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Adams Principles of Neurology > PNS > Flashcards

PNS Flashcards

1
Q

T/F: Unmyelinated fibres are more numerous in peripheral nerves than myelinated ones.

A

True

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2
Q

It is the most susceptible element of the nerve fiber.

A

Myelin sheath

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3
Q

Focal degeneration of the myelin sheath with sparing of the axon

A

segmental demyelination

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4
Q

A reaction of both the axon and myelin distant to the site of disruption of an axon

A

Wallerian degeneration

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5
Q

It is a highly characteristic histopathologic change in the nerve cell body characterised as swelling of the cell cytoplasm and marginalisation and dissolution of the Nissl substance

A

chromatolysis

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6
Q

A condition with an axonal process in which there may be mainly proximal weakness.

A

Porphyria

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7
Q

T/F: The maximum degree of denervation atrophy after an acute injury to the axons occurs in 90-120 days and reduces muscle volume by 75-80%.

A

True

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8
Q

T/F: In the toxic and metabolic neuropathies, sensory loss usually exceeds weakness.

A

True

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9
Q

T/F: Polyradiculopathy differs from polyneuropathy in that the neurologic signs are asymmetrical with an erratic distribution.

A

True

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10
Q

Autoantibody seen in almost all patients with ophthalmoplegia in GBS

A

anti-GQ1b

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11
Q

Autoantibody with the highest tigers being associated with GBS cases that follow Campylobacter infection

A

Anti-GM1

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12
Q

In GBS, vital capacity below this level necessitates mechanical ventilation

A

10 ml/kg

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13
Q

The only serious reaction encountered in IVIg infusion

A

Anaphylaxis in those who congenitally lacked IgA

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14
Q

T/F: 5-10% of GBS patients encounter recurrence of the acute polyneuropathy.

A

True

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15
Q

Curative of acute ureic polyneuropathy

A

kidney transplantation

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16
Q

Initial and often the most prominent symptom of acute intermittent porphyria

A

moderate to severe colicky abdominal pain

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17
Q

The use of intravenous glucose and intravenous hematin is deemed as the most effective therapy

A

Porphyric Polyneuropathy

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18
Q

Agents that can produce a polyneuropathy that may be fatal in a matter of days

A

Triorthocresyl phosphate (TOCP), thallium, arsenic and other organophosphates

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19
Q

T/F: Almost all cases of paraneoplastic sensory neuropathy demonstrate anti-Hu antibodies.

A

True

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20
Q

It has the characteristic presentation of a motor mononeuropathy in the distribution of the radial nerves

A

Lead neuropathy

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21
Q

T/F: Chemotherapeutic agents particularly cisplatin, carboplatin and bortezomib are known to evoke a dose dependent predominantly sensory polyneuropathy

A

True

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22
Q

T/F: Isolated involvement of practically all the major peripheral nerves has been described in diabetes and the most frequently affected is the femoral nerve.

A

True

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23
Q

T/F: More than half of all cases of mononeuropathy multiplex can be traced to a systemic vasculitis involving the vasa nervorum.

A

True

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24
Q

T/F: A characteristic feature of Churg-Strauss disease is the excess of circulating and tissue eosinophils and a tendency of the vasculitis to involve the lungs and skin.

A

True

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25
Q

Cryoglobulinemia is associated with this infection?

A

Hepatitis C

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26
Q

Triad of cranial nerve palsies, radiculitis and aseptic meningitis

A

Lyme disease

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27
Q

T/F: In contrast to acute GBS, many cases of CIDP respond favourably to the administration of prednisone

A

True

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28
Q

T/F: Multifocal motor neuropathy predominate in men.

A

True

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29
Q

Intermediate host for leprosy

A

Armadillos

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30
Q

T/F: A polyneuropathy that advances slowly over 10 years or more is almost invariably genetic in origin.

A

True

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31
Q

It is a multi system neurologic disease characterized by the widespread deposition in nervous tissue of corpora amylacea

A

Polyglucosan disease

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32
Q

Clinical manifestation of retinitis pigments, ataxia and chronic polyneuropathy coupled with an increase in blood phytanic acid.

