Neurology Flashcards

1
Q

Clinical features in secondary headaches - space-occupying lesion

A
  • Worse when lying down or coughing
  • Morgning vomiting
  • Headache cause night-time waking
  • Change in mood, personality, or educational performance
  • Visual field defects
  • Cranial nerve abnormalities causing diplopia, new-onset squint or facial palsy
  • Abnormal gait
  • Torticollis (tilting of the head)
  • Growth failure
  • Papilledema
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2
Q

Definition of ac epileptic seizure

A
  • Due to excessive and hyper synchronous electrical activity
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3
Q

Definition of convulsions

A

A seizure with motor components; stiff (tonic), massive jerk (myoclonic), jerking (clonic), trembling (vibratory), thrashing about (hyper motor)

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4
Q

Definition of acute symptomatic epileptic seizures

A
  • Seizure provoked by an acute brain injury, e.g. from ischemia, cerebral contusion, or cortical inflammation. Do not constitute an epilepsy.
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5
Q

Characteristics of a febrile seizure

A
  • In children between age 6 months - 6 years
  • 10% risk if first-degree relative
  • Seizure usually occur real in a viral infection when tmp is raising rapidly
  • Usually generalized tonic-clonic seizures
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6
Q

Most common cause of transient loss of consciousness?

A

Syncope –> due to transient impairment of brain oxygen delivery

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7
Q

Causes of paroxysmal disorders (‘Funny turns’)

A
  • ‘Blue breath-holding’ spells - toddlers hold breath when upset, expiratory apnea
  • Reflex asystolic syncope - due to cardiac systole from vagal inhibition. Triggered by pain or discomfort from head trauma, cold food, fright or fever.
  • Syncope
  • Migraine
  • Benign paroxysmal vertigo
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8
Q

Most common cause of epilepsy

A
  • Genetic (i.e. “idiopathic”) - complex inheritance
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9
Q

Definition of generalized seizures

A
  • Discharge arises from both hemispheres
  • Loss of consciousness if >3 seconds duration
  • No warning
  • Symmetrical seizures
  • Bilaterally synchronous seizure discharge on EEG
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10
Q

Defintion of focal seizure

A
  • Seizure aries from one or part of one hemisphere
  • May be heralded by an aura which reflects the site of origin
  • May or may not be associated with change in consciousness or evolve to generalized tonic-clonic seizure
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11
Q

Jacksonian march

A

A frontal seizure that is clonic, the travel proximally.

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12
Q

Characteristics of a temporal lobe seizure

A
  • Auditory or sensory (smell or taste) phenomena

- Lip-smacking, plucking at one’s clothing, walking in a non-purposeful manner

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13
Q

Characteristics of occipital seizures

A
  • Stereotyped visual hallucinations
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14
Q

Characteristics of parietal lobe seizures

A
  • Contralateral altered sensation (dysaesthesias)

- Distorted body image

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15
Q

Causes of seizures

A
  1. Epilepsi
    * Genetic (70-80%) = “idiopathic”
    * Structural, metabolic - cerebral malformation, HIE, IVH, cerebral tumor, neurodegenerative disorders
  2. Acute symptomatic seizures
    * Stroke, intracranial infection
    * Hypoglycemia, hypocalcemia, hypomagnesemia
    * Poisons/toxins
  3. Febrile seizures
  4. Non-epileptic seizures
    * Convulsive syncope
    * Sudden rise in ICP
    * Sleep disorders
    * Functional/medically unexplained
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16
Q

Absence seizure

A
  • Transient loss of consciousness
  • Abrupt onset and termination
  • Flickering of the eyelids, minor alteration in muscle tone.
  • Can often be precipitated by hyperventilation
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17
Q

Types of generalized epileptic seizures

A
  • Absence
  • Myoclonic
  • Tonic
  • Tonic-clonic
  • Atonic
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18
Q

Myoclonic seizure

A
  • Brief, often repetitive, jerking movements of the limbs, neck or trunk.
  • Physiology: Hiccups (myoclonus of diaphragm), passing though stage II sleep
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19
Q

Tonic seizure

A
  • Generalized increased muscle tone
20
Q

Tonic-clonic seizure

A
  • Rhythmical contraction of muscle groups following the tonic phase
  • Tonic phase - children may fall
  • Do not breath - cyanosed
  • Clonic phase - jerking of the limbs. Irregular breathing, cyanosis persist, saliva accumulate in mouth
  • Biting of tongue, incontinence of urine.
  • Seizure last from few second to minutes - then unconsciousness or deep sleep for up to several hours.
21
Q

Atonic seizure

A
  • Combined with a myoclonic jerk - then transient loss of muscle tone causing a sudden fall to the floor or drop of the head.
22
Q

Characteristics of frontal lobe seizure

A
  • Motor phenomena
23
Q

What is the drug used in rescue therapy of >5min seizure

A

Buccal midazolam

24
Q

Infantile spasms (West syndrome)

A
  • Age: 3-12 months
  • Violent flexor spams of head, trunk and limbs –> the extension of arms
  • Multiple bursts of 20-30, each last 1-2 s.
  • Most have underlying neurologic cause
  • Treatment: Vigabatrin and/or corticosteroids
25
Q

Lennox-Gastatut syndrome

A
  • Age: 1-3 years
  • Multiple seizure types - atonic, atypical absences and tonic seizures in sleep
  • Poor prognosis
26
Q

Childhood absence epilepsy

A
  • Age: 4-12 years
  • Momentary, unresponsive stare with motor arrest
  • May twitch eyelid, hand, or mouth minimally
  • Sudden onset, last <30 s.
  • No recall of seizure, realizes that they have missed something
27
Q

Which drug should be avoided in absence and myoclonic seizures?

