Systems Path Exam #2: CNS

The type of epilepsy that manifests as a general seizure with a tonic, clonic, and recovery phase. A. Grand mal B. Petit mal C. Jacksonian D. Temporal lobe

A. Grand mal

An autosomal recessive disorder that is lethal in infancy or early childhood, and is characterized by the accumulation of a ganglioside within the neurons of the CNS. Retinal involvement produces a cherry-red spot on the macula. A. Tay-Sach's disease B. Gaucher's disease C. ALD (Adrenoleukodystrophy) D. MLD (Metachromatic leukodystrophy) E. Wilson's disease

A. Tay-Sach's disease

The disease/condition that is inherited with an autosomal dominant mode of inheritance that manifests with cafe-au-lait spots of the skin together with a progressive development of neurofibromas is known as: A. Type I Neurofibromatosis B. Type II Neurofibromatosis C. Creutzfeldt-Jacob disease D. AOTA E. Only A and C

A. Type I Neurofibromatosis

In myelomeningocele the protrusion of the defective spinal canal contains: A. Meninges and displaced parts of the cerebellum B. Meninges and vertebral bodies C. Meninges and portion of spinal cord D. Meninges only E. Skin only

C. Meninges and portion of spinal cord

During an automobile accident your patient strikes their head hard on the forehead area (frontal lobe region). The acceleration-deceleration injury results in a countercoup lesion. Where would that be? A. In the basal ganglia B. In the brain stem C. On the patient's left cerebellar lobe (hemisphere) D. Occipital lobe E. Right parietal cortex

D. Occipital lobe

This is a disease produced by degeneration of the substantial nigra that leads to lack of dopamine in the basal ganglia. A. Alzheimer's B. Amyotrophic lateral sclerosis C. Huntington's Chorea D. Parkinson's disease E. Friedreich's ataxia

D. Parkinson's disease

Little's disease by another name is called what? A. Anencephaly B. Generalized neurofibromatosis C. Petit mal epilepsy D. Spastic diplegia E. Dwarfism

D. Spastic diplegia

This newborn has a high fever that peaked quickly. She is extremely "fussy" and exhibits a positive reaction to Brudzinski's sign. Which combination below would be most likely? A. Viral meningitis; Echovirus B. Paretic neruosyphilis; congenital infection C. Encephalomyelitis; Coxsackie arbovirus D. Suppurative meningitis; Group B strep agalactiae E. NOTA

D. Suppurative meningitis; Group B strep agalactiae

Which of the following is the name given to the malformation of the spinal cord that presents in adulthood with a dissociated anesthesia that has a cape-like distribution? A. Spina bifida occulta B. Arnold-Chiari malformation C. Hydrocephalus D. Rachischisis E. Syringomyelia

E. Syringomyelia

Generally speaking, this is considered the most common primary glial cell tumor of the CNS, all ages included. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

F. Astrocytoma

This is a degenerative disease that produces a progressive dementia with the histological picture of neurofibrillary tangles, senile plaques, and amyloid infiltration of blood vessels. A. Alzheimer's B. Amyotrophic lateral sclerosis C. Huntington's Chorea D. Parkinson's disease E. Friedreich's ataxia

A. Alzheimer's

A 55-YO male who has been healthy all his life is now afflicted with progressive, symmetric muscular weakness. This first involved the head and neck region, such that he had difficulty in speaking, in making eye movements, and with swallowing. In the past year, the weakness in upper and lower extremities has increased, and he can no longer stand, walk, and feed himself. He most likely has: A. Amyotrophic lateral sclerosis B. Huntington disease C. Multiple sclerosis D. Guillain-Barre syndrome E. Parkinson disease

A. Amyotrophic lateral sclerosis

Which of the following is classified as a prion encephalopathy in which the patient experiences a rapidly progressive dementia, ataxia, weakness, and eventually death within 1-2 years? A. Creutzfeldt-Jacob disease B. Amyotrophic lateral sclerosis C. Progressive multifocal leukoencephalopathy D. Metachromatic leukodystropy

