JM Chapter 22 Flashcards
combined LMN muscle atrophy plus
UMN hyper-reflexia, leading to progressive spasticity. This is the most common typ
amyotrophic lateral sclerosis (Lou Gehrig disease)
- wasting beginning in the distal muscles; widespread
fasciculation
progressive muscle atrophy
Results in wasted I tongue, weakness of chewing and swallowing, and
of facial muscles
progressive bulbar (LMN) palsy and pseudobulbar palsy (LMN lesions in the brain stem
motor nuclei
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.
Resting tremor-Parkinsonian
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
Action or postural tremor
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.
Intention tremor (cerebellar disease)
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.
Flapping (metabolic tremor)
Triad of features
Positive family history
Tremor with little disability
Normal gait
Essential tremor
Pakinsonsm
Positive frontal lobe signs,
Pakinsonsm
grasp and glabellar taps
is the gold standard for therapy
Pakinsonsm
L-dopa
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
MULTIPLE Sclerosis
Inv of Multiple sclerosis
Lumbar puncture
MRI
In outpatient setting the tx for Multiple Sclerosis for moderate relapse
PREDNISOLONE
For mild relapse, tx for multiple sclerosis is
Rest and assurance
Severe relapse of attacks of MS tx is
Methylprednisolone 1f in 200 ml, saline by slow IV infusion daily for 3 days
What isbthe tx of spasticity in MS
Physiotheraoy
baclofen 10-25mg note
Alternative: Dantroleme
Tx for Paroxysmal eg neuralgia
Carbamazepine or Gabapentine
Classic glove and stocking sign
Peripheral Neuropathy
Reflexes absent
Both proximal and distal muscles affected
Csf protein elevated
GBS
Tx of GBS
Physiotherapy
Tracheostomy and artificial ventilation
Plasma exchange or IV Ig (0.4 G/KG DAY FOR 5 DYS)
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.
Charcot-Marie-Tooth syndrome
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.
Familial periodic paralysis
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.
Myasthenia gravis
Clinical features
Myasthenia gravis
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
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
Myasthenia gravis
Myasthenia gravis
Diagnosis
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)
is recommended early for generalised myasthenia, especially in all younger
patients with hyperplasia of the thymus, even if not confirmed preoperatively.
Thymectomy
useful for acute crisis or where temporary improvement is required or
patients are resistant to treatment
Plasmapheresis
first-line
Pharmacological agents: for Myasthenia Gravis
Pyridostigmine, Neostigmine or
Distigmine),
useful for all grades of MG (should be introduced slowly)
corticosteroids
combination of ocular and facial weakness
Smiling may have a characteristic snarling quality.
MYASTHENIA Gravis
ptosis, eye facing ‘down and out’, dilated pupil, sluggish light reflex
3rd cranial nerve palsy-
ptosis, miosis (constricted pupil), ipsilateral loss of sweating
Horner syndrome
progressive external ophthalmoplegia or limb weakness, induced
by activity- no pupil involvement
Mitochondrial myopathy
ptosis and diplopia, no pupil involvement
Myasthenia gravis
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.
Dystonia
can induce a severe generalised dystonia (e.g. oculogyric
crisis)
Neuroleptic and dopamine receptor blocking agents (e.g. L-dopa,
metoclopramide)
treated with benztropine 1-2 mg IM or IV.8
is a focal dystonia of the muscles around the eye resulting in uncontrolled
blinking, especially in bright light.
.Blepharospasm
FOCAL DYSTONIA
affects the jaw, tongue and mouth, resulting in jaw grinding
movements and grimacing. Proper speech and swallowing may be disrupted
Oromandibular dystonia
combination of blepharospasm and oromandibular dystonia.
Meige syndrome
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
Hemifacial spasm
are all occupational focal
dystonias of the hand and/or forearm initiated by performing these skilled acts,
Writer’s cramp, typist’s cramp, pianist’s cramp,
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
Cervical dystonia
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.
Laryngeal or spastic dystonia
The current treatment for focal or segmental (spread to an adjacent body region) dystonias is
localised injection of purified botulinum
Motor and vocal tics are a feature of
Tourette disorder.
Treatment of Tourette disorde if socially disabling
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
is an acute unilateral lower motor neurone paresis or
paralysis, is the commonest cranial neuropathy.
Idiopathic facial (7th nerve) palsy,
classic type is Bell palsy,
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
Ramsay-Hunt syndrome,
Ramsay-Hunt syndrome is associated with what diseases
herpes simplex virus (postulated)
diabetes mellitus
hypertension
thyroid disorder, e.g. hyperthyroidism
Management
Ramsay-Hunt syndrome
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
