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PACES Revision Cards > Neurology Flashcards > Flashcards

Neurology Flashcards

1
Q

Neurological examples of trinucleotide repeat disorders?

A

Huntington’s Disease (CAG)
Spinocerebellar Ataxia Disorders (CAG)
Friedrich’s Ataxia (GAA)
Myotonic Dystrophy (CTG)

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2
Q

MRC Grading of Power

A

5 = full power against resistance

4 = reduced power, able to move against some resistance

3 = able to move against gravity; NOT against resistance

2 = unable to move against gravity; some movement if gravity eliminated

1 = visible flicker of muscle contraction

0 = no muscle contraction or movement

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3
Q

UMN Features

A

?Contractures
Increased tone
+/- Clonus
Brisk reflexes
Upgoing (extensor) plantar reflexes

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4
Q

Localisation of UMN

A

Brain (including parasagittal sinus) to spinal cord (terminates at L1-L2, conus medullaris)

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5
Q

Spasticity vs Rigidity

A

SPASTICITY = increased tone, velocity dependent (faster you move the limb, the greater increase in tone)
= lesion of pyramidal tract

RIGIDITY = increased tone; velocity INDEPENDENT (same increased tone whether you move the limb fast or slow)
= lesion of extrapyramidal tract

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6
Q

Pyramidal vs Extrapyramidal Tracts

A

PYRAMIDAL TRACT = Cerebral cortex to spinal cord via medullary pyramids = conscious control of muscles from the cerebral cortex

EXTRAPYRAMIDAL TRACT = Originate in the brainstem, carrying motor fibres to the spinal cord = involved in the control of movement and coordination

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7
Q

Differentials for UMN pattern of limb weakness

A

Differential will be guided by timing of onset of symptoms

Vascular - ischaemic stroke, haemorrhage

Infection - Encephalitis, meningitis, cerebral abscess, spinal abscess, transverse myelitis

Trauma

Demyelinating - MS, NMO

Compressive lesion e.g. malignancy, prolapsed vertebral disc

Cerebral palsy

Hereditary e.g. hereditary spastic paraparesis

Autoimmune e.g. AI encephalitis

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8
Q

Differentials for SPASTIC HEMIPARESIS

A

Contralateral stroke
- ACA affects legs > arms (face often spared)
- MCA affects arms > legs
Space-occupying lesion
Cord Compression e.g. Tumour
Intrinsic cord disease e.g. MS, tumour, vascular
Brown-Sequard syndrome

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9
Q

Presenting SPASTIC HEMIPARESIS

A

If acute onset - concerned presenting as a stroke

To complete my examination I would like to…
1) Fundoscopy
2) Full cranial nerve examination and neurological examination
3) Cardiovascular examination
4) Speech and swallow assessment
5) Check the BP and blood glucose

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10
Q

Investigating SPASTIC HEMIPARESIS

A

If acute onset = urgent CT head (esp if <4.5 hours onset, may be candidate for thrombolysis)

BP, BM
12-lead ECG
FBC, ESR, baseline renal and liver function
Coagulation studies
Lipid profile, HbA1c (ischaemic stroke RFs)
Neuroimaging

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11
Q

Further investigations for Ischaemic Stroke

A

Carotid dopplers (if anterior circulation stroke suspected)
24 - 72 hour tape
Echo
MRI with diffusion weighted imaging +/- MRA (dissection, aneurysm, AVM, venous sinus thrombosis, posterior fossa lesion suspected)
Vasculitic and thrombophilia screen = ANA, ANCA, antiphospholipid antibodies, lupus anticoagulant, factor V leiden, protein C and S, antithrombin III
HIV and syphilis serology
?Bubble echo (if young patient and want to exclude PFO,VSD etc)

