Rotation- Neurology (Pretest, Case Files)

Front 1

An increased T2 signal in periventricular distribution is typical of the plaques seen in what demylinating disease?

Back 1

Multiple Sclerosis.

Front 2

Intention tremors or kinetic tremors are most characteristic of damage to what part of the brain?

Back 2

The Cerebellum.

Front 3

Damage to the thalamus is more likely to produce a sensory disturbance or a motor disturbance?

Back 3

Sensory disturbance.

Front 4

What type of motor symptoms do internal capsule lesions usually produce?

Back 4

Weakness.

Front 5

What are the therapeutic levels of Phenytoin (Dilantin) and at what supratherapeutic range does it produce nystagmus?

Back 5

10-20 mg/dL are therapeutic and 20-30 mg/dL produce nystagmus?

Front 6

Damage to what brainstem structure may produce characteristic conjugate deviations of the eyes such that conjugate eye movements are rhythmic and directed downward, but they lack the rapid component characteristic of nystagmus? *such abnormal movement is called eye bobbing.

Back 6

The Pons.

Front 7

A lesion at the cervicomedullary junction, such as a meningioma at the foramen magnum, will produce what type of nystagmus?

Back 7

Down-beating nystagmus with both eyes rhythmically deviating downward, with the rapid component of this nystagmus directed downward as well.

Front 8

The facial nerve innervates this muscle of the inner ear. With paralysis of this muscle, undamped transmission of acoustic signals across the stapedius bone of the middle ear produces hyperacusis. Cranial Nerve VII is responsible for its innervation. Muscle?

Back 8

Stapedius muscle.

Front 9

What is the next step in the management of a patient if a subarachnoid hemorrhage is strongly suspected, but not blood is seen on CT?

Back 9

Lumbar puncture is the next best step, as an MRI would not be much more helpful if the CT showed no blood; an angiogram is too invasive to use without first analyzing CSF with an LP.

Front 10

Alpha wave is the most prominent brain wave finding in adults. The relaxed adult exhibits this type of wave, which disappears with eye opening and with concentration on mathematical activities. What is the frequency at which Alpha wave activity runs?

Back 10

8 to 13 Hz over the posterior aspects of the head.

Front 11

These types of deficits are particularly likely in hypertensive persons with small infarctions called lacunae. This is the most common type of lacunar stroke?

Back 11

Pure motor stroke; the affected person usually has hemiplegia unassociated with cognitive, sensory, or visual deficits. The posterior limb of the internal capsule is the usual site of injury.

Front 12

Pure sensory strokes are most likely in the same persons who are susceptible to pure motor strokes and other lacunae. What is the most common structure affected in this pure sensory stroke?

Back 12

Thalamus.

Front 13

The patient with this syndrome has ischemia that damages the nucleus and descending tracts of the 5th nerve, the nucleus ambiguus, lateral spinothalamic tracts, inferior cerebellar peduncle, descending sympathetic fibers, vagus, and glossopharyngeal nerves. The patient with this syndrome has ipsilateral ataxia and ipsilateral Horner's syndrome. The trigeminal tract damage may produce ipsilateral loss of facial pain and temperature perception and ipsilateral impairment of the corneal reflex. The lateral spinothalamic damage produces pain and temperature disturbances contralateral to the injury in the limbs and trunk. Dysphagia and dysphonia often develop with damage to the 9th and 10th nerves. Diagnosis of this syndrome?

Back 13

Wallenberg syndrome (Lateral medullary syndrome).

Front 14

The Nucleus Ambiguus located in the ventrolateral medulla, contains the motor neurons that contribute to which two cranial nerves?

Back 14

9th (Glossophayngeal) and 10th (Vagus).

Front 15

The motor neurons of this nucleus and these cranial nerves innervate the striated muscles of the pharynx and larynx as well as provide the preganglionic parasympathetic supply to thoracic organs, including the esophagus, heart, and lungs. Injury to this nucleus and its pathways causes hoarseness and dysphagia. What is the nucleus and the cranial nerves that are involved?

Back 15

Nucleus Ambiguus; 9th and 10th nerve.

Front 16

Most cases of Wallenberg syndrome (Lateral Medullary infarction) are cause by occlusion of what artery?

Back 16

Vertebral artery; several small branches of the distal vertebral artery supply the lateral medulla. In some cases, occlusion of the posterior inferior cerebellar artery (PICA) causes this syndrome.

Front 17

What is the most common cause of cerebral lobar hemorrhage in ELDERLY patients WITHOUT a history of hypertension?

Back 17

Cerebral Amyloid Angiopathy (CAA)!!!, or congophilic angiopathy.

Front 18

Why is lamotrigine not an anticonvulsant drug of choice when a drug needs to be started immediately to control seizure activity?

Back 18

Lamotrigine must be slowly titrated over many week when first started because of the risk of severe rash.

Front 19

Is Sturge-Weber characterized by leptomeningeal agiomas or AV malformations in the brain?

Back 19

Leptomeningeal angiomas, which may LEAD to Av malformations.

Front 20

An aneurysm of which artery of the circle of willis is most likely to compress the Oculomotor nerve (CN III) and symptoms?

Back 20

Posterior Communicating Artery!!!

Front 21

What is the peak time period for alcohol withdrawal seizures versus peak time period for DTs from alcohol withdrawal?

Back 21

The first day of abstinence from alcohol is the peak time period for alcoholic withdrawal seizures, in contrast to DTs which has a peak time period of 2 to 4 days post abstinence.

Front 22

This syndrome is characterized by mental dysfunction, multiple seizure types, and 1.5- to 2-Hz generalized spike-wave discharges on EEG. It is often difficult to control the seizures that develop in children with this syndrome. Many affected children have a history of infantile spasms (West syndrome). Diagnosis?

