conditions Flashcards
(173 cards)
1
Q
Do facial nerve palsies usually have unilateral or bilateral facial weakness?
A
Unilateral
2
Q
Where does the facial nerve exit the brainstem?
A
At the cerebellopontine angle
3
Q
What bone does the facial nerve pass through between the brainstem and the face?
A
The temporal bone
4
Q
What gland does the facial nerve pass through between the brainstem and the face?
A
The parotid gland
5
Q
How many branches does the facial nerve split into?
A
5
6
Q
What are the branches that the facial nerve splits into?
A
Temporal, Zygomatic, Buccal, Marginal mandibular, Cervical
7
Q
What motor function does the facial nerve have?
A
Supplies the muscles of facial expression, the stapedius in the inner ear and the posterior digastric, stylohyoid and platysma muscles in the neck.
8
Q
What sensory function does the facial nerve have?
A
carries taste from the anterior 2/3 of the tongue.
9
Q
What parasympathetic function does the facial nerve have?
A
parasympathetic supply to the submandibular and sublingual salivary glands and the lacrimal gland
10
Q
Will the forehead be spared in an UMN lesion or a LMN lesion
A
UMN lesion
11
Q
In what conditions do unilateral UMN lesions occur in?
A
Cerebrovascular events and tumours
12
Q
In what conditions do bilateral UMN lesions occur in?
A
Pseudobulbar palsies and MND
13
Q
Is Bell’s Palsy an UMN or LMN facial nerve palsy?
A
Unilateral LMN
14
Q
What causes Bell’s Palsy?
A
It is idiopathic
15
Q
What is the disease progression of Bell’s Palsy?
A
Begins suddenly and worsens over 48 hours
16
Q
What are the symptoms of Bell’s Palsy?
A
Dryness or eye, drooping of eyelid, disappearance of nasolabial fold, drooping of mouth, loss of taste anterior 2/3 of tongue, difficulty with eating/drinking and speech, posterior auricular pain, hyperacusis
17
Q
What treatment can you give someone with bell’s palsy within 72 hours?
A
prednisolone 50 mg for 10 days
18
Q
What is ramsay-hunt syndrome?
A
unilateral LMN facial nerve palsy
19
Q
What is ramsay-hunt syndrome caused by?
A
varicella zoster virus
20
Q
What symptoms make you think of ramsay-hunt syndrome rather than bell’s palsy?
A
painful and tender vesicular rash in the ear canal, pinna and around ear on affected side
21
Q
What treatment should you give for ramsay-hunt syndrome?
A
prednisolone and aciclovir
22
Q
What is neurofibromatosis?
A
Genetic condition that causes neuromas to develop in the nervous system
23
Q
How many types of neurofibromatosis are there?
A
2
24
Q
Which is more common-neurofibromatosis type 1 or 2?
A
1
25
What chromosome is NF1 gene found on?
Chromosome 17
26
What does the NF1 gene code for?
neurofibromin
27
What type of protein is neurofibromin?
tumour suppressor protein
28
What is the type of inheritance in mutations of NF1?
Autosomal dominant
29
What is the acronym for the symptoms of Type 1 Neurofibromatosis?
CRABBING
30
What does C stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Cafe au lait spots
31
What does R stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Relatives with NF1
32
What does A stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Axillary or inguinal freckles
33
What does BB stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
34
What does I stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Iris homortomas are yellow brown spots in the iris
35
What does N stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Neurofibromas or 1 plexiform neurofibroma
36
What does G stand for in the acronym for the symptoms of Neurofibromatosis type 1?
Glioma of the optic nerve
37
How many cafe au lait spots are needed for them to be a symptom of neurofibromatosis?
6 or more
38
How many of the CRABBING symptoms of neurofibromatosis are needed for a diagnosis?
2/7
39
What are the possible investigations for neurofibromatosis?
Clinical, genetic testing, x-rays, CT/MRI
40
What are the complications of neurofibromatosis?
Migraines, Epilepsy, Behavioural and learning difficulties, brain tumour, spinal cord tumours, Scoliosis of the spine, Renal artery stenosis, increased cancer risk, leukaemia, GI stromal tumour, vision loss, malignant peripheral sheath tumour
41
Where is the gene coding for NF2 found?
Chromosome 22
42
What does NF2 code for?
merlin
43
What is the protein merlin?
Tumour suppressor protein that is particularly important in schwann cells
44
What do mutations in the NF2 gene lead to?
Development of schwannomas
45
What type of inheritance is neurofibromatosis type 2?