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12
Q

Management of Ischaemic Stroke

A

ABCDE assessment
Rule out mimics e.g. hypoglycaemia
Urgent CT head (esp if <4.5 hours onset)
Discuss with stroke team
NBM until swallow assessed
Admit to hyperacute stroke unit, with BP monitoring and regular neuro obs
Aspirin 300mg PO/NG/PR for 2 weeks with PPI
Atorvastatin 80mg ON
MDT approach - PTs/OTs/SLT
Secondary prevention e.g. stop smoking
Driving advice

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13
Q

Contraindications to Thrombolysis

A

Seizure at onset of stroke
Stroke/HI < 3 months
Active bleeding
History of UGIB<3 weeks
Major surgery/trauma < 2 weeks
Intracranial neoplasm
Uncontrolled HTN (>200/120)
LP < 7 days
Oesophageal varices
Pregnancy

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14
Q

Complications of Stroke

A

ACUTE
Haemorrhagic transformation
Aspiration pneumonia
Raised intracranial pressure –> herniation
Death

CHRONIC
Pneumonia (HAP/Asp)
DVT –> PE
Pressure sores
Swallow impairment
Depression
Hosp Acquired Infections
Long term morbidity & disability

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15
Q

Haemorrhagic Stroke

A

15-20% of all strokes

CAUSES/RFs
- HTN
- Anticoagulation
- Trauma
- Tumour (1 or 2)
- Aneurysm (e.g. berry aneurysms in ADPKD)
- AVMs
- Infective endocarditis (haem transformation of septic emboli)
- Amyloid Angiopathy

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16
Q

DVLA Advice TIA vs Stroke vs Seizure (Category 1)

A

TIA = stop driving until seen in TIA clinic. If TIA confirmed, stop driving for 1 month

Stroke = stop driving for 1 month, review with doctor

Seizure = cannot drive until 1 year seizure free

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17
Q

DVLA Advice TIA vs Stroke vs Seizure (Category 2 - HGV)

A

TIA = stop driving until seen in TIA clinic. If confirmed, then cannot drive cat 2. for at least 1 year, review with doctor

Stroke = stop driving for at least 1 year, review with doctor

Seizure = cannot drive until seizure free>5 years and seizure free without AEDs for 5 years.
>2 seizures = STOP

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18
Q

Differentials for SPASTIC PARAPARESIS
- bilateral lower limb UMN
- Spastic gait

A

PARASAGITTAL Causes
- Meningioma

SPINAL CORD Causes
- Trauma
- Compressive e.g. SCC, tumour, prolapsed disc
- Demyelinating e.g. MS, NMO
- Infective e.g. transverse myelitis (HSV, VZV, HIV), tropical spastic paraparesis
- Ischaemic e.g. anterior spinal artery occlusion

CONGENITAL Causes
- Hereditary Spastic Paraparesis
- Cerebral palsy
- Friedrich’s Ataxia

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19
Q

Presenting SPASTIC PARAPARESIS

A

If acute onset - MRI to exclude spinal cord compression

1) Take a full history - ?back pain/bladder or bowel disturbance/visual problems
2) Perform a PR (anal tone, saddle anaesthesia)
3) Examine upper limbs and cranial nerves in full as well as speech

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20
Q

Investigating SPASTIC PARAPARESIS

A

If acute onset - need to exclude SCC = Urgent MRI Spine

Bloods
- FBC, renal profile, bone profile
- Myeloma screen (immunoGs, serum electrophoresis)
- Serum oligoclonal bands
- AQ4 antibiodies (?NMO)
- B12
- ANA (vasculitis screen)
- HIV

Imaging - MRI spine

?LP - particularly if history concerning for MS/NMO
? Visual evoked potentials

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21
Q

Multiple Sclerosis

A

= inflammatory demyelinating disease of CNS

RFs = FHx, female > male, increasing latitude

“>2 relapses with evidence of >2 lesions”

Most common form is relapsing-remitting (85%)

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22
Q

Visual Features of MS

A

Optic neuritis
= pain on eye movements, reduced red colour spectrum

Pulrich’s Effect
= reduced depth perception

Internuclear Ophthalmoplegia
= adduction deficit in affected eye and nystagmus in other eye (lesion in MLF)

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23
Q

What is Uhthoff’s phenomenon?

