Neuro Flashcards
Which type of muscle fibre is rich in myoglobulin
Type I
What does myoglobulin do
supplies oxygen to your muscles
What are the main symptoms of myopathy
Proximal weakness
Hypotonia
Wasting (or fatty infiltration)
BUT reflexes are preserved
What would be raised on blood tests in myopathy
Creatine phosphokinase
What is a myopathic unit on a EMG?
Small, short-duration, spiky polyphasic units
What is the inheritance pattern of myotonic dystrophy type I
Autosomal dominant
What is the type of mutation in myotonic dystrophy type I
Expanded trinucleotide repeat (CTG)
Within the myotonic protein kinase gene on chromosome 19
Symptoms of myotonic dystrophy
Progressive muscle weakness, particularly in upper limbs
Myotonia
Many other symptoms (cataracts, frontal balding, dysarthria and dysphagia..)
Fatigue
Is myotonic dystrophy inheritance worse through females or males
Females, with anticipation.
What are the characteristics of metabolic myopathies
Exercise intolerance/pain/fatigue
Sometimes have periodic paralyses with high or low potassium
What is the inheritance pattern of periodic paralysis channelopathies
Autosomal dominant
What is the most common age range for inflammatory myopathies?
Middle age, classically:
Polymyositis and dermatomyositis (30/40 yr old woman)
Inclusion body myositis: over 50s male
What is the management for polymyositis and dermatomyositis
Corticosteroids +/- immunosuppression and removal of tumour if present
What is the treatment for inclusion body myositis
Supportive management (no treatment)
What is the definitive investigation for muscle disease
Muscle biopsy (determines fibre type, inflammation, dystrophic and histochemical changes)
What is the classical gait in Duchenne
Waddling gait
Where does pseudo-hypertrophy happen in Duchenne’s
Calves
What is a common cause of death in Becker’s muscular dystrophy
Cardiac arrhythmias
Characteristics of myopathies due to thyrotoxicosis
Shoulders mainly
Brisk reflexes
+/- fasciculations and atrophy
What drugs can cause myopathy
Steroids
Satins
Potassium-losing drugs
(also: chloroquine, amiodarone, doxorubicin, Zidovudine)
What type of virus is polio virus
Enterovirus
What percentage of patients with poliomyelitis have paralysis
0.1%
Symptoms of poliomyelitis
Abortive: self limiting GI and resp symptoms
Non-abortive: features of abortive with meningism. Complete recovery
Paralytic: abortive symptoms -> non-abortive -> myalgia and asymmetrical paralysis. Sometimes resp failure but usually lower limbs in children. Can cause bulbar involvement.
How is poliomyelitis distinguished from GBS
Polio doesn’t have sensory symptoms and has asymmetry
CSF findings in poliomyelitis
Same as viral meningitis
Raised protein
Increased no. lymphocytes
Normal glucose
Increased numbers of polymorphs initially
Prognosis for polio
If there is good resp support, often low mortality and good improvement in muscle power. In affected limbs in children, bone growth is retarded, resulting in a wasted, shortened limb
Which type of neurofibromatosis has peripheral predominance
Type I
Characteristics of neurofibromatosis type 1
Neurofibromas along peripheral nerves
Café-au-lait spots (>5 or >1.5cm)
Cutaneous fibromas
Scoliosis
Endocrine abnormalities
Neural tumours
Characteristics of neurofibromatosis type 2
Vestibular schwannomas (previously called acoustic neuromas)
Other intracranial and intraspinal tumours
Wernicke-Korsakoff syndrome has a deficiency of which vitamin
B1 (thiamine)
What are the two phases of Wernicke-Korsakoff’s
Acute phase: Wernicke’s encephalopathy
Chronic phase: Korsakoff’s psychosis
Triad symptoms of Wernicke’s encephalopathy
Ocular signs
Ataxia
Confusion
(e.g. nystagmus, broad-based gait, amnesia)
What symptom is classical of Korsakoff’s psychosis
Confabulation
(poor short-term memory so makes it up)
What are the symptoms of Horner’s syndrome
Unilateral Miosis (constricted pupil)
Partial ptosis
Ipsilateral anhidrosis
Causes of Horner’s syndrome
Lesion at any level of the sympathetic pathway (e.g. tumour, stroke, disease, aneurysm, dissection)
What is an acute painful Horner syndrome until proven otherwise?
Dissection of the ipsilateral internal carotid artery
What does Normal Pressure Hydrocephalus cause
Reversible dementia
Triad: Urinary incontinence, dementia and gait abnormalities
Can look like Parkinson’s but doesn’t respond to L-Dopa
Over several months
What is the management for Normal Pressure Hydrocephalus
Ventricle-peritoneal Shunt
What causes Normal Pressure Hydrocephalus
Impaired CSF reabsorption
Triggered by a head injury or bleed.