Back 22

Lennox-Gastaut syndrome.

Front 23

Children with this condition exhibit paroxysmal flexions of the body, waist, or neck and usually have a profoundly disorganized EEG pattern called hypsarrhythmia. Diagnosis?

Back 23

Infantile spasm (West syndrome).

Front 24

This childhood syndrome is associated with loss of language function and an abnormal EEG during sleep. Diagnosis?

Back 24

Landau-Kleffner syndrome.

Front 25

The most common complication of temporal lobectomy is a visual field defect caused by interruption of fibers from the optic tracts passing over the temporal horn of the lateral ventricles. What is the most common visual field defect that is caused?

Back 25

Superior quadrantanopsia; it is more common than hemianopsia.

Front 26

Why is Levetiracetum a good antisezure medication to use?

Back 26

It has relatively good efficacy an relatively lower adverse effect profile.

Front 27

What is the medication of choice to treat seizures associated with Tuberous Sclerosis disease?

Back 27

ACTH (adrenocorticotropic hormone)!!!!

Front 28

Ethosuximide is the drug of choice for treating absence seizures, but it can cause GI distress. What next-line drug is effective in many children who cannot tolerate ethosuximide?

Back 28

Divalproex sodium.

Front 29

If a child has absence seizures that are associated with generalized tonic-clonic seizures what is the drug of choice instead of ethosuximide in this particular setting?

Back 29

Divalproex sodium.

Front 30

Recent studies have established that what drug is the treatment of choice to prevent seizures in women with hypertension at the time of admission for delivery (preeclampsia) and established preeclampsia?

Back 30

MgSO4!!!!; it was shown to result in reduction in recurrent seizures and in maternal morbidity and mortality.

Front 31

This autoimmune disease is often associated with Trigeminal Neuralgia (symptomatic trigeminal neuralgia). Diagnosis?

Back 31

Multiple Sclerosis; other causes of symptomatic trigeminal neuralgia include basilar artery aneurysms, acoustic schwannomas, and posterior fossa meningiomas, all of which may cause injury to the fifth cranial nerve by compression.

Front 32

Unlike patients with trigeminal neuralgia, patients with this facial pain usually feel a constant deep pain. Although this pain is often bilateral, it may be unilateral and fairly limited in its distribution. The cheek, nose, or zygomatic regions are often affected by this idiopathic pain syndrome. The pain is often sensitive to antidepressant medication. Diagnosis?

Back 32

Atypical facial pain syndrome.

Front 33

What is the best initial test and also the most sensitive test to use in the assessment of a suspected subacute, subarachnoid bleed?

Back 33

MRI; initially subdural blood will be denser than brain and thus readily apparent on CT scan. Within a few days of formation, the contents of a subdural hematoma are degraded into a less dense fluid. This fluis is transiently similar in density to the cerebral cortex and may be difficult to distinguish by CT. Eventually it will be hypodense to brain. If the fluid collection is too small to produce substantial deformation of the underlying hemisphere, identification of the subdural collection may be difficult. The least invasive and best assessment tool is MRI

Front 34

At the onset of von Economo's Encephalitis (encephalitis lethargica), patients often develop transient fevers, lethargy, and headache. Disturbed eye movements are the most common sign of of neurologic disease during the acute illness. A variety of movement disorders, including chorea, athetosis, dystonia, and myoclonus, develop with the disease. About 1 in 4 affected persons dies. What is the most common, and most severe, sequelae of this disease? *think of Awakenings*

Back 34

Unremitting Parkinsonism with signs and symptoms similar to those exhibited with idiopathic parkinsonism. One rather unique feature is the occurrence of oculogyric crises, or episodes in which the eyes deviate to one side or upward, associated with other forms of dystonia and autonomic symptoms, sometimes occurring with great regularity.

Front 35

What is the Cranial neurological injury most likely to be associated with sarcoid?

Back 35

Facial paresis (CN VII); almost half the patients with sarcoidosis and neurologic disease have a neurologic sign or symptom as the first obvious complication of the sarcoidosis. These patients report progressive weakness of one side of the face with no substantial loss of sensation over that side of the face. The may feel there is decreased sensitivity to touch on the weak side, but this is more commonly from a loss of tone in the facial muscles than from an injury to the trigeminal nerve.

Front 36

This parasitic infection is endemic to the tropics and may produce a subacutely evolving paraparesis. The fluke itself does not invade the spinal cord, but it does deposit eggs in the valveless veins of Batson, which drain the intestines and communicate with the drainage of the lumbosacral cord. The patient develops granulomas around the ova that lodge in the spinal cord, and these granulomatous lesions crush the cord. Parasite diagnosis?

Back 36

S.mansoni.

Front 37

Does Echinococcus lead to the development of hydatid cysts or cystecercosis?

Back 37

Echino is like a Rhino because it leads to one calcified egg shell appear lesion on MRI, just like there is ONE rhino horn.

Front 38

The CSF examination of a patient with a spongiform encephalopathy, or Creutzfeldt-Jakob disease typically shows what findings?

Back 38

NORMAL CSF!!!; on occasion the protein level may be mildly elevated, and in up to 20% of cases there may be an increase in the ratio of immunoglobulin G to total protein, occasionally with oligoclonal bands. In many, but not all patients a somewhat specific protein (14-3-3 proteinase inhibitor) may be present.

Front 39

If a brain abscess or tumor is suspected in a patient, is a CT with or without contrast, the more appropriate first imaging of choice?

Back 39

WITH CONTRAST!!!; non-contrast is less sensitive.

Front 40

Both HIV and CMV infections of the brain characteristically produce what specific finding on histological analysis?