A

Refsum disease

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33
Q

Drugs which may be helpful to alleviate the pain in Fabry Disease

A

Phenytoin, Carbamazepine, Gabapentin and Amitriptyline

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34
Q

well-known complications of acromegaly

A

nerve entrapment, polyneuropathy

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35
Q

Proven curative of familial amyloid polyneuropathy

A

Liver transplantation

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36
Q

If there is male-to-male transmission in Charcot-Marie-Tooth Disease, what gene should be investigated?

A

GJB1

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37
Q

It may be the only electrophysiologic abnormality early after a traumatic injury of the brachial plexus

A

absence of late responses (F wave)

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38
Q

The usual cause of a lesion of the entire brachial plexus

A

vehicular trauma

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39
Q

T/F: Erb-Duchenne palsy usually persist throughout life

A

True

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40
Q

Genetic basis for heredofamilial brachial plexopathy

A

SEPN1

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41
Q

Dose of radiation associated with radiation damage

A

> 6000 cGy

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42
Q

supplies the serratus anterior muscle which fixates the lateral scapula to the chest wall

A

long thoracic nerve of Bell

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43
Q

supplies the supraspinatus and infraspinatus muscles

A

suprascapular nerve

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44
Q

most frequent nerve entrapment syndrome

A

Carpal tunnel syndrome

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45
Q

consists of hyperflexion of the wrist for 30-60 seconds

A

Phalen Maneuver

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46
Q

elicited by lightly tapping the volar aspect of the wrist at the transverse carpal ligament (distal to the first wrist crease)

A

Tinel sign

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47
Q

manifest by a characteristic clawhand deformity, wasting of the small hand muscles

A

complete ulnar paralysis

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48
Q

T/F: The earliest symptom in metastatic lumbosacral plexopathy is usually pain whereas in radiation plexopathy it is weakness

A

true

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49
Q

this sensory nerve originates from the 2nd and 3rd lumbar roots and supplies the anterolateral aspect of the thigh from the level of the inguinal ligament

A

Lateral Cutaneous nerve

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50
Q

The most common cause of femoral neuropathy

A

Diabetes

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51
Q

Most common cause of sciatica

A

rupture of one of the lower lumbar intervertebral discs

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52
Q

T/F: Diplopia is common in MG but it does not correspond to the innervatory pattern of a nerve, instead it is the result of asymmetricak weakness of several muscles in both eyes.

A

True

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53
Q

T/F: Familial occurrence of Myasthenia Gravis is known but is rare.

A

True

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54
Q

T/F: The period of danger of death in MG is greatest in the 1st year and from 4-7 years after the onset.

A

True

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55
Q

T/F: A later age at onset of MG is associated with higher incidence of fatal respiratory crisis.

A

True

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56
Q

T/F: The number and size of the presynaptic vesicles and their quanta of acetylcholine are normal in Myasthenia graves.

A

True

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57
Q

T/F: A proportion of young women with MG have moderately elevated tigers of ANA without the clinical manifestations of SLE.

A

True

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58
Q

Given several minutes in advance to counteract the unpleasant muscarinic effect of neostigmine

A

Atropine 0.8mg

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59
Q

Starting dose of Azathioprine in MG

A

50 mg BID

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60
Q

An agent used in Lambert-eaton syndrome that blocks potassium channels in the distal motor terminal enhancing the release of Ach vesicles.

A

3,4-diaminopyridine (3,4-DAP)

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61
Q

Prenatal myasthenic disease which consist of arthrogryposis, pterygia and respiratory distress.

A

Escobar Syndrome

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62
Q

The serum enzyme other than CK that is derived predominantly from skeletal muscle.

A

Aldolase

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63
Q

Most susceptible element of the nerve fiber

A

Myelin sheath

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64
Q

Most common cause of acute or subacute generalized paralysis in practice.

A

Guillain-Barre syndrome

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65
Q

Most frequent identifiable antecedent infection in GBS

A

Campylobacter jejuni

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66
Q

T/F: Pain and aching discomfort usually precede weakness in GBS.

A

True

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67
Q

Hypokalemic periodic paralysis is associated with mutations in genes encoding for what ion channels?