Symptoms of acute hydrocephalus
Nausea
Vomiting
Diplopia
Fluctuating conscious level
Papilloedema
Ataxia of gait
Symptoms of headache of raised ICP
Headache aggravated by: bending, coughing, straining, in the morning. Getting worse and worse
With:
Vomiting
Visual obscurations
Progressive focal neuro signs
Papilloedema
What is tonsillar herniation
When a space-occupying lesion causes the cerebellar tonics to herniate through the foramen magnum causing brainstem compression and death - ‘coning’
What are the three subtypes of cerebral palsy
Spastic (90%)
Dyskinetic
Ataxic
What percentage of cerebral palsy is due to hypoxic-ischaemic encephalopathy
10%
How does cerebral palsy present in infancy
Abnormal tone and posture
Delayed motor milestones
Feeding difficulties
What are the symptoms of anterior spinal artery syndrome
Segmental pain
Urinary retention
Flaccid -> spastic paraparesis (corticospinal)
Loss of pain and temperature sensation below lesion (spinothalamic)
areflexia -> hyperreflexia
What are the three sections of the GCS
Eye opening
Verbal responses
Motor responses
GCS: eye opening scale
1-4 AVPU
GCS: verbal response scale
1- None
2 - Incomprehensible sounds
3 - Inappropriate words
4 - Disorientated speech
5 - Orientated speech
GCS: motor response scale
1- None
2 - Extensor response to pain (Decerebrate response)
3 - Flexor response to pain (Decorticate response)
4 - Flexion/withdrawal to pain
5 - Localisation of painful stimulus
6 - Obeys commands
What is ADEM
Acute disseminated encephalomyelitis
Demyelination within the CNS
Presents following a viral infection or vaccination
Can cause encephalitis
What are the three places that oculomotor disorders can arise from
Supranuclear (cerebral hemispheres)
Internuclear (brainstem)
Infranuclear (nerves supplying muscles of the eye)
What are the features of Creutzfeldt-Jakob disease
Memory problems
Behavioural changes
Poor coordination
Visual disturbances
(rapidly progressing dementia +/- myoclonus = always assume prion disease until proven otherwise)
What is the classical CT appearance for cerebral abscess
Ring enhancing lesion
Central area of low density
Surrounding area of oedema
Which is the first line treatment for absence seizures?
Sodium valproate
What is the first line treatment for focal seizures?
Carbemazepine
What is first line treatment for Bell’s palsy?
Prednisolone
What is the first line treatment for Guillain Barré syndrome?
IVIG
What is first line treatment for Ramsey-Hunt syndrome?
Prednisolone and acyclovir
What is Ramsey-Hunt syndrome?
Shingles in the facial nerve
In what order does diabetic polyneuropathy happen? (the different sensory modalities)
First numbness and tingling
Then proprioceptive balance issues
Then motor function
What can Vit B12 deficiency cause?
Peripheral neuropathy
Subacute combined degeneration of the cord
Optic neuropathy
Dementia/encephalopathy
What can unusually cause Horner’s syndrome?
T1 nerve root involvement
What is a Pancoast tumour
Squamous cell lung carcinoma at the apex of the lung - often causes brachial neuropathy
How would you treat fatigue as a symptom of MS?
Modafinil or Amantadine
How could you treat tremor in MS?
Clonazepam
How could treat pain symptoms in MS?
Amitryptiline
How could you treat spasticity in MS?
Baclofen
What is Meralgia Paraesthetica
Compression of the lateral cutaneous nerve of the thigh under the inguinal ligament
What are the symptoms of Meralgia Paraesthetica
Shooting pains on the outside thigh
Numbness and painful tingling over area, highly sensitive to light touch and heat
What is Lhermitte phenomenon in MS?
Shooting pains along the limbs following flexion of the neck
What does the segmental medullary artery supply?
Branches of the cervical parts of the vertebral arteries. Supply the surface and the inside of the spinal canal at each segmental level
How many anterior and posterior spinal arteries?
One anterior, one posterior
What is Jacksonian march?
Twitching starts in the limb then ascends to trunk and then to arm (part of epilepsy)
What does spinal MND look like?
Mixed picture with UMN and LMN signs
What does primary lateral sclerosis MND look like?
Mostly UMN signs
What does progressive muscular atrophy MND look like?
Mostly LMN signs
In any major bleed with Warfarin, what do you give for management
Stop warfarin
Vitamin K 5mg IV
Prothrombin Complex concentrate
What are the classic findings on an MRI head and spine for MS?
Juxtacortical and paraventricular lesions as well as spinal cord lesions
What is autonomic neuropathy a common complication of?
Poorly controlled diabetes
Symptoms e.g. gastroparesis, postural hypotension
What is Charcot Marie Tooth?
Hereditary motor and sensory peripheral neuropathy
What is the most common genetic mutation Charcot Marie Tooth?
Mutation in the peripheral myelin protein 22 (PMP22) gene
What is the different between Charcot Marie Tooth Type 1 and Type 2?
Type 1 is demyelinating (PMP22)
Type 2 is axonal