Neurology - Inflammatory disorders of the nervous system, multiple sclerosis Flashcards
NEU - 3.1
Acute stage of viral meningitis is not associated with:
A) common negative clinical findings of cranial CT
B) negative findings on EEG exam
C) cloudy liquor, elevated cell and protein count
D) elevated lymphocyte count in the CSF
ANSWER
C) cloudy liquor, elevated cell and protein count
EXPLANATION
In acute viral meningitis, the inflammatory process affects only the soft meninges, the brain tissue is left intact. Therefore, cranial CT or EEG examinations detect no signs of damage to the brain tissue. Clouding of the liquor is not common, but is characteristic of purulent meningitis, also associated with highly elevated cell and protein count. In cases of viral meningitis, the liquor may be clear or colorless, with moderate elevation in cell and protein count.
NEU - 3.2
Acute stage of purulent meningitis is not associated with:
A) epileptic seizures
B) confusion
C) violent headaches
D) elevated lymphocyte and monocyte count in the CSF
ANSWER
D) elevated lymphocyte and monocyte count in the CSF
EXPLANATION
In purulent meningitis, the inflammatory process in the soft meninges is diffused and may, to varying degrees, spread to the cortical brain tissues. This may cause focal neurological symptoms or epileptic seizures to occur, and may even lead to cranial oedema which is accompanied by confusion. Violent, diffused headaches commonly accompany the massive, purulent inflammation.
NEU - 3.5
Characteristics of Guillain–Barre syndrome include:
A) segmental demyelinisation may be accompanied by axonal damage
B) axonal lesion does not occur
C) only sensory axonal lesion occurs
D) damage to myelin sheeth is always associated with axonal damage
ANSWER
A) segmental demyelinisation may be accompanied by axonal damage
EXPLANATION
In acute Guillain-Barre syndrome, the damage primarily affects the myelin sheath. Still, depending on the aggressiveness of the inflammation, or the prolonged period of convalescence, even the axon may be damaged. Disease damage affects both motor and sensory nerve fibers.
NEU - 3.6
Useful in prion disease treatment:
A) penicillin
B) AZT (azidothymidin)
C) macrolides
D) none of the above
ANSWER
D) none of the above
EXPLANATION
The infective agent known as prion (’protein only’) does not contain any nucleic acids, only proteins, which is the modified form of a common cellular protein (cellular prion precursor protein). Once the modified protein enters a normal cell, it induces further modification of the normal cell protein. Prion is resistant to formaline, heat, disinfectants, UV radiation, and ionizing radiation. There is no known cure against it.
NEU - 3.8
Initial stage of optic neuritis is characterized by central scotoma and:
A) papillary hyperaemia
B) papillary oedema
C) slight blur in vitreous humor
D) all of the above
E) none of the above
ANSWER
E) none of the above
EXPLANATION
Neuritis retrobulbaris is an inflammation in the optic nerve accompanied by segmental demyelinisation, which does not cause observable lesion in the papilla at an early stage of the disease. Temporal decoloration in the papilla may develop, which degeneration indicates subsequently detected optic nerve damage.
NEU - 3.10
Observed meningitis symptoms may include:
A) vomiting
B) avoiding light
C) signs of meningeal irritation
D) all of the above
E) none of the above
ANSWER
D) all of the above
EXPLANATION
Common symptoms of meningitis are fever, fatigability, irritability, and photo phobia. The neurological signs and symptoms, such as headache, vomiting, and other signs of meningeal excitement are partly due to the excited meninges. Purulent meningitis may be accompanied by forms of confusion, epileptic seizures, or focal neurological signs and symptoms due to diffused cortical involvement and oedema in the brain.
NEU - 3.11
Characteristic symptoms of encephalitis, except:
A) headache
B) decreased protein content in CSF
C) cranial nerve symptoms
D) changes in sleep rhythm
E) confusion, fever, headache, epileptic seizures
ANSWER
B) decreased protein content in CSF
EXPLANATION
Encephalitis is inflammation of the brain matter which may involve the grey or white matter, or both. Protein content of the brain fluid may increase due to damaged blood-liquor barrier, tissue damage, or immunoglobulin production. Total protein content may be within normal limits, but intrathecal immunoglobulin production indicates inflammation.