A

Ca (10% Na)

68
Q

Helpful in the prevention of HPP

A

Low sodium diet, avoidance of large meals and of exposure to cold, acetazolamide

69
Q

Defined as sustained contraction or failure of muscular relaxation.

A

Myotonia

70
Q

Autoantibody associated with the pharyngeal-cervical-brachial syndrome

A

GT1a

71
Q

T/F: in contrast to the acute polyneuropathies, subacute polyneuropathy has prominent sensory features and are of axonal type.

A

True

72
Q

In paraneoplastic neuropathy, improvement occurs if the tumor can be treated effectively except for this type?

A

Sensory neuronopathy

73
Q

Nerve affected by Lead neuropathy

A

Radial nerve

74
Q

It is the most common cause of polyneuropathy in general practice

A

Diabetes Mellitus

75
Q

Nerve biopsy will show necrotizing arterities in medium-sized vessels with infiltrating eosinophils and occlusio of vessels.

A

Polyarteritis nodosa

76
Q

Finding of circulating c-ANCA

A

Wegener granulomatosis

77
Q

What type of arteritis occurs in Rheumatoid Arthritis?

A

Small-vessel fibrinoid type

78
Q

Involvement of a single nerve with sarcoid most often implicates which nerve?

A

Facial nerve

79
Q

Treatment of Lyme neuropathic syndrome

A

Ceftriaxone 2g OD for 1 month

80
Q

Confirmatory test for celiac-sprue neuropathy

A

Antigliadin antibodies, anti-transglutaminase antibodies and histologic examination of a duodenal biopsy

81
Q

T/F: monoclonal proteins underlie the largest group of otherwise unexplained neuropathies in adults.

A

True

82
Q

Most often paraprotein class associated in polyneuropathy in adults.

A

IgM

83
Q

Systemic condition occurring mainly in older persons and characterized by fatigue, weakness and a bleeding diathesis.

A

Macroglobulinemia

84
Q

Most useful screening test for amyloid neuropathy

A

Serum and urine analysis searching for an abnormal paraprotein

85
Q

T/F: In CIDP, the clinical course is monophasic in 1/3, stepwise and progressive in another third and relapsing in the remaining third.

A

True

86
Q

Treatment for multifocal motor conduction block and motor neuropathy

A

IVIg infusion

87
Q

The maximum degree of denervation atrophy after an acute injury to the axons occurs in how many days?

A

90-120 days

88
Q

Main disability in CMT disease

A

Walking difficulty

89
Q

The only distinguishing clinical feature between CMT type 1 and 2?

A

Enlargement of the nerves in type 1

90
Q

The most prevalent form of CMT disease

A

CMT1A

91
Q

A condition which is due to deletion of PMP22 in chromosome 17

A

Hereditary liability to pressure palsies (HNPP) (duplication in CMT disease)

92
Q

T/F: in both CMT1A and HNPP, the PMP22 gene is functionally normal.

A

True

93
Q

Diagnosis is based on a combination of retinitis pigmentosa, ataxia and chronic polyneuropathy coupled with an increase in phytanic acid.

A

Refsum disease (HMSN IV)

94
Q

Manifested by the presence of enlarged yellow-orange (cholesterol-laden) tonsils

A

Tangier’s disease

95
Q

Disease characterized by the congenital absence of the degradative enzyme sulfatase

A

Metachromatic Leukodystrophy

96
Q

Often the most prominent manifestation of amyloidosis

A

Peripheral neuropathy

97
Q

Vasculitic neuropathy characterized by its abrupt onset of pain or numbness at a focal site along a nerve followed in hours or days by motor or sensory loss in the distribution of that nerve and then by involvement in a saltatory fashion of other peripheral nerves

A

Polyarteritis nodosa

98
Q

T/F: most patients with brachial plexus neuropathies develop without apparent cause

A

True

99
Q

Genetic basis of heredofamilial brachial plexopathy

A

Mutation of SEPN1

100
Q

Most common disorder affecting the median nerve and the most frequent nerve entrapment syndrome

A

Carpal tunnel syndrome

101
Q

Manifested by the characteristic clawhand deformity

A

Complete ulnar paralysis

102
Q

Most common cause of femoral neuropathy

A

Diabetes

103
Q

The most frequently compressed nerve

A

Median nerve

104
Q

T/F: The lingual nerve is a branch of the trigeminal mandibular nerve

A

True

105
Q

T/F: Bell’s palsy is probably more common in diabetic and hypertensive patients.