Autosomal dominant
46
What type of neuroma is most commonly associated with NF2?
Acoustic neuromas
47
What are the symptoms of acoustic neuromas?
Hearing loss, Tinnitus, Balance problems
48
What do bilateral acoustic neuromas almost certainly indicate?
Neurofibromatosis type 2
49
What is narcolepsy?
Ability to regulate sleep-wake cycles is impaired
50
What transmitters are produced by the neurons in the hypothalamus that stabilise wakefulness?
Orexin A (hypocretin 1) and orexin B (hypocretin 2)
51
What are the 5 key symptoms of narcolepsy?
Daily sleepiness
Cataplexy
Inability to move at start or end of sleep
Vivid hallucinations
Fragmented sleep
52
What are the two ways to help diagnose narcolepsy?
Polysomnography
Multiple sleep latency test
53
What are the treatments to help with narcolepsy?
Stimulants and antidepressants
54
What is epilepsy?
Recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting in seizures
55
How many seizures do you need to have to be defined as epileptic?
2 or more unprovoked seizures, occurring >/= 24 h apart
56
What is the difference between an epileptic seizure and a non-epileptic one?
Non-epileptic seizures do not involve abnormal electrical activity in the brain
57
What are the causes of epilepsy?
Idiopathic, Cortical scarring, Tumour, Stroke, TB, Alzheimer's, Alcohol withdrawal, neurocutaneous syndromes, neurodegenerative disorders
58
What are the causes of a non-epileptic attack?
Psychogenic, Vasovagal, orthostatic, cardiac arrhythmia
59
What are the risk factors for epilepsy?
FH
Prematurity
Alzheimer's
Drugs
Stroke/infection/Tumour
60
What is vasovagal syncope?
LOC due to to abnormal autonomic response and temporary decrease in blood supply to the brain
61
What is the difference between generalised and focal seizures?
Generllised: no warning, always LOC and discharge from both hemispheres
Focal: discharge from one part of the hemisphere, may be preceding aura, may or may not have LOC
62
What is the tonic phase of a seizure?
Stiffening, increased tone, hold breath
63
What is the clonic phase of a seizure?
Rhythmical jerking, irregular breathing, tongue-biting, incontinence, post-ictal drowsiness/confusion
64
What is a generalised tonic-clonic seizure?
loss of consciousness and tonic and clonic
65
What is the 1st line treatment for generalised tonic-clonic seizures?
Sodium Valporate
66
What is the 2nd line treatment for generalised tonic-clonic seizures?
Lamotrigine or carbamazepine
67
In what part of the brain do focal seizures tend to happen in?
Temporal lobes
68
What do focal seizures present with?
Hallucinations, Memory flashbacks, Deja vu, Strange things on autopilot
May or may not have LOC
69
What is the 1st line management for focal seizures?
Carbamazepine or lamotrigine
70
What is the 2nd line management for focal seizures?
Sodium valporate or levetiracetam
71
Who do absence seizures typically occur in?
Children
72
What is the 1st line treatment for absence seizures?
Sodium valporate or ethosuximide
73
What are atonic seizures?
Brief lapses in muscle tone and can have jerking
74
What might atonic seizures in children indicate?
Lennox-Gastrut syndrome
75
What is the 1st line management for atonic seizures?
Sodium Valporate
76
What is the 2nd line management for atonic seizures?
Lamotrigine
77
What is a myoclonic seizure?
Sudden brief muscle contractions (occasionally drop things)
78
What are myoclonic seizures typically part of?
Juvenile myoclonic epilepsy
79
What is the 1st line management for myoclonic seizures?
Sodium valporate
80
What is the 2nd line management for myoclonic seizures?
Lamotrigine, topiramate or levetiracetam
81
What are infantile spasms also known as?
West syndrome
82
What is the management for infantile spasms?
Prednisolone and vigabatrin
83
What investigations are done for epilepsy?
EEG
MRI
ECG
Bloods to rule out metabolic disturbances
84
What are the side effects of sodium valporate?
Teratogenic
liver damage and hepatitis
hair loss
tremor
Weight gain
85
What are side effects of carbamazepine?
Agranulocytosis
Aplastic anaemia
Induces P450 system so many drug interactions
86
What are the side effects of phenytoin?
Folate and vit D deficiency
Megaloblastic anaemia
Osteomalacia
87
What are the side effects of ethosuximide?
Night tremors
Rashes
88
What are the side effects of lamotrigine?
Stephens-Johnson syndrome
Leukopenia
Headaches
Insomnia
89
What is status epilepticus?