A

Symptoms worsen as temperature increases
- temperature sensitive Na+ channels in demyelinated neurones

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24
Q

Diagnosing/Investigating MS

A

McDonald Criteria

Expanded Disability Status Scale (EDSS)

“Evidence of lesions disseminated in time and space”

MRI (High signal T2, FLAIR sequence)

LP - oligoclonal bands

Visual/Auditory Evoked potential

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25
Q

Positive Prognostic Factors for MS

A

“Typical MS patient”

  • Young age at onset (20-30s)
  • Female
  • Relasping-remitting type
  • Sensory symptoms only
  • Long intervals between relapses/Complete recovery between relapses
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26
Q

How would you manage a patient with MS?

A

MDT approach - PT/OT/SLT

SYMPTOMATIC RX
- Antispasmodics e.g. baclofen
- Anticholinergics/ISC or LTC if bladder issues

ACUTE RX
- High dose steroids for 5-7 days (e.g. PO pred or IV methylpred

DISEASE-MODIFYING THERAPIES (immunomodulatory)
- Beta-Interferon
- Glatiramer
- Natalizumab

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27
Q

What is Neuromyelitis Optica?

A

Autoimmune demyelinating disorder

90% relapsing-remitting

Aquaporin 4 +ve antibodies

= Bilateral optic neuritis + myelitis with 2 of the following:
1) Spinal cord lesion > 3 levels
2) Normal MRI brain
3) AQ4 positive serum antibody

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28
Q

Features of Parkinsonism

A

1) Rigidity
- Unilateral increased tone
- Cogwheeling
- Synkinesia
2) Bradykinesia
- Shuffling gait
- Hypomimia
- Quiet, slow speech
- “Finger tapping” or “Heel tapping”
3) Tremor
- Resting, rotational element
- Usually asymmetrical esp at first
- Synkinesia

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29
Q

Differentials for Parkinsonism

A

*Idiopathic Parkinson’s Disease
*Vascular Parkinsonism (e.g. Basal Ganglia strokes)
*Drug-Induced Parkinsonism e.g. metoclopramide, haloperidol, and risperidone
*Wilson’s Disease

PARKINSON’S PLUS
*Lewy Body Disease
- Hallucinations
- Faster progression, less responsive to medication
*Supranuclear Palsy
- Falls, reduced vertical gaze, poor response to L-dopa
*MSA Type P
- Autonomic and cerebellar dysfunction
*Corticobasal degeneration

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30
Q

Investigating Parkinsonism Features

A

MMSE/MOCA
L+S BP

Routine bloods - vascular RFs

Neuroimaging
- CT head
- MRI head (vasculature of basal ganglia)
- DAT scan (looks at levels of presynaptic dopamine)

Assess response to treatment

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31
Q

Medical Management of Parkinson’s Disease

A

DISEASE MODIFYING MEDICATIONS
- Dopamine replacement = L-dopa + decarboxylase inhibitors = Carbidopa
- Dopamine Receptor Agonists e.g. Ropirinole, Pramipexole, Rotigotine (PATCH!), Apomorphine (LATE) - IMPULSE CONTROL DISORDERS!!!!!!
- COMT inhibitors e.g. Entacapone
- MAO-B inhibitors e.g. selegiline

SYMPTOM CONTROL
- Tremor = Antimuscarinics e.g procyclidine
- Dementia = Rivastigmine
- L-dopa dyskinesias - Amantidine

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32
Q

Management of Parkinson’s Disease

A

MDT Approach - neurologists, PT, OT, SLT, Nutrition team, psychology

MEDICAL
- Dopamine replacement therapies/agents to help reduce breakdown of dopamine
- Symptom control e.g. tremor