Back 40

Microglial nodules; the microglial nodules occurring with HIV are associated with syncytial cells in the brain and spinal cord, a cell type not typically seen with CMV encephalitis. CMV is a common opportunistic infection in patients with AIDS; with HIV infection the microglial nodules are distributed around blood vessels throughout the brain. With CMV, the nodules are more characteristically subpial and subependymal.

Front 41

What type of EEG findings over the Temporal Lobes bilaterally are associated with Herpes encephalitis?

Back 41

Periodic epileptiform discharges,

Front 42

Fungal malignant external otitis and osteomyelitis of the base of the skull in HIV patients is most commonly caused by what fungus?

Back 42

Aspergillus.

Front 43

General paresis is a slowly evolving process that may require years to produce substantial disability. The evolving symptoms are a subtle dementia, characterized by memory loss and impaired reasoning, with later development of dysarthria, myoclonus, tremor, seizures, and UMN signs, leading to a bedridden state. What is the chronic infection that causes this condition and what is the treatment?

Back 43

Syphilis; this is a form of neurosyphilis and it is treated with Penicillin.

Front 44

This infection commonly develops in renal transplant recipients, patients with chronic renal disease, immunosuppressed persons and occasionally in otherwise unimpaired persons. It may also affect neonates. This type of meningitis is not usually seen in older children. It may on occasion lead to intracerebral abscess formation. 3rd-generation cephalosporins are inactive against this infection, and neither ampicillin or penicillin alone are effective. Ampicillin and gentamicin are recommended therapy. Diagnosis?

Back 44

Listeria.

Front 45

As of 1999, 113 cases of this disease due to growth hormone preparations had been reported, with incubation periods ranging from 5 to 30 years. Diagnosis?

Back 45

Creutzfeldt-Jakob disease.

Front 46

AIDS patient with inclusion bodies found in oligodendrocyte nuclei on brain biopsy. Immunosuppression leads to reactivation of the primary CNS infection with this virus that is universal throughout the population. Virus? Condition?

Back 46

JC papova virus; PML.

Front 47

What is the most common type of primary brain tumor?

Back 47

Malignant Astrocytoma; these are graded as 3 or 4. Grade 4 astrocytoma is more commonly called Glioblastoma multiforme. It is malignant in the very conventional sense that it invades adjacent tissue. This type of glial tumor is usually seen in adults; men are more susceptible than women.

Front 48

The incidence of metastases from this primary source accounts for 2/3 of cases of brain metastases presenting without a known primary. Diagnosis?

Back 48

Lung Cancer.

Front 49

The outlook with malignant melanoma, breast cancer, lung cancer, or renal cancer metastatic to the brain is poor and is limited to a matter of months, but malignant melanoma has an especially grim prognosis. Why?

Back 49

This is because it has a high likelihood of bleeding after it metastasizes to brain. Malignant melanoma and choriocarcinoma are likely to produce lethal intracranial hemorrhages, and the former may in fact first become apparent only after it has precipitated an intracranial hemorrhage.

Front 50

Does Prostate cancer typically metastasize to brain?

Back 50

NO!!!

Front 51

This is a nonneoplastic malformation in the brain that may be discovered incidentally, either on imaging performed for other reasons, or at autopsy, or the symptoms may be referable to this region of the brain. Most often the latter involves neuroendocrine function disruption, causing precocious puberty or acromegaly caused by overproduction of growth-hormone releasing hormone. Patients may also experience paroxysms of laugher, known as Gelastic Seizures. Patients may be cured surgically. Diagnosis of brain region affected???

Back 51

HYPOTHALAMUS!!!

Front 52

These are epithelial neoplasms arising in the sellar and third ventricular regions. They may cause HYPO-pituitarism and visual disturbances. Diagnosis?

Back 52

Craniopharyngiomas.

Front 53

This brain lesion is historically benign causes the patient symptoms that constitue Parinaud's syndrome, which may include loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence-reaction nystagmus, in which the eyes appear to jerk back in the orbit on attempted upgaze. Brain region affected?

Back 53

Pineal gland lesion; this syndrome occurs in lesions owing to involvement of the dorsal midbrain in the region of the superior colliculus. Other tumors appearing in the pineal region that can produce a similar clinical picture include germ cell tumors, teratomas, and gliomas.

Front 54

Long-standing hepatic disease may produce a profound encephalopathy. The most obvious change is an increase in what type of neuron?

Back 54

Astrocytes (Alzheimer type II astrocytes); these astrocytes are relatively large cells. Rarely patients show more dramatic changes, which include neuronal loss and focal necrosis.

Front 55

With chronic alcoholism and hepatic insufficiency, patients exhibit a loss of what specific types of neurons?

Back 55

Purkinje cells in the cerebellum; this is a consequence of alcohol toxicity or thiamine deficiency.

Front 56

What CSF change may be seen during a hypertensive encephalopathic crises?

Back 56

Elevated protein, but <100mg/dL; the elevation with hypertensive encephalopathy is variable because intracranial hemorrhage may occur with the hypertensive crisis, but most patients will have moderate increases in CSF protein. Lowering of the blood pressure may reduce the CSF protein.

Front 57

What is the most common neurological complication of Renal failure?

Back 57

Peripheral Neuropathy; the type of peripheral neuropathy most commonly developing with chronic renal failure is a symmetric, distal, mixed sensorimotor neuropathy. The legs are generally affected first and most severely. Men are more commonly affected than women. Most of the peripheral neuropathies in patients with chronic renal failure involve AXONAL degeneration. The neuropathy usually improves with dialysis.