A. Creutzfeldt-Jacob disease

Which of the following is/are secondary causes of CNS pathology? A. Diabetes mellitus B. Cerebrovascular accident C. Hydrocephalus D. Head injury E. Intracranial neoplasia

A. Diabetes mellitus *All others are primary causes

Bleeding caused by rupture of the middle meningeal artery. A. Epidural B. Subdural C. Subarachnoid D. Intracerebral E. NOTA

A. Epidural

Which of the following is characterized by Cowdry inclusion bodies in neurons? A. Herpes simplex encephalitis B. Rabies C. Progressive multifocal leukoencephalopathy D. Toxoplasmosis E. Jakob-Creutzfeldt disease

A. Herpes simplex encephalitis

In Wilson's disease free copper is deposited in the: A. Lenticulate nucleus B. Globus pallidus C. Inferior olivary nucleus D. Dentate nucleus E. Hippocampus

A. Lenticulate nucleus

Knife edge atrophic gyro in the front-temporal lobes on one side, intensely argentophilic cytoplasmic inclusion bodies and a patient who is dead before the age of 47 was suffering dementia. This best describes what condition? A. Lobar sclerosis (Pick's disease) B. Huntington's chorea C. Alzheimer's disease D. ALS

A. Lobar sclerosis (Pick's disease)

Which of the following is often seen in a case of viral encephalitis? A. Lymphocytic perivascular cuffing B. Neurofibrillary tangles C. Granulovacuolar degeneration D. Neuritic plagues E. AOTA

A. Lymphocytic perivascular cuffing

The most common cause of bacterial induced acute leptomeningitis in young adults is: A. Neisseria meningitis B. Haemophilus influenzae C. Staphylococcus aureus D. Streptococcus pneumoniae E. Streptococcus viridans

A. Neisseria meningitis

A well circumscribed glioma of myelin producing cells within the CNS. Demonstrates speckled calcification on radiograph. Mid-life tumor. Good prognosis following resection. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

A. Oligodendroglioma

Which of the following is the viral illness that is characterized by muscle paralysis due to destruction of the anterior horn cells? A. Poliomyelitis B. Guillian-Barre syndrome C. Post-infectious encephalomyelitis D. Neurosyphilis E. Multiple sclerosis

A. Poliomyelitis

Demyelination of the white matter of the brain is an outstanding feature of: A. Progressive multifocal leukoencephalopathy B. Tay-Sach's disease C. Alzheimer's disease D. Hepatic encephalopathy E. NOTA

A. Progressive multifocal leukoencephalopathy

The viral encephalitis that is seen in patients who have AIDS, but is actually due to infection with a papovavirus A. Progressive multifocal leukoencephalopathy B. Rabies C. CMV D. Herpes simplex E. Subacute sclerosing panencephalitis

A. Progressive multifocal leukoencephalopathy

On examination of the CSF obtained by a lumbar puncture of a patient the following results are found in the laboratory: Pressure is increased, CSF appears clear, protein level is increased, glucose level is normal, cells that are present are predominantly lymphocytes. Based on these findings what is the most likely diagnosis? A. Acute suppurative meningitis B. Acute viral meningitis C. Chronic tuberculous meningitis D. Cryptococcal meningitis E. AOTA

B. Acute viral meningitis

The disease of the CNS that begins with degeneration of the anterior horn cells, usually in the lumbosacral region, and progresses by a retrograde degeneration to affect the cells of the pyramidal system. This produces progressive weakness and death. A. Alzheimer's B. Amyotrophic lateral sclerosis C. Huntington's Chorea D. Parkinson's disease E. Friedreich's ataxia

