. B. Vitamin B6.

C. Plasma exchange. D. Vitamin B1. 5. A 56 year old man died of gastric carcinoma. The autopsy revealed pathological changes in the brain shown below.

The changes are due to: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Bleeding diathesis. B. Nutritional deficiency. C. Remote effects of carcinoma. D. Metastasis.

Metabolic Inherited

1. The pathology illustrated is caused by:

The current version of TESTS does not work well with mobile devices. A new version is in preparation.

A. B. C. 2.

A myelin lipid abnormality A myelin protein abnormality Both The change illustrated is due to:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. A lysosomal abnormality. B. A peroxisomal abnormality C. A mitochondrial abnormality D. Could be caused by all of the above 3. Which of the following is associated with skeletal abnormalities? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Niemann-Pick type C B. Neuronal ceroid lipofuscinosis C. Mycopolysaccharidosis D. Globoid cell leukodystrophy *4. A 4 month old by has spasticity, irritability, seizures, and an arrest of neurological development. MRI shows diffuse white matter abnormality. The condition is most likely caused by:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. ALDP deficiency B. Arylsulfatase A deficiency C. Galactocerebrosidase deficiency D. Glucocerebrosidase deficiency 5. The pathology illustrated is caused by:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. A lysosomal disorder B. A peroxisomal disorder C. A mitochondrial disorder D. An amino acid disorder *6. Which is X-linked? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Proteolipid protein 1 abnormality B. GFAP mutation C. eIEF2B mutation D. Arylsulfatase A mutation 7. Ragged red fibers occur primarily in ETC disorders caused by: The current version of TESTS does not work well with mobile devices. A new version is in preparation.

A. Mitochondrial DNA abnormalities. B. Nuclear DNA changes C. Equally both *8. Which of the following is associated with a large head? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. ALD protein deficiency B. Aspartoacylase deficiency C. Arylsulfatase A deficiency D. Proteolipid protein abnormality *9. A 23 month old child has hypotonia, developmental regression, and abnormal signal in the basal ganglia and brainstem. Serum and CSF lactate is elevated. The condition is most likely transmitted: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. From both parents B. From the mother 10. Cardiomyopathy is caused by: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Some lysosomal storage disorders B. Mitochondrial disorders C. Both D. Both *11. Which of the following causes vascular disease? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Homocystinuria B. Phenylketonuria C. Hyperglycinemia D. Maple serum urine disease

Brain Tumors

1. Which are most common overall? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Primary brain tumors B. Metastatic 2. A child with craniospinal irradiation for ALL has a higher risk for developing an astrocytoma compared to other children The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. True B. False 3. Radiation to the head and neck is a risk factor for subsequent development of meningioma The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. True B. False *4. Most primary cerebral lymphomas are: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. T-cell. B. B-cell. C. They are evenly split 5. Homer-Wright rosettes are relatively uncommon in medulloblastoma The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. True B. False 6. Low grade tumors have as many but different chromosomal abnormalities as high grade tumors do The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. True B. False

*7. A 5 year old boy had multifocal brain lesions one of which is shown below. The most likely underlying factor is:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Genetic immunodeficiency B. Previous cranial irradiation for ALL C. EBV infection D. None - this is probably a random event 8. Which of the following are the most ubiquitous neurocarcinogens? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Ambient tobacco smoke B. Industrial solvents C. Nitroso compounds D. Pesticides 9. Which of the following is not a feature of pilocytic astrocytoma? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Granular eosinophilic droplets B. Brisk mitotic activity C. Drop metastases D. Location in the spinal cord E. Hydrocephalus 10. What finding distinguishes glioblastoma multiforme from lower grade astrocytomas?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Mitoses B. Cellular density C. Necrosis D. Marked anaplasia (giant cells) *11. Which of the following lesions extends across the corpus callosum? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Glioblastoma B. Cerebral lymphoma C. Schilder's disease D. All of the above 12. Which of the following may present as a ring enhancing lesion? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Acute multiple sclerosis plaques B. Metastatic tumors C. Glioblastoma D. All of the above 13. A 3 year old boy with a history of headaches and morning vomiting for 10 days presents to the ER after a bad episode of vomiting. He is afebrile. The diagnostic studies might include all of the following except: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Fundoscopic examination B. CSF for enterovirus PCR C. GI endoscopy D. MRI *14. The most common intracranial site of germ cell tumors is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. The pineal gland

B. The pituitary gland C. The hypothalamus D. The midline of the cerebellum *15. Which of the following statements about the posterior fossa lesion shown below is not true

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. It is classified as WHO Grade I B. It evolves to malignant over many years C. Most such lesions arise in the cerebellum D. It shows vascular endothelial proliferation E. It enhances on MRI imaging *17. Which of the following statements about the lesion illustrated below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Most such lesions are WHO grade 2

B. It has the highest incidence of CSF seeding C. It may be exophytic D. It may contain areas indistinguishable from low grade astrocytoma E. It is not highly radiosensitive 18. Which of the following statements about the brain tumor in a 3 year old boy shown below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Most such tumors are supratentorial B. It can be familial C. It can cause hydrocephalus D. It may present with blurred vision E. Ataxia is relatively uncommon as a presenting sign 19. A 4 year old boy with a history of headaches and on-and-off vomiting for 2 weeks had the MRI findings shown below. The

most likely diagnosis is:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Medulloblastoma B. Glioblastoma of the cerebellum C. Ependymoma of the 4th ventricle D. Aqueductal stenosis 20. Which of the following statements about the lesion shown below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. There are over ten histological subtypes B. The tumor is attached to the dura

C. Deletion of chromosome 22 is frequent D. Brain invasion is frequent E. It is more common in women 21. Which of the statements about the lesion shown below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. It may invade bone and extend into the extracranial soft tissues B. Recurrence indicates malignancy C. It shows folding processes and dense junctions D. It is EMA positive *22. Which of the following statements about the illustrated lesion is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation.