NEU - 3.13
Which examination is least informative in polyneuropathy?
A) neurography
B) examination of reflexes
C) muscle biopsy
D) nerve biopsy
ANSWER
C) muscle biopsy
EXPLANATION
Polyneuropathy is a disorder of the peripheral nerves. Motor, sensory and vegetative fibers of neurons may be simultaneously affected, but to varying degrees. Several etiological factors are evident. Course of disease, physical examination, and electroneurography (ENG) are primer criteria to confirm the diagnosis. Neural biopsy is primarily useful in differentiating genetically determined diseases. Thorough gastrointestinal and genetic examinations are often necessary. A muscle biopsy may be performed, since tissue sample findings may indicate neurogenic lesion, but carries hardly any further informative value. Therefore, a muscle biopsy is usually not necessary.
NEU - 3.14
Of the following diagnostic procedures, which is most commonly pathological in Multiple Sclerosis?
A) eye movement examination
B) cranial CT
C) EEG
D) myelography
ANSWER
A) eye movement examination
EXPLANATION
Eye movement disorder is a common characteristic symptom in Multiple Sclerosis. Internuclear ophthamoplegia occurs in case of a pons-mesencephalon lesion, which is rarely caused by other diseases. Convergence of the bulbs may be preserved, but in case of looking sideways, adduction is impaired, and the abducting eye may develop monocular nystagmus. Damage to the vestibulocerebellar pathways or cerebellar lesions may cause nystagmus. Cranial CT using contrast material is able to detect demyelinisational foci, but its sensitivity is lower in contrast with MRI tests. Irregular, circumscribed organic damage or epileptic dysfunction may be present, but is rarely detectable with EEG test. Myelography is used to assess space-occupying lesions in the spinal canal, therefore it cannot detect the demyelinisational foci in Multiple Sclerosis.
NEU - 3.15
Diagnostic criteria for NMO (neuromyelitis optica) are:
1) Clinical signs of optic neuritis and/or spinal lesion
2) Presence of aquaporin-4 antibodies in serum
3) Presence of LTM (extensive myelitis overlying several segments) on spinal MR findings
4) Presence of OGP (sign of intrathecal IgG synthesis) in CSF
A) answer 1, 2 and 3 correct
B) answer 1 and 3 correct
C) answer 2 and 4 correct
D) only answer 4 correct
E) all 4 answers correct
ANSWER
A) answer 1, 2 and 3 correct
EXPLANATION
Diagnostic criteria for neuromyelitis optica (NMO) are: 1. longitudinally extensive myelitis with lesion extending over 3 vertebral segments on MRI examination, 2. optic neuritis (neuritis retrobulbaris unilaterally or bilaterally, often recurrent) 3. the presence of antigens against the aquaporin 4 antigen in serum (and/or in liquor). OGP is seldom detectable in the liquor, therefore it does not belong to diagnostic criteria
NEU - 3.16
Leading clinical signs of acute Guillain–Barre syndrome are:
1) possible cranial nerve lesion
2) ascending paresis
3) flaccid or absent deep reflexes
4) muscular atrophy
A) answers 1, 2 and 3 are correct
B) answers 1 and 3 are correct
C) answers 2 and 4 are correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
A) answers 1, 2 and 3 are correct
EXPLANATION
Acute Guillain-Barre syndrome involves peripheral motor and sensory symptoms. Deep reflexes may be flaccid or absent. Its typical form is characterized by ascending paresis and sensory loss starting from the lower extremity. The process may extend to the level of the cranial nerves. The disease rarely may begin by lesion of the cranial nerves, and this is its cranial form. In acute Guillan-Barre syndrome, muscle atrophy is not a common symptom.