A

Carbamazepine

28
Q

Definition of status epilepticus

A

Seizure lasting 30 minutes or repeated seizures for 30 minutes without recovery of consciousness

29
Q

Features of a neuromuscular disorder

A
  • Key feature: weakness
  • Hypotonia (floppiness)
  • Delayed motor milestones
  • Unsteady/abnormal gait
  • Fatiguability
  • Muscle cramps
30
Q

Gowers’ sign

A
  • Suggestive of proximal muscle weakness
  • Child need to turn prone to rose to a standing from supine position
  • ‘Climb up’ the legs with the hands
  • Associated with Duchenne muscular dystrophy
31
Q

Disorders of the anterior horn cell

A

Signs of denervation: weakness, loss of reflexes, fasciculation, wasting

  1. Spinal muscular atrophy
  2. Poliomyelitis
32
Q

Disorders of the peripheral nerve

A
  1. Hereditary motor sensory neuropathies
  2. Guillain-Barré
  3. Bell palsy
33
Q

Disorders of neuromuscular transmission

A
  1. Myasthenia gravis
34
Q

Muscle disorders

A
  1. Muscle dystrophies (Duchenne, Becker, Congenital)
  2. Inflammatory myopathies (Benign acute myositis, Polymyositis)
  3. Myotonic disorders (Dystrophia myotonica)
  4. Metabolic myopathies
  5. Congenital myopathies
35
Q

Genetics of Spinal muscular atrophy

A
  • Autosomal recessive

- Mutation in SMA1 gene

36
Q

Pathophys. of Spinal muscular atrophy

A

Degeneration of the anterior horn cells

- Leading to progressive weakness and wasting of skeletal muscles

37
Q

Types of Spinal muscular atrophy

A

SMA1. Severe infantile (Werdnig-Hoffman disease) - most common
SMA2. late infancy, slower progression
SMA3. chronic juvenile

38
Q

Cause of Guillain-Barré syndrome

A
  • 2-3 weeks after an URT infection or campylobacter gastroenteritis
  • Antibodies against protein components of myelin
39
Q

Clinical features of Guillain-Barré syndrome

A
  • Ascending, progressive symmetrical weakness over a few days to 2 weeks
  • Loss of tendon reflexes
  • Loss of autonomic involvement –> tachycardia, bradycardia and other arrhythmias, hypertension, orthostatic hypotension, urinary retention, ileus, loss of sweating
  • Bilateral facial weakness (easily missed in children)
  • Difficulty with chewing and swallowing
  • Risk of aspiration
40
Q

Bell palsy is paresis of which nerve?

A

Lower motor neuron of the VIIth cranial nerve

41
Q

Causes of Bell palsy

A

Etiology is unknown

Associated with post-infection - Herpes simplex, Lyme disease

42
Q

Clinical features of myasthenia gravis

A
  • Presentations usually after 10 years of age
  • Ophthalmoplegia
  • Ptosis
  • Loss of facial expression
  • Difficulty chewing
  • Generalized, especially proximal, weakness
43
Q

How to diagnose myasthenia gravis?

A
  1. Observe improvement following administration of I.V. Edrophonium over a few minutes
    or
  2. oral Pyridostigmine or Neostigmine over days
  3. 60-80% have positive actetylcholine receptor antibodies
44
Q

Treatment of myasthenia gravis

A
  • Choline esterase inhibitors - Pyridostigmine or Neostigmine
  • Immunosuppressive therapy
  • Prednisolone, Azathioprine, Mycophenolate mofetils, Monoclonal Ab
  • Thymectomy
  • Plasmapheresis
45
Q

Genetics of Duchenne muscular dystrophy

A
  • X-linked recessive

- Deletion of the gene for dystrophin (connects cytoskeleton of a muscle fibre to the surrounding extracellular matrix)

46
Q

What are the causes of dystrophin deficiency?

A
  1. Influx of calcium ions
  2. Breakdown of the calcium calmodulin complex
  3. An excess of free radicals –> leading to myofibre necrosis
47
Q

Clinical features of Duchenne muscular dystrophy

A
  • Waddling gait
  • Language delay
  • Gower’s sign - need to turn prone to rise
  • Pseudohypertrophy of the calves
  • Life expectancy - late twenties from respiratory failure or cardiomyopathy