JM Chapter 22

Question 1

combined LMN muscle atrophy plus
UMN hyper-reflexia, leading to progressive spasticity. This is the most common typ

Answer 1

amyotrophic lateral sclerosis (Lou Gehrig disease)

Question 2

• wasting beginning in the distal muscles; widespread
  fasciculation

Answer 2

progressive muscle atrophy

Question 3

Results in wasted I tongue, weakness of chewing and swallowing, and
of facial muscles

Answer 3

progressive bulbar (LMN) palsy and pseudobulbar palsy (LMN lesions in the brain stem
motor nuclei

Question 4

The hand tremor is most marked with the arms supported c
the lap and during walking.

The characteristic movement is ‘pill-rolling’

The resti
tremor decreases on finger-nose testing. The best way to evoke the tremor is to distract the
patient, such as focusing attention on the left hand with a view to ‘examining’ the right hand o
by asking the patient to turn the head from side to side.

Answer 4

Resting tremor-Parkinsonian

Question 5

This fine tremor is noted by examining the patient with the arms outstretched and the fingers
apart.

The tremor may be rendered more obvious if a sheet of paper is placed over the dorsum
the hands.

The tremor is present throughout movement, being accentuated by voluntary

Answer 5

Action or postural tremor

Question 6

This coarse oscillating tremor is absent at rest but exacerbated by action and increases as the
target is approached.

IT occurs in cerebellar lobe disease, with
lesions of cerebellar connections and with some medications.

Answer 6

Intention tremor (cerebellar disease)

Question 7

A flapping or ‘wing-beating’ tremor is observed when the arms are extended with
hyperextension of the wrists. It involves slow, coarse and jerky movements of flexion and
extension at the wrists.

Answer 7

Flapping (metabolic tremor)

Question 8

Triad of features
Positive family history
Tremor with little disability
Normal gait

Answer 8

Essential tremor

Question 9

Pakinsonsm

Answer 9

Question 10

Positive frontal lobe signs,
Pakinsonsm

Answer 10

grasp and glabellar taps

Question 11

is the gold standard for therapy
Pakinsonsm

Answer 11

L-dopa

Question 12

Anxiety / depression
Impaired vision
Diplopia
Ataxia
vertigo
Numbness / paresthesia /
Band sensation around trunk or limbs
Bladder urgencies
Incontinence
Constipation
Mor3 common in females
Facial palsy
Trigeminal neuralgia

Answer 12

MULTIPLE Sclerosis

Question 13

Inv of Multiple sclerosis

Answer 13

Lumbar puncture
MRI

Question 14

In outpatient setting the tx for Multiple Sclerosis for moderate relapse

Answer 14

PREDNISOLONE

Question 15

For mild relapse, tx for multiple sclerosis is

Answer 15

Rest and assurance

Question 16

Severe relapse of attacks of MS tx is

Answer 16

Methylprednisolone 1f in 200 ml, saline by slow IV infusion daily for 3 days

Question 17

What isbthe tx of spasticity in MS

Answer 17

Physiotheraoy
baclofen 10-25mg note
Alternative: Dantroleme

Question 18

Tx for Paroxysmal eg neuralgia

Answer 18

Carbamazepine or Gabapentine

Question 19

Classic glove and stocking sign

Answer 19

Peripheral Neuropathy

Question 20

Reflexes absent
Both proximal and distal muscles affected
Csf protein elevated

Answer 20

GBS

Question 21

Tx of GBS

Answer 21

Physiotherapy
Tracheostomy and artificial ventilation

Plasma exchange or IV Ig (0.4 G/KG DAY FOR 5 DYS)

Question 22

inherited autosomal dominant polyneuropathy with an insidious onset from puberty.

Clinical features include weakness in the legs, variable distal sensory loss and muscle atrophy
giving the ‘inverted champagne bottle’ appearance of the legs.

Answer 22

Charcot-Marie-Tooth syndrome

Question 23

young patient (usually adolescent)
day after vigorous exercise awakens with weakness in limbs (for 4-24 hours)
flaccid paralysis/loss of deep tendon reflexes
Related to potassium levels measure during symptoms. Classify as high, low or normal.

Answer 23

Familial periodic paralysis

Question 24

occasional mild ptosis to fulminant quadriplegia and
respiratory arrest,

affects muscle
strength.

associated with thymic tumour and other

autoimmune diseases, for example, RA, SLE, thyroid and pernicious anaemia.

Answer 24

Myasthenia gravis

Question 25

Clinical features Myasthenia gravis

Answer 25

Painless fatigue with exercise Weakness also precipitated by emotional stress, pregnancy, infection, surgery ocular: ptosis (60%) and diplopia (see FIG. 22.7 about 10% ); ocular myasthenia only remains in bulbar: weakness of chewing, swallowing, speech (ask to count to 100), whistling and head lolling limbs (proximal and distal) generalised respiratory: breathlessness, ventilatory failure

Question 26

40-year-old woman with a 12-month history of increasing muscular weakness including drooping of the eyelids Ptosis, especially on the right side, is apparent

Answer 26

Myasthenia gravis

Question 27

Myasthenia gravis Diagnosis

Answer 27

Serum anti-acetylcholine receptor antibodies Electrophysiological tests if antibody test negative CT scan to detect thymoma Edrophonium test still useful but potentially dangerous (atropine is the antidote)

Question 28

is recommended early for generalised myasthenia, especially in all younger patients with hyperplasia of the thymus, even if not confirmed preoperatively.