Front 58

Restless leg syndrome occurs primarily at night, shortly after the patient lies down. It differs from akathisia, which is a restlessness that occurs during the daytime. What medical conditions is restless leg often associated with?

Back 58

Anemia and peripheral neuropathy and is seen in patients with chronic renal disease, diabetes mellitus, and many other medical conditions.

Front 59

What non-medical intervention can help ease the symptoms associated with restless leg syndrome?

Back 59

Exercising before going to bed may help alleviate much of the discomfort.

Front 60

What is the eye condition that arises in patients who chronically smoke tobacco and drink ethanol?

Back 60

Deficiency amblyopia (aka nutritional optic neuropathy, tobacco-alcohol amblyopia)

Front 61

The most prominent characteristic of Alzheimer's disease histologically, are neuronal loss, fibrillary tangles, loss of synapses, and amyloid (or neuritic) plaque formation. These histopathological features are evident throughout the cerebral cortex, but the neurofibrillary tangles and neuronal loss are most prominent in what part of the brain?

Back 61

Hippocampus; the cell loss may be so substantial that the patient develops marked compensatory enlargement of the ventricles, a condition called hydrocephalus ex vacuo.

Front 62

What is the most common cause of dementia in the general population?

Back 62

Alzheimer disease.

Front 63

Up to 28% of patients who undergo ventriculoperitoneal shunting for NPH may suffer major complications. What complication in particular should you be on the lookout for?

Back 63

Subdural hematoma; this occurs because the reduction in intracranial pressure brought on by the reduction in CSF volume may cause the brain to pull away from the covering meninges, stretching and potentially rupturing the bridging veins.

Front 64

The major clinical features of Alzheimer disease are memory impairment, aphasia, apraxia, and neuropsychiatric impairment, including mood disturbances, delusions and hallucinations, personality changes, and behavior disturbances. What is the main language disturbance associated with Alzheimer disease?

Back 64

Decreased fluency, dysnomia, and transcortical sensory aphasia, which referes to the ability to understand complex linguistic structures. Repetition of verbal material is intact.

Front 65

The neurological symptoms occurring early in the course of Creutzfeld-Jakob disease are often cerebellar or visual. Patients may have ataxia, clumsiness, or dysarthria, as well as diploplia, distorted vision, blurred vision, field deficits, changes in color perception and visual agnosia. Ultimately cortical blindness may occur. The diagnosis may be supported by the finding of periodic sharp waves at 1-2-Hz frequency on EEG and what elevated protein marker in the CSF?

Back 65

14-3-3 protein.

Front 66

Treatment with antibiotics in this disease may be curative and for this reason, this diagnosis is important to remember in unusual cases of DEMENTIA WITH MOVEMENT DISORDERS. This is a rare multisystem disease with GI complaints such as steatorrhea, abdominal pain, and weight loss, which reflect bowel infection. Central nervous system infection, which may occur in the absence of GI disease may produce seizures, myoclonus, ataxia, supranuclear gaze disturbances, hypothalamic dysfunction, and dementia. Oculomasticatory myorhythmia (pendular convergence eye movements of the eyes in association with contraction of the masticatory muscles) may occur and is considered pathognomonic. At times other muscles of the body may be involved. Diagnosis can be made by biopsy of the jejunum, though sometimes brain biopsy may be required and may show PAS-positive cells. Diagnosis?

Back 66

Whipple disease; Tropheryma whippelii.

Front 67

In Huntington's disease, as the caudate atrophies the shape of what of what brain structure?

Back 67

Lateral ventricle; as the caudate atrophies the frontal tip of the lateral ventricle becomes increasingly rhomboidal in shape. The head of the caudate is usually atrophic early in the course of Huntington disease and this will usually be evident by the time the patient is symptomatic, if not sooner. On MRI or CT scanning, the head of the caudate gives the frontal and parietal components of the lateral ventricle its typical comma, or boomerang appearance.

Front 68

The current theory of Parkinson disease pathology is based on the premise that the substantia nigra pars compacta has decreased dopamine production, which eventually leads to overinhibition of thalamocortical pathways. High frequency stimulation of what three brain structures is most likely to improve symptoms?

Back 68

Thalamus, Globus pallidus, Subthalamic nucelus; The Thalamus may be directly intervened on to decrease this overinhibition. Alternatively the Globus pallidus interna may be lesioned or stimulated, because it directly inhibits the thalamus. A third approach is to lesion or stimulate the subthalamic nucleus, which has an excitatory connection on the globus pallidus interna and substantia nigra pars reticulata.

Front 69

In a psychiatric patient who has developed parkinsonism secondary to long-term neuroleptic use, what is the best course of action to treat the parkinsonism symptoms?

Back 69

Use of an Anticholinergic medication such as Trihexyphenidyl (Artane); it is presumed to decrease signs of parkinsonism cause by drugs that interfere with with dopamine neurotransmission by creating a relative deficiency of acetylcholine neurotransmission. In a very simplistic view of the CNS, the cholinergic and dopaminergic systems have antagonistic actions. The offending psychiatric medication should also be slowly tapered off. Also, although useful in Parkinson disease, administering L-dopa IS NOT ADVISED in this case as it may worsen the psychiatric condition and would probably not help the parkinsonism.

Front 70

What medications are typical to use for patients with Tourette's syndrome?

Back 70

Typical Antipsychotics.

Front 71

This is a form of focal dystonia characterized by blepharospasm, forceful jaw opening, lip retraction, neck contractions, and tongue thrusting. Sometimes these features are produced by phenothiazine or butyrophenone use, but they may also occur idiopathically, more often in men than women, with onset in the sixth decade. Botulinum toxin injection has been more effective in treatment than any oral medication. Diagnosis?