SURGICAL
- Deep Brain Stimulation (indicated if on medication >5x day, nil cognitive imp and suitable surgical candidate)

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33
Q

Types of Tremor

A

ESSENTIAL
- Postural/on action (kinetic)
- FHx
- Alcohol/beta blockers help

PARKINSONIAN
- Present at rest
- Low frequency, rolling
- Asymmetrical (at first)
- Increased with distraction

DRUG INDUCED
- Fine tremor, usually bilateral
- E.g. salbutamol, tacrolimus, ciclosporin

CEREBELLAR
- Absent at rest, present with movement, getting worse closer towards target = INTENTION
- Low frequency

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34
Q

Differentials for Chorea

A

Chorea = d2 damage to Basal Ganglia (esp caudate nucleus)

ST VITUS DANCE + Wilson’s

S - Sydenham’s chorea (Rheum fever)
T - Trauma
V - Vascular (stroke)
I - Increased RBC (Polycythaemia)
T - Thyrotoxicosis
U - Uraemia
S - SLE

D - Drugs e.g. L-dopa, antipsychotics. COCP
A - APS
N - Neurodegenerative e.g Huntington’s, Prion disease
C - Chorea Gravidarum
E - Exposure to toxins

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35
Q

LMN Features

A

Wasting/muscle fasciculations
Reduced tone
Reduced/absent reflexes
Normal or downgoing plantar reflexes

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36
Q

Localisation of LMN

A

Anterior horn cell –> nerve root –> plexus –> peripheral nerve –> NMJ –> muscle

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37
Q

Differentials of LMN pattern of limb weakness

A

ANTERIOR HORN CELL
- MND (Nil sensory!!)
- Poliomyelitis

CAUDA EQUINA
- Disc prolapse/abscess/malignancy

LUMBOSACRAL PLEXOPATHY
- Trauma/tumour/abscess
(Usually unilateral)

PERIPHERAL NEUROPATHY
(See separate flashcards)
- Metabolic vs Inflamm vs Infective vs Congenital vs Toxic

NEUROMUSCULAR JUNCTION
- Myasthenia Gravis, LEMS

MYOPATHIES
(See separate flashcards)

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38
Q

Differentials for FLACCID PARAPARESIS

A

ANTERIOR HORN CELL
- MND (No sensory features!!!!!!!!!!!!)
- Poliomyelitis

CAUDA EQUINA
- Disc prolapse
- Abscess
- Malignancy

(Predominantly MOTOR) PERIPHERAL NEUROPATHY
- Inflam = GBS, CIDP, sarcoid
- Infective = HIV
- Toxic = Lead
- Diabetic amyotrophy
- HSMN

NMJ
- Myasthenia, LEMS

MYOPATHIES
- Polymyositis, dermatomyositis
- Muscular dystrophies
- Myotonia

39
Q

Presenting FLACCID PARAPARESIS

A

If acute onset - MRI to exclude spinal cord compression

1) Take a full history - ?back pain/bladder or bowel disturbance/visual problems
2) Perform a PR (anal tone, saddle anaesthesia)
3) Examine upper limbs and cranial nerves in full as well as speech

40
Q

Investigations for FLACCID PARAPARESIS

A

BP, L+S BP, ECG
?Spirometry (FVC), ABG

Bloods
- Routine
- Peripheral neuropathy screen (see other flashcards)
- Anti-AChR antibodies
- CK

Neuro imaging

Special Extra Tests (guided by history)
* LP - ?GBS (raised protein)
* EMG/NCS
* Nerve biopsy or Muscle biopsy
* Genetic testing

41
Q

MND (Anterior Horn Cell Disorder)

A

Progressive degenerative disease of UMN and LMN
Affects Anterior Horn Cells and Motor Cranial Nuclei

Progressive disease (50% die within 2 years of diagnosis)

Mixture of UMN and LMN signs
- Brisk reflexes, upgoing plantars
- Wasting and fasciculations
NO SENSORY DEFICIT

Average age at diagnosis 65

Most common type Amyotrophic Lateral Sclerosis (ALS)

42
Q

Investigation and Management of MND

A

Clinical diagnosis of exclusion - exclude peripheral neuropathy, myopathies

EMG may show active denervation

MDT approach - PT/OT/SLT/nutrition

Riluzole only disease modifying therapy - slows progression by 3 months

NIV - can prolong life by 7 months

Advanced care planning and palliative team involvement

43
Q

What is Kennedy’s disease?