Seizure lasting longer than 5 min or repeated seizures with no recovery of consciousness in between
90
What is the immediate management for status epilepticus?
IV Benzodiazepine
91
What is multiple sclerosis?
Immune-mediated, Chronic, inflammatory, demyelinating disease of CNS
92
What type of hypersensitivity reaction is MS?
Type IV
93
What are the risk factors for MS?
Female
HLA-DR2
Infections
Vitamin D deficiency
94
What are the 4 types of MS?
Primary Progressive
Secondary Progressive
Relapsing-Remitting
Progressive Relapsing
95
What is the most common type of MS?
Relapsing-Remitting
96
What are the differentials for MS?
Neuroinflammatory diseases: Vasculitis, Neurosarcoidosis, Neuromyelitis optica, Anti-MOG disease
Infections: Lyme disease, syphilis, HIV
Cerebrovascular disease: TIA/Stroke
MND
97
What are the clinical presentations of MS?
Optic nerves: Optic neuritis (visual blurring/vision loss, pain on movement, decreased colour differentiation
Brainstem: diplopia/facial weakness or pain/ internuclear opthalmoplegia
Spinal cord: Bladder/Bowel/sexual dysfunction
Dorsal column: Tingling, parasthesia and loss of proprioception (useless hand syndrome)
Corticospinal tracts: Paraparesis, UMN defecit (increased tone + hyperreflexia)
Cerebellar signs: Intention tremor, nystagmus, vertigo, dysarthria
Long-term complications: Excessive fatigue, depression, memory loss
98
What is the common trio of symptoms in MS?
Charcot's neurologic triad: Dysarthria, nystagmus, intention tremor
99
What criteria is used for MS diagnosis?
McDonald's criteria: a) 2 characteristic episodes of neuro dysfunction separated in time b) deficits represent lesions in at least 2 different areas of CNS
100
What bloods do you do for MS and what might you find?
FBC, U &Es, LFT, ESR, TFT, glucose, calcium, B12, Syphilis, lyme disease and HIV serology to rule out DDx
Autoimmune screen including anti-MOG Ab and aquaporin 4 Ab
101
What is gold standard scan for MS and what would you see?
MRI: multiple hyperintense lesions in periventricular white matter
102
What might you do in MS if MRI is inconclusive?
LP and CSF
103
What might you see in CSF in MS?
Moderately increased or normal protein
Moderately raised or normal WBCs
Oligoclonal bands on electrophoresis (not always present in MS)
104
What sign is specific to MS?
Lhermitte's sign: electric shock down back and radiates to limbs when bending neck forward
105
What is the management for an MS relapse?
High dose corticosteroids: methylprednisolone
106
What is the long-term management for MS?
Immunotherapies: IFN-beta (immunomodulator), immunosuppressants ( dimethyl fumarate)
107
What is the management for symptoms of MS?
Fatigue- amantadine
Neuropathic pain- TCAs
Spasticity- baclofen, BZDs
Depression- CBT, SSRIs
Bladder/bowel dysfunction- anticholinergics, laxatives
108
What is the prognosis of MS?
life expectancy 6-10y less than normal
109
What is MND?
Degenerative disease of UMNs and LMNs in the spinal cord and cortex
110
What are the general symptoms of MND?
Progressive weakness but no sensory deficit (dysphagia, dysphonia, breathing difficulties)
Mixed UMN and/or LMN signs (spasticity/fasciculations)
111
What investigations are done for MND and what would you see?
EMG: active denervation, chronic neurogenic changes
Bloods: rule out other causes; B12,folate
Spinal MRI: rule out myelopathy/radiculopathy
112
What are the 4 subtypes of MND in order of commonity?
Amyotrophic Lateral Sclerosis
Progressive Bulbar Palsy
Progressive muscular atrophy
Primary Lateral Sclerosis
113
What are the Symptoms and associated signs of ALS?
UMN and LMN
Focal muscle weakness
Fasciculations + spastic paresis +/- fronto-temporal dementia
114
What are the symptoms and associated signs of Progressive Bulbar Palsy?
UMN and LMN
Dysarthria, dysphagia (choking/regurgitation)
Tongue fasciculations
115
What are the symptoms and associated signs of progressive muscular dystrophy?
LMN
Limb weakness- gradually spreads from distal to proximal
Fasciculations + painless wasting
116
What are the symptoms and associated signs of primary lateral sclerosis?
UMN
Slowly progressing tetraparesis
+ pseudobulbar palsy
117
What is the prognosis of MND?