Back 71

Meige syndrome

Front 72

This condition develops in the elderly and may be overlooked or misdiagnosed. The elevated calcium (>11.5 mg/dL) that is characteristic of this disturbance is dismissed as an immobilization phenomenon or misconstrued as evidence of an occult neoplasm. The appearance of PSEUDOGOUT should raise the suspicion of this diagnosis substantially. The calcium level may in fact be normal when it is checked. Patients can exhibit excessive sleepiness, slowing of movements, mild depression, and proximal muscle weakness. Proximal limb muscles may be obviously atrophied. There may be an elevated serum creatinine and decreased creatinine clearance. Chronic feeling of abdominal discomfort may be present. Diagnosis?

Back 72

HYPERPARATHYROIDISM!!!

Front 73

One in how many persons, carries the diagnosis of MS in the U.S.?

Back 73

1 in 1000.

Front 74

What evoked response pattern is most often abnormal in patients with early MS?

Back 74

Visual Evoked Response (VER); optic neuritis occurs early and often in many patients with MS. This involves inflammation and demyelination of the optic nerve and slows conduction along the optic nerve. Components of the VER may be slowed or even absent

Front 75

Why might gastric disturbances be expected in a patient placed in the hospital for an acute flare-up of Multiple Sclerosis? What should you prescribe to prevent these disturbances?

Back 75

Gastric disturbances are a possible side effect of corticosteroid use. Ranititdine is an appropriate prophylactic treatment.

Front 76

This disease may produce developmental regression at about 6 months of age. The infant develops extensor posturing and rigidity. Myoclonic seizures may develop. Underlying this disease is a defect in N-acetylaspartic acid metabolism. Elevated levels of this compound can be detected in blood and urine, but elevated levels in the brain establish the diagnosis. Changes in brain white matter are widespread and may result in a spongiform appearance. There is increased brain volume and weight, leading to macrocephaly. Diagnosis?

Back 76

Canavan disease.

Front 77

Patients with Multiple Sclerosis often develop what type of bladder complications?

Back 77

Spastic bladder (upper motor neuron); there is little or no residual urine in the bladder after emptying because bladder contractility is good, but distensibility is greatly reduced from the spasticity. The bladder does not distend substantially because of corticospinal tract disease, which produces spasticity. The patient usually has urgency or incontinence.

Front 78

What environmental condition commonly exacerbates the symptoms of Multiple sclerosis?

Back 78

Increased, or Hot temperatures; patients with demyelinating diseases often notice exacerbation of symptoms with increased temps. In fact, one old method of diagnosing MS called for the patient to be submerged in a tub of warm water; if the deficits worsened this was considered a sign of MS. This heat sensitivity, also called Uhthoff's phenomenon, explains why patients often feel worse in the summer or on taking hot showers. MS should be considered when seeing a patient who complains of exercise-induced symptoms, because the increased heat that is generated by exercise may be enough to exacerbate the deficits.

Front 79

This disease is a demyelinating disorder that belongs to a group of degenerative diseases known as sudanophilic leukodystrophies. Leukodystrophy referes to the disturbance of white matter, and sudanophilic referes to the Sudan-staining characteristics of te involved white matter. Children with this disease typically become symptomatic during the first months of life, but survival may extend into the third decade of life. Most affected persons are male. Diagnosis?

Back 79

Pelizaeus-Merzbacher disease.

Front 80

Cystic lesions found in the CNS of a patient with von-Hippel-Lindau disease are most likely hemangioblastomas. These hemangioblastomas often bleed and produce potentially lethal intracranial hematomas. Radiation therapy is not the best choice for treatment. What is?

Back 80

Surgical resection of resectable lesions as soon as possible; although radiation therapy is not the best choice, stereotactic radiosurgery may be an option.

Front 81

What cervical vertebral malformation is often associated with a Chiari malformation of the hindbrain?

Back 81

C1 fusion to the base of the skull.

Front 82

About what percent of cases do women that are Fragile-X carries have mild mental retardation?

Back 82

50% of cases.

Front 83

In Heterochromatic Leukodystrophy there is a deficiency of arylsulfatase A activity and an accumulation of Sulfatide. On examination patients may be spastic, yet nerve conduction studies reveal slowed moteo and sensory conduction velocities. CSF protein may be elevated and MRI will reveal white matter abnormalities. Leukocyte testing will reveal deficient arysulfatase A activity. What other test will provide useful diagnostic information in this condition?

Back 83

Nerve biopsy, as Sulfatide may be evident in Schwann cells as prismatic and tuffstone inclusions, as well as in tissue outside the nervous system. At autopsy there may be evidence of dysmyelination or demyelination in the CNS as well as the peripheral nervous system.

Front 84

With Hartnup disease there is intestinal malabsorption of tryptophan and other neutral amino acids. Tryptophan serves as a precursor for this molecule that patients with Hartnup disease may respond to in large supplementary doses. Supplement?

Back 84

Nicotinamide; with more than 400mg of nicotinamide daily, the tryptophan malabsorption becomes less problematic. Affected children develop a scaly erythematous rash on the face similar to that seen with pellegra. The disease is usually symptomatic during infancy.

Front 85

In what hereditary disease do do intracranial calcifications follow the gyral pattern of the cerebral cortex and subsequently produce the railroad track pattern that is evident on plain X-ray of the skull?

Back 85

Sturge-Weber; calcium is deposited in the brain of the patient with Sturge-Weber syndrome, presumably because the abnormal vessels overlying the brain allow calcium, as well as iron, across the defective blood-brain barrier.

Front 86

Signs of erythrocytosis with cerebellar signs, microscopic hematuria, and hepato-splenomegaly suggests what hereditary syndrome?