A

True

106
Q

In Bell’s palsy, what precedes recovery of motor function?

A

Recovery of taste

107
Q

Bilateral facial palsy that is acute in onset and is associated with parotid gland swelling from sarcoidosis

A

Uveoparotid fever or Heerfordt syndrome

108
Q

The sequential painless affection of contiguous or noncontiguous nerves over several days or weeks is particularly characteristic of what condition?

A

Meningeal carcinomatosis or lymphomatosis

109
Q

Most frequent and most elusive symptom of myopathy.

A

Weakness

110
Q

Another form of fibrous contracture that is found in newborns involving multiple muscle groups.

A

Arthrogryposis

111
Q

T/F: As a general rule, muscle diseases are identified by a predominantly proximal weakness that is symmetric.

A

True

112
Q

Popeye effect

A

Fascioscapulohumeral dystrophy

113
Q

Most common cause of isolated quadriceps femoris weakness

A

IBM or a restricted form of Becker muscular dystrophy

114
Q

The distinguishing feature of the disease is the early appearance of contractures in the flexors of the elbow, extensors of the neck and posterior calf of the muscles.

A

Emery-Dreifuss muscular dystrophy

115
Q

The distinguishing feature of the disease is the early appearance of contractures in the flexors of the elbow, extensors of the neck and posterior calf of the muscles.

A

Emery-Dreifuss muscular dystrophy

116
Q

T/F: The later the onset of Erb dystrophy, the more likely the course will be benign.

A

True

117
Q

Most common adult muscular dystrophy

A

Myotonic dystrophy Type 1

118
Q

Associated with an exaggerated forward curvature of the neck (“swan neck”)

A

DM1

119
Q

Most common distal muscular dystrophy associated with dysferlin mutation

A

Miyoshi dystrophy

120
Q

Characterized by a docal dissolution of myofibrils followed by an accumulation of degradative products

A

Myofibrillar myopathy

121
Q

Optimal dose of prednisone which appears to slightly retard the tempo of progression of Duchenne dystrophy

A

0.75mg/kg given daily

122
Q

T/F: With the exception of phosphoglycerate kinase deficiency, all the glycogenoses are inherited as autosomal recessive traits.

A

True

123
Q

The primary abnormality is a deficiency in myophosphorylase

A

McArdle disease

124
Q

How many percent of patients with myasthenia have hyperthyroidism?

A

5% (MG is 20-30x higher in hyperthyroidism)

125
Q

In myoglobinuria, this is said to protect the kidneys by preventing myoglobin casts

A

Alkalinization of the urine by ingestion or infusion of sodium bicarbonate

126
Q

The muscle biopsy shows elements of both myopathic and neuropathic disease with the special feature in muscle of rimmed vacuoles on the Gomori trichrome stain that are more central in the muscle fibers than those seen with inclusion body myositis.

A

Colchicine myoneuropathy

127
Q

This enzyme is thought to function primarily during aerobic exercise and facilitate the regeneration of ATP from ADP through the action of adenylate kinase.

A

Myoadenylate

128
Q

Disease characterized by severe cramping of muscle, universal alopecia, amenorrhea, intestinal malabsorption, epiphyseal destruction and retarded growth

A

Satoyoshi syndrome

129
Q

Uncommon disease characterized by myotonia, muscular hypertrophy, nonprogressive course and dominant inheritance.

A

Myotonia congenita (Thomsen Disease)

130
Q

Tonic spasm of muscle after forceful voluntary contraction

A

Myotonia

131
Q

Infantile form of acid maltase deficiency

A

Pompe disease

132
Q

The pathological hallmark for mitochondrial myopathy

A

Ragged red fibers

133
Q

T/F: During the rigor phase in malignant hyperthermia, the oxygen consumption in muscle increases three-fold and serum lactate 15-20 fold.