A

= rare, X-linked slowly progressive neuromuscular disorder
Trinucleotide CAG repeat expansions in androgen receptor (AR) gene

Typically an adult-onset disease (20 - 50s)

Limb and facial weakness
Fasciculations of the tongue / face (perioral)
Decreased or absent deep tendon reflexes
Dysphagia, Dysarthria
Androgen insensitivity –> gynaecomastia, erectile dysfunction, reduced fertility, testicular atrophy

MANAGEMENT
Symptomatic and supportive
Life expectancy is normal - ~10% die from swallowing complications d2 bulbar weakness.

44
Q

Poliomyelitis (Anterior Horn Cell Disorder)

A

Caused by the poliovirus
Mainly affects children under 5 years of age.
1 in 200 infections leads to irreversible paralysis.

Flaccid paralysis - usually of one limb, with wasting

Typically no sensory features

45
Q

Peripheral Neuropathies

Which peripheral nerves are myelinated?

A

Motor Nerves = Myelinated

Sensory Nerves = Mixture of myelinated and unmyelinated

Autonomic nerves = Unmyelinated

46
Q

Peripheral Neuropathies

Demyelinating vs axonal loss

A

DEMYELINATING LOSS
- Mainly motor fibres first
- NCS = reduced velocity

AXONAL LOSS
- Sensory loss first
- Affects longest nerve fibres first (feet before hands)
- NCS = reduced Amplitude

47
Q

Peripheral Neuropathies

Causes of predominantly SENSORY peripheral neuropathy

A

A - Alcohol
B - B12 or B1 deficiency
C - CKD, CTD, Cancer (e.g. myeloma), Chemotherapy agents (e.g. vincristine, cisplatin)
D - Diabetes (NUMBER 1 CAUSE)
- Drugs (amiodarone, isoniazid etc)
E - Endocrine causes (Hypothyroid)
F - Folate deficiency
G - Granulomatous disease e.g. sarcoidosis
H - Hereditary e.g. HSMN
I - Infection (HIV, leprosy)

48
Q

Peripheral Neuropathies

Causes of predominantly MOTOR peripheral neuropathy

A
  • Acute inflammatory demyelinating polyradiculopathy (GBS)
  • Chronic IDP
  • Porphyria
  • Heavy Metal toxins e.g Lead
  • HSMN - Charcot-Marie-Tooth disease
  • Diabetes (diabetic amyotrophy)
49
Q

Investigation of Peripheral Neuropathy

A

Urine dip - glucose, protein
BM

Bloods
- Routine FBC, U&Es, LFTs
- B12 and folate
- HbA1C, fasting glucose
- TFTs

Nerve Conduction Studies and EMG
- reduced Amplitude = Axonal

EXTRA TESTS (if initial N)
- Electrophoresis & Immunoglobulins
- Vasculitis screen
- HIV/syphilis serology
- LP
- Genetic testing ?HSMN

50
Q

Hereditary Sensorimotor Neuropathy

A

Encompasses Charcot-Marie-Tooth disease

HSMN Type 1
- Autosomal Dominant
- Includes CMT 1 = defect in PMP-22 gene (myelin)
- Symptoms start in puberty
- MOTOR features predominate e.g. distal muscle wasting (inverted champagne bottle legs, clawed hands), pes cavus, hammer toes
- Areflexia common
- Sensory loss also common (can have sensory ataxia with positive Rhombergs)
- Bilateral foot drop with high-stepping gait.