B. Amyotrophic lateral sclerosis

What is the site of the abnormality in Amyotrophic Lateral Sclerosis? A. Pigmented nucleus of brain stem B. Anterior horn of spinal cord C. Dorsal root ganglion D. Peripheral nerve E. Neuromuscular junction

B. Anterior horn of spinal cord

The cause of immediate death following a high velocity bullet wound to the head. A. Cerebral hemorrhage B. Brain herniations due to expansion of propellant gases C. Death of neurons D. Inflammation of brain substances E. Block of CSF circulation

B. Brain herniations due to expansion of propellant gases

The effects of blunt trauma causing brain damage on the opposite side of the brain to the site of injury. A. Coup injury B. Contre coup injury C. Converting coup injury D. Concussion E. Coma

B. Contre coup injury

As stated in class, the most common factor associated with the etiology of epilepsy is what? A. Neoplasms B. Idiopathic C. Head injury D. Metabolic disease of some type E. Infection (meningitis)

B. Idiopathic

The disease that is defined as a non-progressive motor disorder that is present at birth and effects only one side of the body due to unilateral thrombosis or infection of the cerebral hemispheres. A. Little's disease B. Infantile hemiplegia C. Spastic diplegia D. AOTA E. Only A and C

B. Infantile hemiplegia

Occlusion of the origin of the right middle cerebral artery will produce: A. Right hemiplegia B. Left hemiplegia C. Pseudobulbar palsy D. Paraplegia E. NOTA

B. Left hemiplegia

Highly malignant tumors exclusively found in the cerebellum of children are called: A. Glioblastoma multiforme B. Medulloblastoma C. Ependymoma D. Meningioma E. Oligodendroglioma

B. Medulloblastoma

Well circumscribed tumor attached to the dura mater. Possesses estrogen receptors. Primarily seen in middle aged females. Foci of calcifications, called psammoma bodies, are usually evidenced. Easily resected. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

B. Meningioma

Which of the following is the headache that is manifested usually with prodromal visual signs due to vasoconstriction and then a one-sided headache due to vasodilation? A. Meningitis B. Migraine C. Increased intracranial pressure D. Tumor

B. Migraine

Which of the following is NOT a microscopic characteristic of Alzheimer's disease? A. Granulovacuolar degeneration B. Perivascular "cuffing" C. Neuritic plaques D. Neurofibrillary tangles E. AOTA

B. Perivascular "cuffing"

Which of the following is NOT a microscopic characteristic of Alzheimer's disease? A. Hirano bodies B. Perivascular "cuffing" C. Neuritic plaques D. Neurofibrillary tangles E. AOTA

B. Perivascular "cuffing"

Which of the following is an example of a generalized, global, diffuse seizure in which there is an absence of visible convulsion behavior? On an EEG there is a classic 3-Hz "spike and wave" activity. This type of seizure is also seen primarily in children. A. Grand mal epilepsy B. Petit mal epilepsy C. Jacksonian epilepsy D. Myoclonic epilepsy E. Temporal lobe epilepsy

B. Petit mal epilepsy

Which of the following is an example of a generalized, global, diffuse, seizure in which there is an absence of visible convulsive behavior? On an EEG there is a classic 3-Hz "spike and wave" activity. This type of seizure is also seen primarily in children. A. Grand mal epilepsy B. Petit mal epilepsy C. Jacksonian epilepsy D. Myoclonic epilepsy E. Temporal lobe epilepsy

B. Petit mal epilepsy

Which of the following is the congenital condition in which there is the presence of a unilateral "port wine stain" on the face associated with a congenital malformation of the meningeal vessels that can lead to seizures and mental retardation? A. Berry aneurysm B. Sturge-Weber syndrome C. Arnold-Chiari malformation D. Little's disease E. Capillary hemangioma

B. Sturge-Weber syndrome

Which of the following is the name given to the congenital malformation of blood vessels in which there is a cavernous angioma of the face (port wine stain) in combination with the angioma continuing through the skull into the inside of the cranium producing neurological problems? A. Marfans syndrome B. Sturge-Weber syndrome C. Arnold-Chiari malformation D. Berry aneurysm E. Takayasu's disease