Life expectancy shortened to 6 months to 3 years from onset of symptoms
118
What is the management for MND?
Muscle cramps- quinine, baclofen
Disease-modifying medication: Riluzole, slows progression
119
What is the meaning of neuropathy?
Pathological process affecting a peripheral nerve/nerves (LMN ONLY)
120
What are the most common causes of neuropathies?
Diabetes
Vit B deficiency
Drugs/toxins
Alcohol
Demyelination
Idiopathic
121
What are the categories of neuropathies?
Demyelination
Axon degeneration
Compression
Infarction
Infiltration
122
What are the demyelination causes of neuropathies?
Antibodies against myelin- RA, CTDs, myxoedema
Inflammatory- Guillain-Barre, chronic inflammatory demyelination
Inherited- Charcot-Marie-Tooth Type 1
123
What are the axon degeneration causes of neuropathies?
Metabolic- DM, B12/folate deficiency, CKD
Toxins- alcohol, lead, radiation, drugs
Antibodies against axons: RA, CTDs
Inherited- Charcot-Marie-Tooth type 2
124
What are the infiltration causes of neuropathies?
Granuloma, Sarcoid, cancer
125
What are the investigations and results in neuropathies?
Bloods: FBC, ESR, U&Es, LFTs, glucose, B12, folate
Paraproteins & antibodies: ANCA, VDRL test
Myeloma screen
EMG & NCS
LP: raised protein in GBS
Peripheral nerve biopsy
126
If someone has a neuropathy that has symmetrical weakness and sensory loss what could it be?
CIDP
GBS
127
If someone has a neuropathy that has symmetrical sensory loss without weakness what could it be?
Toxins
Metabolic disorders
Hereditary (CMT)
B12 deficiency
Friedreich's ataxia
Cervical myelopathy
128
If someone has a neuropathy that has symmetrical weakness without sensory loss what could it be?
CMT
Spinal muscular atrophy
129
If someone has a neuropathy that has asymmetrical weakness and sensory loss what could it be?
Vasculitis
Infections (lyme disease, sarcoid)
HNPP
Single nerve/root damage
Polyradiculopathy/plexopathy
130
If someone has a neuropathy that has asymmetrical weakness without sensory loss what could it be?
Multifocal motor neuropathy
131
What is the most common acute polyneuropathy?
Guillain-Barre syndrome
132
What is the pathogenesis of Guillain-Barre syndrome?
viral infection triggers inflammatory response & autoimmune demyelination
133
What are the symptoms of Guillain-Barre syndrome?
1-3w after viral infection e.g. gastroenteritis/strep
Ascending paralysis- starts in legs
Loss on tendon reflexes
Facial weakness
Bulbar weakness- dysarthria, dysphagia
Respiratory muscle weakness
Autonomic features e.g. postural hypotension, arrhythmias
134
What are the investigations and results in Guillain-Barre syndrome?
NCS: slowed (demyelination)
LP: raised protein (usually normal cell counts)
135
What is the management of Guillain-Barre syndrome
High dose IV immunoglobulin/plasma exchange
136
What are the complications of Guillain-Barre syndrome?
Pneumonia
SIADH
Pseudo-obstruction
137
What is Miller-Fisher syndrome?
Similar to GBS and can present on diarrhoeal illness
Classic triad of opthalmoplegia, areflexia, ataxia
138
What is the pathogenesis and onset of chronic inflammatory demyelinating polyradiculopathy?
antibody-mediated demyelination
over months
139
What are the symptoms and management of chronic inflammatory demyelinating polyradiculopathy?
Progressive/relapsing proximal and distal weakness and sensory loss
Immunosupression
140
What is the pathogenesis and onset of multifocal motor neuropathy?
antibody-mediated multifocal demyelination
over months
141
What are the symptoms of multifocal motor neuropathy?
progressive asymmetrical distal limb weakness (starts hands/feet)
142
What is the pathogenesis of Charcot-Marie-Tooth?
Genetic condition causing axonal degeneration (onset in children or 40s)
143
What are the symptoms of Charcot-Marie-Tooth?
Progressive symmetrical numbness, tingling & weakness (starting in hands/feet and spreads proximally)
Clawed toes, high arches, foot drop, muscle wasting
Family history potentially
144
What is the specific investigation for Charcot-Marie-Tooth?
NCS= slowed due to demyelination and axonal damage, rules out diabetes
145
What are the symptoms of diabetic neuropathy?