Demyelinating = reduced velocity on NCS

Rx = Supportive; MDT approach with PT/OT/orthotics/orthopaedics; Analgesia

51
Q

Differentials for Pes Cavus

A

Charcot Marie Tooth disease

Spina bifida

Polio

Friedreich’s ataxia

Syringomyelia

Cerebral palsy

Muscular dystrophy

52
Q

Hereditary Neuropathy with Liability to Pressure Palsy (HNPP)

A

Trivial trauma to peripheral nerves = mononeuropathy

Auto Dominant (PMP 22 gene on chromo 17)
20-30 year olds

Reduced velocity on NCS (demyelinating)

Rx = supportive (splints, padding, orthoses)

53
Q

What is Guillian Barre Syndrome (GBS)?

A

= acute inflammatory demyelinating polyneuropathy (AIDP)

Onset over days- weeks.
Often preceeded by resp infection or diarrhoeal illness (e.g. campylobacter)

FEATURES
Ascending flaccid limb weakness
Distal paraesthesiae - sensory deficits usually mild/patchy
Areflexia.

Can also affect autonomic system = tachycardiac, labile BP, arrhythmias, bladder/bowel issues

Can also affect cranial nerves: ptosis, opthalmoplegia, facial nerve palsy, bulbar weakness

Becomes chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if doesn’t cease by 6 weeks.

54
Q

What is Miller Fischer syndrome?

A

= form of acute inflammatory demyelinating polyneuropathy (AIDP)
Miller-fischer syndrome is a proximal variant of GBS causing ataxia, opthalmoplegia, areflexia

Anti-GQ1b antibodies!

55
Q

How would you investigate for and manage Acute Inflammatory Demyelinating Polyneuropathy?

A

Spirometry
Bedside ECG (risk of arrhythmias)

Bloods
- Routine (FBC, renal function, liver function, inflam markers)
- Antibodies to gangliosides (anti-GM1)

Lumbar Puncture
- Raised CSF protein, normal WCC

Management = mainly supportive, MDT approach
- Refer to ITU if vital capacity <1.3L/bulbar dysfunction. May need tracheostomy
- Give IVIG +/- methylprednisolone, or plasma exchange (liaise with neurology)
- SLT & nutrition team esp if bulbar involvement

56
Q

Ulnar Nerve Palsy

A

Ulnar nerve root = C8-T1

Elbow key site for injury/compression

Wasting of dorsal interossei and hypothenar eminance
Clawing of 4th and 5th fingers

MOTOR =
- Small muscles of hand (excluding LOAF) e.g. palmar and dorsal interossei (PAD/DAB) = palmar ADduct, dorsal ABduct
- Flexor carpi ulnaris
- Adductor pollicis

SENSORY =
- Dorsal and palmar surface of hand over 5th and half of 4th finger

FROMENT’S sign = weak adductor pollicis brevis

57
Q

Median Nerve Palsy

A

Median nerve root = C6-T1

Wrist (carpal tunnel) key site for injury/compression

Wasting of thenar eminence

MOTOR =
- LOAF muscles of hands = mainly affects thumb movement
- Weak thumb abduction, flexion and opposition
- Wrist and finger flexion

SENSORY =
-Palmar surface of hand from thumb to half of 4th digit
- Dorsal surface of digits 1-4 (not on back of hand)

PHALEN’S test = numbness on upside down prayer sign

TINEL’s test = numbness whilst tapping over flexor retinaculum

58
Q

Causes of Carpal Tunnel Syndrome

A

= median nerve compression between flexor retinaculum and carpal bones

Idiopathic
Work-related repetitive strain
MSK
- RA
- Gouty tophi
- OA
- Wrist fractures
Endocrine
- Pregnancy
- Acromegaly
- DM
- Hypothyroidism
CKD
Myeloma
Amyloidosis
Vasculitides

59
Q

Radial Nerve Palsy

A

Radial nerve root = C5-T1

Site for injury/compression can occur in axillary, humeral and elbow regions

Wrist drop! Finger drop!