B. Sturge-Weber syndrome

Ruptured saccular congenital aneurysms, so-called berry aneurysms, cause hemorrhage that is best classified as: A. Subdural B. Subarachnoid C. Epidural D. Intracerebral E. Intraventricular

B. Subarachnoid

A 45-YO male has been healthy all of his life until the recent onset of headaches. A cerebral angiogram demonstrates a 7-mm saccular aneurysm at the trifurcation of the right middle cerebral artery. Which of the following events is most likely to result from this lesion? A. Epidural hematoma B. Subarachnoid hemorrhage C. Subdural hematoma D. Cerebellar tonsillar herniation E. Hydrocephalus

B. Subarachnoid hemorrhage

Trauma to the brain with the resultant tear of the meningeal veins and a slow venous hemorrhage is most likely to result in which of the following? A. Subarachnoid hemorrhage B. Subdural hematoma C. Epidural hematoma D. AOTA E. A and C only

B. Subdural hematoma

The condition of the spinal cord that is seen in tertiary syphilis and produces locomotor ataxia due to loss of vibration sense and proprioception. A. General paresis of the insane B. Tabes dorsalis C. Meningovascular syphilis D. Argyll-Robertson pupil E. Gummatous necrosis

B. Tabes dorsalis

An older patient complains of several episodes of loss of speech. These episodes usually last for only a few minutes and then speech returns with no apparent change in neurological function. Auscultation of the carotid arteries produces the finding of a bruit. What is the most likely explanation of the above history? A. The patient is experiencing recurrent bleeding from a Berry aneurysm B. The patient is experiencing embolic phenomenon producing Transient Ischemic attacks C. The patient is experiencing recurrent cerebral infarctions due to thrombosis in cerebral blood vessels D. The patient is experiencing multiple epileptic seizures following trauma E. The patient is imagining the whole thing

B. The patient is experiencing embolic phenomenon producing transient ischemic attacks

You are practicing in a small town along the Mississippi coast. It is late summer. Several of your patients complain of constant exhaustion, stiff neck, blurred vision. A few develop a high fever. One patient develops ophthalmoplegia. Which condition below might you consider before the others? A. Rabies (animal borne) B. Viral encephalitis (arthropod borne) C. Prion encephalopathy (food contaminant) D. Toxic neuropathy (water contaminant) E. NOTA

B. Viral encephalitis (arthropod borne)

This disease is transmitted genetically as autosomal dominant. The onset of the characteristic physical manifestations usually arise in the 4th-5th decades with a progressive intellectual deterioration, ceaseless involuntary choreiform movements, paranoid delusions, and personality changes. Death occurs within 10 years and is usually a result of respiratory complications. A. Parkinson's disease B. Multiple sclerosis C. Huntington's disease D. Tabes dorsales E. NOTA

C .Huntington's disease

An elderly patient presents with increasing confusion, dizziness, and headaches. On questioning, the patient remembers falling and hitting their head 4 weeks previously. What is the most likely diagnosis? A. Epidural hemorrhage B. Acute subdural hemorrhage C. Chronic subdural hemorrhage D. Subarachnoid hemorrhage E. Intracerebral hemorrhage

C. Chronic subdural hemorrhage

An upper motor neuron lesion is associated with: A. Extension of the arms B. Flexion of the legs C. Dorsiflexion of the big toe when the sole of the foot is stroked D. Flaccidity of paralyzed muscles E. Diminution of the tendon reflexes

C. Dorsiflexion of the big toe when the sole of the foot is stroked

A tumor which develops from cells which line the ventricles of the CNS. Most often found in the 4th ventricle in children and young adults. Also found in spinal cord. Slow growth. Resection is a cure. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