Back 86

von-Hippel-Lindau syndrome; this hereditary disorder is characterized by polycystic liver disease, polycystic kidney disease, retinal hemangiomas (telangectasias), and cerebellar tumors. With this syndrome adenomas can occur in many organs, and hemangiobalstomas may develop in the spinal cord or brainstem, as well as in the cerebellum.

Front 87

This hereditary syndrome usually becomes symptomatic in early childhood. The peripheral blood smear will exhibit abnormally shaped erythrocytes (acanthocytes), and the plasma lipid profile will reveal very low cholesterol and triglyceride content. Acanthocytes are spiked or crenated RBCs (in this syndrome due partly to vitamin E deficiency). These are very unusual findings in a patient with ataxia, which the patient likely has, and thus are diagnostic of this condition. Diagnosis?

Back 87

Abetalipoproteinemia.

Front 88

Von Hippel-Lindau syndrome is associated with a high incidence of renal carcinomas. These malignant tumors develop years after the cerebellar hemangioblastomas, liver disease, or polycystic renal disease becomes symptomatic. People surviving intracranial hemorrhage often succumb to what end due to this disease?

Back 88

Metastatic Renal Cell carcinoma!!!

Front 89

Along with adenoma sebaceum, and periventricular tubers, this ocular lesion is virtually pathognomonic for Tuberous Sclerosis. Lesion?

Back 89

Retinal phakoma; these are gliomatous tumors that require no treatment and are principal criterion for making the diagnosis of tuberous sclerosis.

Front 90

Telengectasias in the retina. What hereditary syndrome?

Back 90

Von Hippel-Lindau.

Front 91

By 5 years of ag, more than half of patients with Tuberous Sclerosis will have intracranial nodules that have calcified. These nodules usually do not become malignant, but they may have enlarged sufficiently to produce an obstructive hydrocephalus. Ventriculoperitoneal shunting may be needed if obstruction develops. Where are these intracranial nodules located?

Back 91

They are subependymal glial nodules.

Front 92

What hormone is usually given as a gel intramuscularly to control infantile spasms in children with tuberous sclerosis?

Back 92

ACTH!!!; 40 to 80 mg is divided into two doses. Treatment continues until the infantile spasms abate or the EEG pattern of hypsarrhythmia resolves. This usually requires 6 to 8 weeks of treatment. The ACTH should not be stopped abruptly.

Front 93

A type 1 Chiari malformation becomes symptomatic in what way in adults?

Back 93

Ataxia

Front 94

A type 2 Chiari malformation becomes symptomatic in what way, at what age?

Back 94

Type 2 malformations most often become symptomatic at birth or during infancy and may produce hydrocephalus with retardation.

Front 95

What percentage of patients with Tuberous Sclerosis show mental retardation?

Back 95

65%, and of these 50% are severely mentally retarded.

Front 96

What percent of patients with Tuberous Sclerosis develop Lennox-Gastaut syndrome?

Back 96

20% develop this syndrome with persistent complex partial seizures and significant mental retardation.

Front 97

What medication is useful in treating fatigue associated with Multiple Sclerosis?

Back 97

Amantidine.

Front 98

Are men, or are women, with dermatomyositis, more at risk for harboring an underlying malignancy?

Back 98

Men.

Front 99

A woman presents with proximal muscle weakness and pain and a heliotrope rash about her eyes. The term heliotrope refers to the lilac color of the periorbital rash that is characteristic of this disease. This rash surrounds both eyes and may extend onto the malar eminences, the eye lids, the bridge of the nose, and the forehead. It is usually associated with an erythematous rash across the knuckles and at the vase of the nails and may be associated with flat-topped purplish nodules over the elbows and knees. Diagnosis?

Back 99

Dermatomyositis.

Front 100

1 in how many male infants are affected by Duchenne dystrophy?

Back 100

1 in 3,000 male infants.

Front 101

Despite the drain from the population of males carrying the abnormal gene, the incidence of Duchenne dystrophy is stable. Males often die before they reach sexual maturity or are too impaired after adolescence to mate. There are no changes in the ovaries of women bearing a child with Duchenne dystrophy to suggest that the mutation is arising de novo in the ovary. Women with apparently normal dystrophin genes do, however, give birth to affected sons. Thus, how are the mutated dystrophin genes of Duchenne dystrophy thought to be maintained in the population?

Back 101

High spontaneous mutation rate!!!

Front 102

Do children with Duchenne dystrophy have mild cognitive impairment compared to their siblings who do not carry the gene?

Back 102

YES!!!

Front 103

Men with this condition characteristically exhibit problems with relaxing their grip, hypersomnolence, premature baldness, testicular atrophy, and cataracts. The EMG pattern displayed by these patients is often referred to as the diver bomber pattern because of the characteristic sound produced when the evoked action potential is heard. Diagnosis?

Back 103

Myotonic dystrophy.

Front 104

In ALS, early involvement of musculature supplied by which nerves has a much graver prognosis than early limb involvement?

Back 104

Cranial nerves!!! This may be a consequence of disturbed swallowing with recurrent aspiration as a result, or disturbed ventilatory activity. Fasciculations of the tongue develop with deterioration of hypoglossal nuclei.

Front 105

What part of the cerebellum is injured the most in chronic alcoholism?

Back 105

The Superior Vermis of the cerebellum loses Purkinje cells and exhibits atrophy of the molecular layer in alcoholic persons after years or decades of ethanol use.

Front 106

Persons who cured felt in the nineteenth century often developed pronounced personality changes, tremor, and ataxia. This type of poisoning is now more typically seen in paper, pulp, and electrochemical plants that use this metal as a part of compounds that are involved in their manufacturing process. Pathologic changes in the CNS are usually prominent in the cerebellum and include extensive damage to the granular cell layer of the cerebellum. The calcarine cortex of the occipital lobe is also especially vulnerable, and damage to this tissue correlates with constriction of visual fields. Diagnosis?