Symmetrical distal sensory loss
146
What is the pathogenesis and symptoms of B12 deficiency?
degeneration of dorsal & lateral white matter of spinal cord
Sensory ataxia & subacute combined degeneration of the spinal cord
Progressive weakness + parasthesia
147
What is trigeminal neuralgia?
Facial pain experienced along the distribution of the trigeminal nerve
148
What are the RF for trigeminal neuralgia?
Increased age
Multiple sclerosis
149
What are the differentials of trigeminal neuralgia?
Dental pain
Mandibular osteomyelitis
Temperomandibular joint syndrome
Migraine/ cluster syndrome
Temporal arteritis
Post-herpetic neuralgia
150
What is the aetiology of trigeminal neuralgia?
Idiopathic compression of trigeminal nerve (often by vascular loop of superior cerebellar artery) or demyelination
151
What is the clinical presentation of trigeminal neuralgia?
Facial pain- sharp/stabbing episodes +/- constant background of pain
No associated neurological deficit
152
What is the investigation for trigeminal neuralgia?
CT/MRI
153
What is the management for trigeminal neuralgia?
1st line: anticonvulsants (carbamazepine/oxcarbazepine)
2nd line: gabapentin, pregabalin, amitriptyline, nortriptyline, lamotrigine
Medication resistance: microvascular decompression/ablative surgery
154
what are the differentials for trigeminal neuralgia?
Dental pain
Mandibular osteomyelitis
Temporomandibular joint syndrome
Migraine/cluster headache
Temporal arteritis
Post-hepatic neuralgia
155
What are the RF for trigeminal neuralgia?
Increased age
Multiple sclerosis
156
What is myasthenia gravis?
An autoimmune disorder of the neuromuscular junctions
157
What is the pathogenesis of myasthenia gravis?
autoantibodies against the post-synaptic ACh receptor block neuromuscular junction
158
What is myasthenia gravis associated with?
thymic hyperplasia and Thymoma
159
What are the symptoms of myasthenia gravis?
Weakness and fatiguability that worsens throughout the day
Of proximal (limits walking), bulbar (dysphagia/dysarthria), ocular (fluctuating diplopia & pstosis- worsens with prolonged looking up) and respiratory muscles (breathing difficulties)
No LMN/UMN signs
160
What are the investigations of myasthenia gravis?
Serum antibodies: anti-ACh receptor, anti-Musk
EMG: repeated nerve stimulation- decreasing response (can be diagnostic)
Imaging: CT for thymus masses
161
What is the management for myasthenia gravis?
Oral anticholinesterase: prolong ACh action (decreased breakdown) e.g. pyridostigmine
Immunosuppression: steroids +/- disease modifying drugs (azathioprine, methotrexate, mycophenolate)
Thymectomy if hyperplasia/thymoma
162
How do you manage an acute crisis of myasthenia gravis? (Sudden worsening of weakness +/- respiratory muscles)
Rapid plasmaphoresis &/IV immunoglobulins
Ventilation
NG feeding
163
What is the pre-synaptic NMJ disorder associated with small cell carcinoma of the lung?
Lambert-Eaton myasthenic syndrome
164
What is lambert-eaton myasthenic syndrome?
Pre-synaptic NMJ disorder associated with small cell carcinoma of the lung
165
How often is myasthenia gravis monitored?
Lifelong every 4 months
166
What is essential tremor?
Involuntary, rhythmical shaking movement most commonly affecting the upper limbs
167
What are the clinical presentations of essential tremor?
Progressive tremor:
- Bilateral and symmetrical (4-12Hz)
- Usually affects hands/arms, but can also affect voice and head
- Present during action and when holding a position
- Not usually present at rest (but can be when very severe)
- Improves with alcohol
No associated neurological symptoms
168
What are the investigations for essential tremor?
Examination- including Archimedes' spiral
CT/MRI brain if neurological symptoms
TFTs- rule out hyperthyroidism
DAT scan- if tremor is particularly asymmetrical and/or signs of parkinsons
169
What is the management for essential tremor?
Psychological/functional impact:
1st: propranolol or primidone
2nd: gabapentin or topiramate
170
What are the differentials for essential tremor?
Parkinson's disease
Dystonic tremor
Psychogenic tremor
Drug-induced tremor (lithium, antipsychotics, caffeine)
171
What RFs are associated with essential tremor?
Increasing age
FHx
172
What is archimedes' spiral?
by asking a patient to draw a spiral, the frequency, amplitude and direction of tremor can be assessed
173
How is treatment resistant essential tremor be managed?
Deep brain stimulation