C. Ependymoma

Which of the following is the most common cause of seizures in a child age 1-5 years? A. Idiopathic epilepsy B. Infections C. Febrile seizures D. Brain neoplasm E. Congenital abnormality

C. Febrile seizures

A 7-YO child develops headaches, vomiting, and a staggering gait. A cerebellar neoplasm is diagnosed. The neoplasm is MOST likely a(n): A. Ependymoma B. Glioblastoma multiforme C. Medulloblastoma D. Neuroblastoma E. Oligodendroglioma

C. Medulloblastoma

The most common type of neoplasms found in the CNS are what? A. Congenital neoplasms B. Astrocytomas C. Metastatic neoplasms D. Oligodendrogliomas

C. Metastatic neoplasms

Which of the following is the type of Spina bifida that is most commonly associated with serious neurological defects? A. Spina bifida occulta B. Meningocele C. Myelomeningocele D. Anencephaly

C. Myelomeningocele

Following an injury to the CNS it is found that there are collections of neutrophil leukocytes around a dead neuronal cell body. What is the name given to the process that is taking place here? A. Chromatolysis B. Atrophy C. Neuronophagia D. Wallerian degeneration

C. Neuronophagia

The cerebrospinal fluid in meningococcal meningitis does NOT show: A. Raised pressure B. Raised protein content C. Normal glucose level D. Many polymorphs E. Gram-negative intracellular cocci

C. Normal glucose level

A young male complains of a sudden onset of severe headache during athletic activity. The headache is described as having "bursting" quality. This headache is followed within a few hours by the development of a coma. Lumbar puncture examination of the CSF shows the presence of blood. What vascular abnormality is most likely the cause of this problem? A. Rupture of an intracerebral aneurysm B. Acute, bacterial meningitis C. Rupture of a Berry aneurysm D. Thrombosis of the internal carotid artery E. Cerebral edema following a head injury

C. Rupture of a Berry aneurysm

Which of the following is the infection of the CNS that has been shown to occur some years after a previous clinical measles infection. This causes a progressive dementia that leads to death after several years. A. Progressive multifocal leukoencephalopathy (PML) B. Postinfectious encephalitis C. Subacute sclerosing panencephalitis (SSPE) D. Spongiform encephalopathy E. Multiple sclerosis

C. Subacute sclerosing panencephalitis

Which of the following is the infection of the CNS that has been shown to occur some years after a previous clinical measles infection. This causes a progressive dementia that leads to death after several years. A. Progressive multifocal leukoencephalopathy (PML) B. Postinfectious encephalitis C. Subacute sclerosing panencephalitis (SSPE) D. Spongiform encephalopathy E. Multiple sclerosis

C. Subacute sclerosing panencephalitis (SSPE)

A patient is found to have loss of position sense and proprioception in the lower extremities leading to a "high stepping and slapping" gait. In which of the following conditions is this type of gait a characteristic manifestation? A. General paresis of the insane B. Meningovascular syphilis C. Tabes dorsalis D. Syringomyelia E. Poliomyelitis

C. Tabes dorsalis

Which one of the following organisms may infect the fetus during 3rd trimester of pregnancy by a transplacental route and cause necrotizing calcification in the brain? A. Neisseria gonorrhea B. Rabies virus C. Toxoplasma gondii D. Candida albicans

C. Toxoplasma gondii

A child is found to have a hemiplegia associated with convulsions and a mild mental retardation. On questioning of the parents you discover that the child has been affected this way since birth. What is the most likely cause of this condition? A. Prematurity B. Hypoxia during a full term delivery and difficult labor C. Unilateral thrombosis of cerebral vessels in utero D. Spina bifida E. Congenital hydrocephalus

C. Unilateral thrombosis of cerebral vessels in utero

A serious complication associated with meningococcal meningitis in which there is vasomotor collapse, widespread skin hemorrhages, and bilateral adrenal hemorrhages caused by meningococcal sepsis is known as: A. Kluver-Bucy syndrome B. Marie-Charcot syndrome C. Waterhouse-Friderichsen syndrome D. Sturge-Weber syndrome