Back 106

Mercury poisoning.

Front 107

Miner with parkinsonism?

Back 107

Manganese poisoning.

Front 108

Excess of which fat soluble vitamin has the potential to produce pseudotumor cerebri?

Back 108

Vitamin A.

Front 109

What is the preferred non-surgical intervention for treating pseudotumor cerebri: Acetazolamide or repeated lumbar puncture?

Back 109

Repeated lumbar puncture; diuretics are sometimes used to manage patients who are not pregnant, but they are usually less effective than repeated lumbar punctures when this is practical.

Front 110

This is the most common form of retinal degeneration. It is a hereditary degenerative disease involving the retinal receptors and adjacent pigment cells. As this degeneration progresses, small accumulations of pigment appear about the periphery of the retina. Optic disc pallor is evident later in the disease. This disease develops along with Bassen-Kornzweig disease (abetalipoproteinemia), Refsum disease, and other metabolic disorders that produce extensive nervous system damage. Diagnosis?

Back 110

Retinitis pigmentosa.

Front 111

Children with Sturge-Weber syndrome have large port-wine spots on their faces, contralateral hemiparesis, retardation and seizures. Skull radiographs reveal intracranial calcifications that are associated with leptomeningeal angiomatosis. This syndrome results from a defect on Chromosome 3. What are the associated eye findings that can accompany this syndrome?

Back 111

Glaucoma.

Front 112

Papillitis is due to inflammation of the optic nerve head, whereas papilledema is due to increased ICP. Patients with papillitis also have pain on moving the globe and sensitivity to light pressure on the globe. About 1 in 10 patients have both eyes involved simultaneously. Papillitis is often an early sign of Multiple sclerosis. What is the visual difference between papillitis and papilledema?

Back 112

Visual LOSS; this is substantial with papillitis, and usually inconsequential with papilledema

Front 113

Marcus Gunn pupil, or afferent pupillary defect, develops as a result of damage to the optic nerve which reduces the light perceived in the affected eye. If the other eye has less or no optic atrophy, the consensual response of the pupil to light perceived by the better eye will constrict the pupil in the atrophic eye, even though direct light to the injured eye does not elicit a strong pupillary constriction. This results in paradoxical dilatation of the atrophic pupil when a flashlight is redirected from the normal eye to the injured eye. What autoimmune disease is the this physical finding commonly associated with?

Back 113

Multiple sclerosis!!!; developing as a complication of optic neuritis.

Front 114

The cones of the retina are packed into the macula, and the primary focus of the lens is the macula. Therefore injury to the the macula has what results, in terms of vison, for the patient?

Back 114

Significant loss of acuity, with preservation of peripheral vision; e.g. macular degeneration. The macula is usually evident on opthalmologic examination because it normally reflects a point of light that can be seen through the opthalmoscope located 3 to 4mm temporally from the optic disc.

Front 115

This type of vision loss occurs from retinal degeneration (e.g. retinitis pigmentosa), Vitamin A deficiency, and colorblindness.

Back 115

Nyctalopia; night blindness.

Front 116

Scintillating scotomas are the classic signature of what type of headache?

Back 116

The aura of migrane headache.

Front 117

The vessels apparent on fundoscopic examination of the retina are arterioles and venules. In a patients with this condition you will see segmental narrowing of arterioles, and the retina may exhibit arteriolar straightening and arteriolar-venular compression. The thickened arteriolar wall compresses the venule at the point where they cross, which is a pattern often referred to as nicking. What condition does this person have?

Back 117

HYPERTENSION!!!

Front 118

Arteriolar aneurysms seen on fundoscopic examination may appear as small red dots on the surface of the retina; they may appear as one of the first manifestations of this chronic disease. They are rarely larger than 90um across, and they may be more obvious in green light. A proliferative retinopathy may occur along with these microaneurysms in patients with this disease. Diagnosis?

Back 118

DIABETES MELLITUS!!!

Front 119

What are the three most common types of ocular nerve palsies in order of most common to least common?

Back 119

6th nerve (2x)> 3rd nerve

6th nerve (6x)> 4th nerve

VI > III > IV

Front 120

This syndrome and associated eye findings arise as a result of osteomyelitis of the petrous pyramid. Chronic ear infections may extend to the petrous pyramid and produce this syndrome from affecting these nerves if the ear infections are not properly managed. Diagnosis?

Back 120

Gradenigo syndrome; lateral gaze palsy and blurry vision with facial pain, due to abducens and trigeminal nerve inflammation. The abducens and trigeminal nerves are affected as the pass closely to the tip of the petrous bone.

Front 121

The superior oblique muscle extends far anterior in the orbit, and it is at high risk of injury with trauma to the orbit or the full face. What is this nerve that innervates the superior oblique?

Back 121

Trochlear (CN IV)!!!; injury to the fourth nerve with facial trauma will usually induce a slight head tilt to compensate for impaired intorsion of the affected eye.

Front 122

Varicella zoster, spreads to the face along the trigeminal nerve. What is the most common ocular motor nerve to also be involved with shingles of the face?

Back 122

The fourth nerve is most commonly involved because it shares its nerve sheath with the opthalmic division of the trigeminal nerve. The third and sixth nerves may also be involved with varicella zoster, but this occurs much less frequently than involvement of the fourth nerve.

Front 123

Oculomotor fibers that have been damaged reversibly may regenerate and connect to the wrong target. This aberrant regeneration is seen most often with lesions that chronically compress the third nerve. Aneurysms, cholesteatomas, and neoplasms could lead to this aberrant regeneration and should be suspected in patients demonstrating what physical findings that indicate this abberrant regeneration?