A. It is an extra-axial lesion B. It is often seen in neurofibromatosis 1 C. It is benign D. There may be multiple tumors E. It extends along the vertebral foramina *23. Which of the following statements about the lesion shown below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. It may be an intra-spinal tumor B. Malignant degeneration is more frequent after irradiation C. The optic nerve is a common intracranial location D. It may occur in an autosomal dominant pattern

24. Which of the following is not a feature of the illustrated lesion?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Infrasellar location B. Calcification C. Gross cysts D. Papillary pattern E. Rosenthal fibers in surrounding tissue 25. Which of the following statements about the lesion shown below is not true?

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. The lesion is benign but may recur B. It resembles some tumors arising in the jaws

C. It is usually part of a benign teratoma D. It may be intraventricular 26. A 43 year old patient has had headaches and declining mental function for 5 weeks. MRI shows mild hydrocephalus with periventricular enhancing lesions. A stereotactic biopsy is obtained (shown below). The most likely diagnosis is:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. HIV encephalitis B. Progressive multifocal leukoencephalopathy C. Cerebral lymphoma D. HSV encephalitis E. Cereral abscess *27. A 42 year old woman had right-sided weakness progressing to hemiparesis in 3 to 4 weeks. MRI shows an enhancing lesion in the left centrum semiovale adjacent to the corpus callosum. A stereotactic biopsy is obtained (shown below).

The most likely diagnosis is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. An inflammatory demyelinative disease B. Cerebral lymphoma C. CNS vasculitis with ischemic infarction D. Progressive multifocal leukoencephalopathy E. Glioblastoma *28. A 57 year old patient had fever, seizures and obtundation for 4 days. MRI shows a necrotic hemorrhagic lesion of the right temporal lobe with surrounding edema. A stereotactic biopsy is shown below.

The most likely diagnosis is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Cerebral abscess

B. Hemorrhagic infarct C. Cerebral lymphoma D. HSV encephalitis *29. A 6 year old girl with diabetes insipidus, visual disturbances, and headaches for 3 weeks had an MRI scan showing a 2 cm suprasellar mass and mild hydrocephalus. A stereotactic biopsy is shown below.

The most likely diagnosis is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Craniopharyngioma B. Germ cell tumor C. Langerhans histiocytosis D. Pituitary adenoma *30. A 27 year old woman with headaches and vomiting had a cystic cerebellar mass with a protruding nodule. Stereotactic biopsy is shown below.

The most likely diagnosis is:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Metastatic clear cell carcinoma B. Hemangioblastoma C. Oligodendroglioma D. Old infarct with macrophage reaction *31. The illustrated structures may be present in:

The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Pilocytic astrocytoma B. Oligodendroglioma C. Both D.They are non-specific and may be present in diverse neoplastic and non-neoplastic conditions 32. A previously healthy 53 year old man had an insidious onset of headaches, obtundation, and facial paralysis. CSF shows 33 lymphocytes, glucose 36, and protein 72. The most likely diagnosis is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Meningeal carcinomatosis B. Tuberculous meningitis C. Subacute encephalitis D. Multiple sclerosis *33. The most common tumors of the pineal gland are: The current version of TESTS does not work well with mobile devices. A new version is in preparation.

A. Pineal parenchymal tumors (pinealoma and pineoblastoma) B. Germ cell tumors *34. You are called to the OR to do a frozen section of a suprasellar mass in a 5 year old boy. Which of the following would be most likely? The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Pituitary adenoma B. Craniopharyngioma C. Pilocytic astrocytoma D. Endodermal sinus tumor 35-40. Match the following tumors with the underlying conditions A. Neurofibromatosis 1 (VRNF) B. Neurofibromatosis 2 (BANF) C. von Hippel-Lindau disease D. Tuberous sclerosis The current version of TESTS does not work well with mobile devices. A new version is in preparation. 35. Spinal schwannoma: 35A, 35B, 35C, 35D 36. Meningioma: 36A, 36B , 36C, 36D 37. Optic nerve astrocytoma: 37A, 37B, 37C, 37D 38. Subependymal giant cell astrocytoma: 38A, 38B, 38C, 38D 39. Pheochromocytoma: 39A, 39B, 39C, 39D 40. UBO-type lesions on MRI: 40A, 40B, 40C, 40D 41-45. Match the numbered tumors with the lettered locations A.Intra-axial B.Extra-axial The current version of TESTS does not work well with mobile devices. A new version is in preparation. 41. 42. 43. 44. 45. Meningioma: 41A, 41B Ependymoma of the 4th ventricle: 42A, 42B Eight nerve schwannoma: 43A, 43B Medulloblastoma: 44A, 44B Pituitary adenoma: 45A, 45B

*46. A three year old boy had a cerebellopontine angle tumor with the ultrastructural features illustrated below.

The diagnosis is: The current version of TESTS does not work well with mobile devices. A new version is in preparation. A. Meningioma B. Ependymoma C. Schwannoma D. Exophytic pilocytic astrocytoma