C. Waterhouse-Friderichsen syndrome

Which of the following is an inherited demyelinating disease that is characterized by accumulation of very long chain fatty acids (VLCFAs) in tissues causing neuronal degeneration and failure of the adrenal cortex. This condition has an X-linked recessive mode of inheritance. A. Multiple sclerosis B. Progressive multifocal leukoencephalopathy C. Metachromatic Leukodystrophy D. Adrenoleukodystrophy E. Gauchers disease

D. Adrenoleukodystrophy

Which of the following is an inherited demyelinating disease that is characterized by accumulation of very long chain fatty acids (VLCFAs) in tissues causing neuronal degeneration and failure of the adrenal cortex. This condition has an X-linked recessive mode of inheritance. A. Multiple sclerosis B. Progressive multifocal leukoencephalopathy C. Metachromatic leukodystrophy D. Adrenoleukodystrophy E. Gaucher's disease

D. Adrenoleukodystrophy

A disease/condition is defined as being a congenital malformation in which a flattened occiput causes the herniation of the cerebellar tonsils through the foramen magnum thus compressing the brain stem and causing hydrocephalus. This is often associated with spina bifida. Which of the following diseases/conditions is it? A. Anencephaly B. Sturge-Weber syndrome C. Craniopharyngioma D. Arnold-Chiari malformation E. Congenital hydrocephalus

D. Arnold-Chiari malformation

A disease/condition is defined as being congenital malformation in which a flattened occiput causes a herniation of the cerebellar tonsils through the foramen magnum thus compressing the brain stem and causing hydrocephalus. This is often associated with spina bifida. Which of the following diseases/conditions is it? A. Anencephaly B. Sturge-Weber syndrome C. Craniopharyngioma D. Arnold-Chiari malformation E. Congenital hydrocephalus

D. Arnold-Chiari malformation

Which of the following forms of brain injury does not cause significant macroscopic or microscopic changes in the brain? A. Brain contusion B. Brain laceration C. Coup lesion D. Brain concussion

D. Brain concussion

Hepatolenticular degeneration is an inherited metabolic disorder in which the patient accumulates excess _____ in the denticulate nucleus and the liver. It appears to be associated with a deficiency in a carrier protein called cerulosplasmin. A. Iron B. Sphingomyelin C. Phenylalanine D. Copper

D. Copper

The tumor of the CNS that is found most commonly in the 4th ventricle inside the skull or in the central canal of the spinal cord. A. Astrocytoma B. Oligodendroglioma C. Medulloblastoma D. Ependymoma E. Meningioma

D. Ependymoma

A 50-YO man develops headaches, vomiting, apathy, convulsions, and papilledema. On radiologic examination, a butterfly-like lesion involving both cerebral hemispheres and extending through the corpus callosum is found. Biopsy reveals a grade IV tumor with pleomorphic hyper chromatic cells around foci of hemorrhagic necrosis. The MOST likely diagnosis is: A. Astrocytoma B. Ependymoma C. Metastatic carcinoma D. Glioblastoma multiforme E. Medulloblastoma

D. Glioblastoma multiforme

This is considered the most aggressive malignant tumor of all brain neoplasms. Can spread across the midline. Death usually occurs within one year. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

D. Glioblastoma multiforme

A person develops a post-viral ascending paralysis and experiences some respiratory paralysis, which is supported by a ventilator. In a few weeks he recovers his function completely. What is the most likely diagnosis? A. Multiple sclerosis B. St. Louis encephalitis C. Amyotrophic lateral sclerosis D. Guillain-Barre syndrome E. Tourette's syndrome

D. Guillain-Barre syndrome

Which of the viruses that cause encephalitis affect predominately the temporal lobes producing a hemorrhagic necrosis of that area of the brain? A. Arbovirus B. Rabies virus C. Cytomegalovirus (CMV) D. Herpes simplex virus E. Polio virus