NEU - 3.19
Characteristic alterations in the CSF in multiple sclerosis:
1) increased glucose content
2) elevated levels of relative immunglobulin- IgG values
3) increased polymorphonuclear leucocyte count in 1/3 of cases
4) increased protein content in app. 1/3 of cases
A) answer 1, 2 and 3 correct
B) answer 1 and 3 correct
C) answer 2 and 4 correct
D) only answer 4 correct
E) all 4 answers correct
ANSWER
C) answer 2 and 4 correct
EXPLANATION
The sugar content in the liquor does not change in Multiple Sclerosis, it is the protein content which changes. Total protein count tends to be within normal limits, but in app. 1/3 of the cases a moderate increase can be observed. Liquor IgG levels are increased, intrathecally synthesized IgG appears in oligoclonal bands (oligoclonal gammopathy, OGP) through electrophoresis. OGP is found in 80-90% of the cases, therefore, it is a significant sign for diagnosis. The appearance of polymorphonuclear leucocytes in the liquor are contraindications for Multiple Sclerosis diagnosis.
NEU - 3.20
Characteristic symptoms of Guillain–Barre syndrome are:
1) flaccid paresis, absent deep reflexes
2) sensory deficit does not occur
3) cell-protein dissociation is commonly observed in the CSF
4) survival extends usually for a few months, maximum two years
A) answers 1, 2 and 3 correct
B) answers 1 and 3 correct
C) answers 2 and 4 correct
D) only answer 4 correct
E) all of the answers correct
ANSWER
B) answers 1 and 3 correct
EXPLANATION
Guillain-Barre syndrome is an inflammatory polyneuropathy, affecting the motor, sensory and vegetative fibers of the peripheral nerves. Due to the damage to motor fibers, hypotonia, paresis, flaccidity or absence of deep reflexes are observed. Sensory dysfunction is characterized by polyneuropathy. If sensory dysfunction is absent, supervision of the diagnosis is indicated. Acute Guillain-Barre syndrome involves elevation in liquor protein content, but is not followed by elevation in cell count. This phenomenon is termed cell-protein dissociation, and also appears in spinal space-occupying lesions (liquor under compression). Recovery may last for months. Death may result in 3-5% of cases during the progressive stage of disease, due to paralysis of respiratory muscles, or cardiac arrhythmias from autonomic dysfunctions. In later stages of the disease, lung embolism, or pneumonia may lead to death (permanent immobilization).
NEU - 3.21
Characteristics of multiple sclerosis are:
1) it is more common is white people
2) it is most common in a relapse-remission pathological form
3) multifocal neurological symptoms
4) first symptoms usually present over age 50
A) answer 1, 2 and 3 are correct
B) answer 1 and 3 are correct
C) answer 2 and 4 are correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
A) answer 1, 2 and 3 are correct
EXPLANATION
Epidemiological studies show that prevalence of Multiple Sclerosis is higher among white people. Detection of several neurological foci is fundamental in establishing the correct diagnosis. Relapse and remission commonly alternate. In app. 50% of cases, the disease begins between 20-40 years, its first presentation is very rare over 50 years of age.
NEU - 3.22
Common clinical signs of multiple sclerosis are:
1) paraesthesia
2) optic neuritis
3) paresis
4) ataxia
A) answers 1, 2 and 3 are correct
B) answers 1 and 3 are correct
C) answers 2 and 4 are correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
E) all 4 answers are correct
EXPLANATION
In Multiple Sclerosis demyelinisation foci may appear in any part of the neurological system. Therefore, clinical signs may be varied. The disease often begins with inflammation of the optic nerve (neuritis retrobulbaris), or with unusually located paraesthesia. Paresis and ataxia also appear in most cases.
NEU - 3.23
Common chronic symptoms of multiple sclerosis are:
1) pathological tiredness (fatigue)
2) disordered optic pathways and eye movement
3) vigorous reflexes
4) epilepsy
A) answers 1, 2 and 3 correct
B) answers 1 and 3 correct
C) answers 2 and 4 correct
D) only answer 4 correct
E) all of the answers correct
ANSWER
A) answers 1, 2 and 3 correct
EXPLANATION
Patients are usually fatigable due to involvement of the motor system, and coordination disturbances. Due to damage to pyramidal pathways, vigorous or increased deep reflexes are observed. Eye movement abnormalities are common physical symptoms (internuclear ophthalmoplegia). Epileptic seizures are rare, but may occur in Multiple Sclerosis.