Back 123

Pupillary constriction upon engagement of CN III motor fibers; such as constriction with attempted adduction of the globe, not attributable to accommodation.

Front 124

In the MLF syndrome, the patient has incomplete adduction ipsilateral to the lesion in the MLF on conjugate lateral gaze. ON attempted conjugate lateral gaze away from the side of the lesion, the patient has nystagmus in the abducting eye. The fast component of the nystagmus is directed temporally. This is also referred to as internuclear opthalmoplegia. What type of injury would have to occur in the brainstem to injure the MLF?

Back 124

A mesencephalic or pontine injury.

Front 125

Rapid downward deviation of both eyes followed by slow upward conjugate eye movements describes the phenomenon of Ocular bobbing. This is an involuntary movement that usually develops with damage to what brainstem structure?

Back 125

Pons.

Front 126

Rhythmic jerking nystagmus elicited by having a patient look at a rotating drum with stripes on it is normal pattern of eye movement that should be elicitable with the normal patient. This is called optokinetic nystagmus. If the nystagmus is less obvious on rotating the drum in a given direction, the patient may have a lesion in what part of the brain, which is responsible for this finding?

Back 126

Parietal Lobe.

Front 127

This pupillary abnormality is usually seen in otherwise healthy young women and may occur in isolation or in association with absent tendon reflexes. It is probably due to degeneration of the ciliary ganglia. Although the cause is obscure, it may reflect a mild polyneuropathy. In most cases this as a benign phenomenon. Diagnosis?

Back 127

Benign tonic pupillary dilatation (Adie tonic pupil).

Front 128

These two immunosuppressive drugs may both induce a syndrome resembling hypertensive encephalopathy. It has been called by some, reversible posterior leukencephalopathy. although it involves more than white matter and may also occur in the anterior frontal regions. Patients may develop headache, visual dysfunction related to occipital lobe dysfunction, confusion, and seizures. Usually there is associated hypertension. the visual loss may include cortical blindness or scotomas. Imaging may show bilateral, more or less symmetrical signal changes in the white matter and occasionally the cortex of the occipital and parietal lobes. Drugs?

Back 128

Cyclosporin and Tacrolimus (FK 506).

Front 129

Which branch of the opthalmic artery supplies the optic nerve?

Back 129

The Posterior Ciliary Artery.

Front 130

Does optic neuritis associated with MS typically affect one eye or both eyes at the same time?

Back 130

One eye at a time; although both may eventually become involved.

Front 131

Cerebellar damage may be associated with severe vertigo caused by a stroke if the tissue damaged is in the distribution of what cerebellar artery?

Back 131

PICA; with occlusion of the medial branches of PICA the vestibular nuclei are infarcted and vertigo is common. Even with occlusion of the lateral branches, vertigo is likely.

Front 132

What tones are most susceptible to hearing loss during the initial phases of Meniere's disease?

Back 132

LOW TONES!!!

Front 133

This disease is characterized by repeated brief episodes of fullness in the ear, tinnitus, hearing loss, and severe vertigo. The episodes may last from hours to days. Attacks may be so severe that they cause the patient to fall to the ground due to severe disequilibrium. The cause is generally idiopathic, but is thought to relate to distension of the semicircular canal and an increase in the volume of the endolymphatic fluid. For this reason this condition has also been called endolymphatic hydrops. Treatment is generally with salt restriction and diuretics. Diagnosis?

Back 133

Meniere's disease.

Front 134

When CNS lesions involve UMN and there is a evolution of areflexia and flaccidity into hyperreflexia and spasticity, what is the usual time period in which this takes place?

Back 134

3 days to 3 weeks.

Front 135

This artery is a major anterior artery and may supply the lower two-thirds of the spinal cord. It is at risk of occlusion during abdominal aortic aneurysm repair. It enters at approximately T10-L1. Artery?

Back 135

The Artery of Adamkiewicz; other branches of the aorta or internal iliac arteries may also supply the thoracic and lumbar cord. The upper segments of the spinal cord are usually supplied off the vertebral arter.

Front 136

With this condition, inflammation is largely limited to the substance of the cord. This type of reaction may occur with a variety of noninfectious proceses, such as MS and sarcoid, or infectious processes, such as viral and parasitic infections. This is a broad category diagnosis. Diagnosis?

Back 136

Transverse myelitis.

Front 137

Which Schistosoma is endemic to South America and send embolic ova, characteristically to the spinal cord?

Back 137

Schistosoma mansoni.

Front 138

At what level of the CNS do the Corticospinal tracts decussate?

Back 138

At the junction of the Medulla and the spinal cord.

Front 139

What is the most common cause of mononeuritis multiplex?

Back 139

Diabetes mellitus.

Front 140

In this congenital ataxia disease, degeneration occurs primarily in the spinal cord rather than the cerebellum or brainstem. Both the dorsal and ventral spinocerebellar tracts are involved. The other spinal cord structures exhibiting degeneration include the posterior columns and the lateral corticospinal tracts. Diagnosis?

Back 140

Friedreich's Ataxia.

Front 141

More than 10% of patients with Friedreich disease develop what longterm chronic disease?

Back 141

Diabetes mellitus.; a more life threatening complication of this degenerative disease is the disturbance of the cardiac conduction system that often develops. Visual problems occur when the hyperglycemia of the diabetes mellitus is uncontrolled, but even patients without diabetes develop optic atrophy late in the course of the degenerative disease.

Front 142

The gene responsible for Friedreich ataxia resides on which chromosome?

Back 142

Chromosome 9.

Front 143

This is a test

Back 143

This is a test