D. Herpes simplex virus

Which of the viruses that cause encephalitis effect predominately the temporal lobes producing a hemorrhagic necrosis of that area of the brain? A. Arbovirus B. Rabies virus C. Cytomegalovirus (CMV) D. Herpes simplex virus E. Polio virus

D. Herpes simplex virus

A patient presents with episodes of involuntary muscle twitches that start in the right hand and then move proximally to involve the arm and shoulder girdle but with no associated loss of consciousness. What is the name given to this manifestation? A. Grand mal epilepsy B. Petit mal epilepsy C. Partial epilepsy D. Jacksonian epilepsy E. Myotonic epilepsy

D. Jacksonian epilepsy

A previously well 40-YO woman suddenly notices impairment of her sight together with impairment of speech. She slowly recovers but 6 months later experiences an episode of leg weakness and unsteadiness of gait. This recovers but further attacks occur. The most likely diagnosis is: A. Parkinson's disease B. Glioma C. Meningioma D. Multiple sclerosis E. Recurrent cerebral thrombosis or emboli

D. Multiple sclerosis

A frequent cause of cerebral embolism in the days following a myocardial infarction. A. Deep venous thrombosis B. Carotid atherosclerosis C. Vertebral artery atherosclerosis D. Mural clot in left ventricle

D. Mural clot in left ventricle

The mechanism of hydrocephalus caused by Arnold-Chiari malformation is: A. Inflammatory B. Over production of CSF C. Deficient absorption of CSF D. Obstruction due to tonsillar herniation E. Idiopathic

D. Obstruction due to tonsillar herniation

Which of the following is part of the diseases of the CNS known as the spongiform encephalopathies? A. Scrapie B. Mad cow disease C. Kuru D. Jakob-Creutzfeldt disease E. AOTA

E. AOTA

Which of the following diseases is not characterized primarily by demyelination? A. Multiple sclerosis B. Metachromatic leukodystrophy C. Post-infectious encephalitis D. Progressive multifocal leukoencephalopathy E. Alzheimer's disease

E. Alzheimer's disease

This autosomal recessive disease presents in late childhood. Lesions are isolated primarily in the cerebellum, spinal cord, or brainstem. Features are Pes cavus, Babinski's sign, staggering gait or rigidity, with eventual myocardial complications. It is what? A. Amyotrophic lateral sclerosis B. Congenital syphilis C. Sturge-Weber syndrome D. Congenital hydrocephalus E. Friedreich's ataxia

E. Friedreich's ataxia

This is an inherited disease that manifests in late childhood as clumsiness and tremor with a staggering gait and unsteadiness on standing. A. Alzheimer's B. Amyotrophic lateral sclerosis C. Huntington's Chorea D. Parkinson's disease E. Friedreich's ataxia

E. Friedreich's ataxia

An epileptic attack that commences with muscular contractions of the distal part of a limb and spreads to involve the whole body is called? A. Psychomotor epilepsy B. Petit mal C. Grand mal D. Idiopathic epilepsy E. Jacksonian seizure

E. Jacksonian seizure

Which of the following is characterized by rapidly progressive dementia, spongiform encephalopathy, and diffuse atrophy of the brain? A. Herpes simplex encephalitis B. Rabies C. Progressive multifocal leukoencephalopathy D. Toxoplasmosis E. Jakob-Cruetzfeldt disease

E. Jakob-Cruetzfeldt disease

Solid tumor of the cerebellum. Occurs primarily in children and young adults. Aggressive growth, but very radiosensitive. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

E. Medulloblastoma

Which of the following is the disease of the CNS that is characterized by focal patches of demyelination with formation of plaques and is usually a chronic disease leading eventually to complete disability? A. Poliomyelitis B. Guillain-Barre syndrome C. Post-infectious encephalomyelitis D. Neurosyphilis E. Multiple sclerosis