A

True

134
Q

Distinct form of periodic paralysis characterized by the triad of weakness, ventricular dysrhythmias with long QT syndrome and dysmorphic features.

A

Andersen disease

135
Q

The phenomenon of pseudomyotonia is observed in what condition?

A

Hypothyroidism

136
Q

Condition of persistent and intense spasms, particularly of the proximal lower limbs and lumbar paraspinal muscles.

A

Stiff man syndrome

137
Q

Most characteristic finding in stiff man syndrome

A

Axial spasm

138
Q

Myopathic condition with predominantly distal weakness and facial atrophy

A

Myotonic dystrophy

139
Q

Most common type of Carnitine Palmitoyltransferase Deficiency

A

Type 1

140
Q

Most common dystrophic change in a nonmuscular tissue in myotonic dystrophy

A

Lenticular opacities

141
Q

What antibody is found in myasthenia gravis patients with negative AchR antibodies

A

MuSK antibody

142
Q

Treatment of choice for multifocal motor neuropathy

A

IVIg infusion

143
Q

Mechanism of action of nitric oxide damage to the spinal cord

A

Cobalamin inactivation

144
Q

Sural nerve biopsy shows multiple focal thickening of myelin sheaths called tomaculum

A

Hereditary neuropathy with liability to Pressure palsies (HNPP)

145
Q

Most common neurologic manifestation of multiple myeloma

A

Compressive radiculopathy

146
Q

May present with distal arm and proximal leg weakness

A

Inclusion body myositis

147
Q

Most prevalent neuromuscular disease in adults

A

Classic myotonic dystrophy type 1

148
Q

T/F: Dermatomyositis affects children and adults while polymyositis affects adults and rarely children.

A

True

149
Q

Causes disruption of muscle microtubule-dependent cytoskeletal network which leads to deficient transport and intracellular accumulation of lysosomes and autophagic vacuoles

A

Colchicine myopathy

150
Q

Mostly affected in mitochondrial disorders

A

Transfer RNA

151
Q

T/F: Nearly all cases of PEO are because of mitochondrial disorders.

A

True

152
Q

Most typical feature of MERFF

A

Myoclonus

153
Q

A 33 year old man presents with progressive weakness and impaired muscle relaxation, cataract, “hatchet face” and male pattern baldness with facial weakness. The genetic abnormality is most likely

A

Expanded unstable CTG repeat on chromosome 19q

154
Q

This is also known as Rippling muscle disease because of severe elevation of Creatine kinase

A

Caveolinopathy

155
Q

Bunina bodies is pathognomonic of what disease?

A

ALS

156
Q

Cause of the waddle in Duchenne muscular dystrophy

A

Bilateral weakness of the gluteus medius

157
Q

T/F: In dermatomyositis patients who respond to steroids, the serum CK decreases before the weakness subsides while with relapse, the serum CK rises before the weakness returns.

A

True

158
Q

The clinical manifestations of neuropathic disorders are distal weakness and atrophy. However, what neuropathic condition present with proximal weakness instead?

A

Spinal muscular atrophy

159
Q

T/F: Paresthesia and sensory loss in CTS often involves all fingers but should not involve the thenar eminence.

A

True

160
Q

T/F: In cases of chronic muscle diseases, avoid biopsies of severely affected muscles but in cases of relatively acute muscle disease, select severely affected muscles.

A

True

161
Q

Most common cause of femoral neuropathy

A

Diabetes

162
Q

T/F: In entrapment neuropathies, function is gradually impaired, motor more than sensory

A

False (sensory more than motor)

163
Q

T/F: In contrast to acute polyneuropathies, however, most that are subacute have prominent sensory features and are of axonal type.

A

True

164
Q

T/F: Combined paralysis of the levator and orbicularis oculi muscles indicate a myopathic disease.

A

True

165
Q

T/F: Carbohydrate is metabolized during aerobic and anaerobic phases of metabolism; fatty acids are metabolized only aerobically.

A

True

Adams Principles of Neurology (19 decks)

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