NEU - 3.24
Common chronic symptoms of multiple sclerosis may be:
1) dizziness
2) confabulation
3) weakness in the extremities, spasticity
4) brain oedema
A) answers 1, 2 and 3 are correct
B) answers 1 and 3 are correct
C) answers 2 and 4 are correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
B) answers 1 and 3 are correct
EXPLANATION
Multiple Sclerosis may involve an elated mood, loss of critical sense and cognitive decline due to damage to the frontal lobe. Memory is usually not impaired and confabulation is not a characteristic symptom. The disease does not cause brain oedema or confusion. Damage to central motor neurons, the brain stem and to cerebellum cause characteristic symptoms.
NEU - 3.25
Useful diagnostic procedures of multiple sclerosis:
1) patient’s medical history and detailed neurological examination
2) cranial MRI examination
3) evoked potential tests
4) CSF test
A) answer 1, 2 and 3 correct
B) answer 1 and 3 correct
C) answer 2 and 4 correct
D) only answer 4 correct
E) all 4 answers correct
ANSWER
E) all 4 answers correct
EXPLANATION
The McDonald criteria is used to establish the diagnosis for Multiple Sclerosis. It involves the documentation of the disseminated appearance of symptoms in terms of both time and space relationships. An accurate medical history is inevitable to confirm the course of disease (primary progressive form, relapse-remission form etc.). Physical examination, MRI, and triggered response reaction tests are used to detect the neurological lesions in Multiple Sclerosis. In app. 90% of cases, oligoclonal gammopathy (OGP) appears in the liquor. Which means, all the above mentioned modalities of assessment are significant elements in establishing the correct diangosis.
NEU - 3.26
The following patients are at an increased risk for multiple sclerosis:
1) young adults
2) women
3) North-European whites
4) a person who has a close relative suffering from multiple sclerosis
A) answers 1, 2 and 3 correct
B) answers 1 and 3 correct
C) answers 2 and 4 correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
E) all 4 answers are correct
EXPLANATION
The disease appears between the ages of 20-40 in half of the cases, and women are app. 1,4 to 2 times more likely to contract it than men. Disease prevalence is highest in white people living under moderate climate (average of 30-80 patients in every 100,000 inhabitants). Blood relatives are at a 15 times higher risk for developing the disease.
NEU - 3.27
Diagnostic specificities of multiple sclerosis (MS):
1) high IgG index in the CSF is specific for MS
2) extended latency values on VEP (visually evoked potentials) are specific for MS
3) gadolinium accumulating white matter lesions detected on MRI are specific for MS
4) dissemination of clinical signs in space and time is prime criterion for the diagnosis
A) answers 1, 2 and 3 are correct
B) answers 1 and 3 are correct
C) answers 2 and 4 are correct
D) only answer 4 is correct
E) all 4 answers are correct
ANSWER
D) only answer 4 is correct
EXPLANATION
There is no single paraclinical test that is specific for MS, therefore, test results must always be evaluated together with the clinical picture. Diagnosis is primarily based on the dissemination of clinical signs in space and time. Intracerebral immunoglobulin synthesis is determined by calculating the IgG index values. Intracerebral IgG synthesis is an important sign of the disease, but not specific for MS. Triggered response reaction tests are used to detect subclinical lesions, but are not disease specific, either. Native MRI tests are similar from that respect, and are used to test for the demyelinisating character of the foci, and may also indicate the multiplied nature of the process, while possible gadolinium enhancement may indicate disease activity. These deviations on MR findings do not occur only with MS.
NEU - 3.28
During the treatment of multiple sclerosis relapse:
1) high dose intravenous methylprednisolon for 3–5 days are administered
2) steroid use is not prescribed in the treatment of psychosis
3) in severe diabetes or acute infection, steroids are not administered
4) beta-interferon is administered instantenously
A) answer 1, 2 and 3 correct
B) answer 1 and 3 correct
C) answer 2 and 4 correct
D) only answer 4 correct
E) all 4 answers correct
ANSWER
A) answer 1, 2 and 3 correct
EXPLANATION
Short term treatment with the administration of high-dose steroids (so called shock therapy), or schub treatment, is advised for Multiple Sclerosis. Due to the common steroid side effects, this treatment is contraindicated in patients with psychosis, diabetes mellitus, or infectious diseases. β-interferon treatment is an immunomodulatory periodic treatment.