E. Multiple sclerosis

A patient is seen to develop ptosis as the day progresses. The patient complains also of developing diplopia by the time it gets to the evening and can no longer watch television. An injection of the short-acting anti-cholinesterase known as edrophonium corrects these symptoms for a couple of minutes. Which of the following is it most likely to be? A. Guillain-Barre syndrome B. Amyotrophic lateral sclerosis C. Neurosyphilis D. Bell's Palsy E. Myasthenia gravis

E. Myasthenia gravis

Which of the following is lysosomal storage disease that is inherited as an autosomal recessive mode of inheritance and causes accumulation of sphingomyelin in the neurons of the brain? A. Tay-Sach's disease B. Wilson's disease C. Adrenoleukodystrophy D. Phenylketonuria E. Niemann-Pick's disease

E. Niemann-Pick's disease

Calcifications are commonly seen in which of the following tumors? A. Astrocytoma B. Medulloblastoma C. Neurofibroma D. Ependymoma E. Oligodendroglioma

E. Oligodendroglioma

The disease/condition that is inherited with an autosomal dominant mode of inheritance and that manifests with cafe-au-lait spots on the skin together with a progressive development of peripheral neurofibromas is known as: A. Type I neurofibromatosis B. Type II neurofibromatosis C. Von Recklinghausen's disease D. AOTA E. Only A and C

E. Only A and C

Wernicke's Korsakoff's syndrome, which usually includes amnesia, confabulation, and general mental deterioration, is typically associated with which of the following? A. Alcoholism B. Pernicious anemia C. Thiamine deficiency D. AOTA E. Only A and C

E. Only A and C

This infection of the CNS is characterized by the presence of Negri bodies within brain neurons and hyper excitability of the CNS. A. Varicella/Zoster encephalitis B. Subacute sclerosing parencephalitis C. Jakob-Creutzfeldt disease D. St. Louis encephalitis E. Rabies

E. Rabies

Which of the following is the most common type of neoplasm found within the CNS? A. Astrocytoma B. Meningioma C. Ependymoma D. Oligodendroglioma E. Secondary metastasis

E. Secondary metastasis

The spinal dysraphism usually results in a paraplegic or fatal infection. What is it? A. Spastic diplegia B. Multiple sclerosis C. Guillain-Barre syndrome D. Syringomyelia E. Spina bifida

E. Spina bifida

Which of the following conditions is described as progressively enlarging cyst within the spinal cord? It eventually presents clinically as a dissociated anesthesia with a cape-like distribution. A. Guillain-Barre syndrome B. Marie-Charcot syndrome C. Spastic diplegia D. Amyotrophic lateral sclerosis E. Syringomyelia

E. Syringomyelia

All of the following are associated with multiple sclerosis EXCEPT: A. Increased incidence in association with certain HLA haplotypes B. Irregularly scattered focal areas of demyelination in the brain and spinal cord C. Optic nerve, brain stem, and paraventricular areas are favored sites D. Progressive highly variable course, characterized by exacerbations with long asymptomatic remissions E. Widespread demyelination occurs closely following a viral illness or immunization

E. Widespread demyelination occurs closely following a viral illness or immunization

This neoplasm arises from embryonic remnants of the foregut. Located superior to sella turcica, and may compress the hypothalamus, optic chiasm, and 3rd ventricles. Tendency to develop calcification around cholesterol crystals. A. Oligodendroglioma B. Meningioma C. Ependymoma D. Glioblastoma multiforme E. Medulloblastoma F. Astrocytoma G. Craniopharyngioma

G. Craniopharyngioma

This is an inherited disease that manifests after the age of 30-40 and leads to death within 10 years of onset. A. Alzheimer's B. Amyotrophic lateral sclerosis C. Huntington's Chorea D. Parkinson's disease E. Friedreich's ataxia

Huntington's Chorea