Answer 28

Thymectomy

Question 29

useful for acute crisis or where temporary improvement is required or patients are resistant to treatment

Answer 29

Plasmapheresis

Question 30

first-line Pharmacological agents: for Myasthenia Gravis

Answer 30

Pyridostigmine, Neostigmine or Distigmine),

Question 31

useful for all grades of MG (should be introduced slowly)

Answer 31

corticosteroids

Question 32

combination of ocular and facial weakness Smiling may have a characteristic snarling quality.

Answer 32

MYASTHENIA Gravis

Question 33

ptosis, eye facing 'down and out', dilated pupil, sluggish light reflex

Answer 33

3rd cranial nerve palsy-

Question 34

ptosis, miosis (constricted pupil), ipsilateral loss of sweating

Answer 34

Horner syndrome

Question 35

progressive external ophthalmoplegia or limb weakness, induced by activity- no pupil involvement

Answer 35

Mitochondrial myopathy

Question 36

ptosis and diplopia, no pupil involvement

Answer 36

Myasthenia gravis

Question 37

are sustained or intermittent abnormal repetitive movements or postures resulting from alterations in muscle tone. spasms may affect one (focal) or more (segmental) parts of the body or the whole body (generalised). often regarded as nervous tics.

Answer 37

Dystonia

Question 38

can induce a severe generalised dystonia (e.g. oculogyric crisis)

Answer 38

Neuroleptic and dopamine receptor blocking agents (e.g. L-dopa, metoclopramide) treated with benztropine 1-2 mg IM or IV.8

Question 39

is a focal dystonia of the muscles around the eye resulting in uncontrolled blinking, especially in bright light.

Answer 39

.Blepharospasm FOCAL DYSTONIA

Question 40

affects the jaw, tongue and mouth, resulting in jaw grinding movements and grimacing. Proper speech and swallowing may be disrupted

Answer 40

Oromandibular dystonia

Question 41

combination of blepharospasm and oromandibular dystonia.

Answer 41

Meige syndrome

Question 42

involves involuntary, irregular muscle contractions and spasms affecting one side of the face. It usually starts with twitching around the eye and then spreads to involve all the facial muscles on one side. It is usually due to irritation of the facial nerve in its intracranial course and surgical intervention may alleviate this problem

Answer 42

Hemifacial spasm

Question 43

are all occupational focal dystonias of the hand and/or forearm initiated by performing these skilled acts,

Answer 43

Writer's cramp, typist's cramp, pianist's cramp,

Question 44

a focal dystonia of the unilateral cervical muscles. It usually begins with a pulling sensation followed by twisting or jerking of the head, leading to deviation of the head and neck to one side. In early stages patients can voluntarily overcome the dystonia

Answer 44

Cervical dystonia

Question 45

is a focal dystonia of the laryngeal muscles resulting in a strained, hoarse or creaking voice. It may lead to inability to speak in more than a whisper.

Answer 45

Laryngeal or spastic dystonia

Question 46

The current treatment for focal or segmental (spread to an adjacent body region) dystonias is

Answer 46

localised injection of purified botulinum

Question 47

Motor and vocal tics are a feature of

Answer 47

Tourette disorder.

Question 48

Treatment of Tourette disorde if socially disabling

Answer 48

haloperidol 0.25 mg (0) nocte, very gradually increasing to 2 g (max.) daily? or clonidine 25 mcg (o) bd for 2 weeks, then 5075 mcg bd

Question 49

is an acute unilateral lower motor neurone paresis or paralysis, is the commonest cranial neuropathy.

Answer 49

Idiopathic facial (7th nerve) palsy, classic type is Bell palsy,

Question 50

is due to infection with herpes zoster causing facial nerve palsy, vesicles may be seen on the psilateral ear. Bell phenomenon - when closing the eye it turns up under the half-closed lid Abrupt onset (can worsen over 2-5 days) Weakness in the face (complete or incomplete) Preceding pain in or behind the ear Impaired blinking

Answer 50

Ramsay-Hunt syndrome,

Question 51

Ramsay-Hunt syndrome is associated with what diseases

Answer 51

herpes simplex virus (postulated) diabetes mellitus hypertension thyroid disorder, e.g. hyperthyroidism

Question 52

Management Ramsay-Hunt syndrome

Answer 52

prednisolone 1 mg/kg (o) up to 75 mg (usually 60 mg) daily in the morning for 5 days (start within 48 hours of onset) Adhesive patch or tape over eye if corneal exposure (e.g. windy or dusty conditions, during sleep) Artificial tears if eye is dry and at bedtime Patient education and reassurance