MOTOR =
- Extensors of forearm, wrist and fingers & triceps brachii
- Weak wrist and elbow extension
- Unable to straighten fingers
- Weakness of finger adduction and abduction (can be overcome if hand placed on flat surface)

SENSORY =
- Palmar base of thumb, dorsal surface of radial side of hand (does not innervate any finger sensation)

60
Q

Differentials for Foot Drop

A

Charcot-Marie-Tooth Disease
Common Peroneal Nerve palsy
MND
L4/L5 root lesion

61
Q

Differentials for Foot Drop

A

Think anterior horn cell—nerve root—plexus—peripheral nerve—NMJ—muscle

Muscle (weak anterior tibialis): any cause of myopathy
Peripheral Nerve:
** Common peroneal nerve palsy (mono/polyneuropathy) eg. trauma to fibular head, surgery on leg, compression of fibula neck, mononeuritis multiplex of any cause, Charcot Marie Tooth disease
** Sciatic nerve palsy eg. trauma
Plexus: Lumbosacral plexopathy
Nerve Root: L5 root lesion eg. prolapsed disc
Anterior horn cell eg. MND (NB: no sensory deficit)

62
Q

Common Peroneal Nerve Palsy

A

FEATURES
Weak ankle dorsiflexion
INversion is INtact, EVersion “EVicted”
Ankle reflex intact
If Deep branch only: preserved eversion and sensory loss only in webspace between 1st and 2nd toes and not lateral lower leg or foot dorsum

63
Q

Sciatic Nerve Palsy

A

L4 - S3

FEATURES
Weak knee flexion
Weak plantarflexion and dorsiflexion
Weak eversion and inversion
Widespread lower limb sensory loss

64
Q

L5 Lesion

A

L5 lesion:
Cannot straight leg raise
Weak inversion and eversion
Weak dorsiflexion

Reduced sensation on sole of foot as well as anterolateral shin and foot dorsum

Ankle jerk preserved

65
Q

Causes of Mononeuritis Multiplex

A

Diabetes! (Top cause)

(CALVS)

C - Cancer
A - Amyloidosis
L - Lyme disease/Leprosy
V - vasculitides e.g. EPGA, GW, RhA, SLE
S - Sarcoidosis

66
Q

Types of Diabetic Neuropathy

A

1) “Glove and Stocking” Polyneuropathy
- Distal, symmetrical
- Progressive (distal to proximal)
- Axonal loss

2) Autonomic Neuropathy
- Postural hypotension
- Gastroparesis
- Gustatory sweating

3) Diabetic Amyotrophy
- Microvasculitis
- Asymmetrical, proximal weakness
- Hair loss, poor vascular supply

67
Q

Myasthenia Gravis

A

Autoimmune disease of Acetylcholine receptors at NMJ (IgG antibodies to AChR in 85%)

Associated with thymomas (15%) and other AI conditions e.g. pernicious anaemia

Muscle fatiguability!
Proximal muscle weakness
Extraocular muscle weakness –> diplopia, ptosis
Reflexes normal but reduce with repeated testing

3 Forms
- Ocular (85%)
- Oropharyngeal
- Generalised

68
Q

Investigations for Myasthenia Gravis

A

Spirometry (FVC)

Bloods
- Routine, including TFTs
- CK (exclude myopathy)
- Anti AChR antibodies

Single fibre EMG (jitter)

CT thorax (exclude thymoma)

{Sussman Guidelines}

69
Q

Management of Myasthenia Gravis

A

MDT Approach - neuro, PT, OT, SLT, respiratory team

Assess respiratory function

Avoid precipitating medications

Pyridostigmine (inhibits anticholinesterase)

Immunosuppression e.g. steroids (risk of paradoxical reaction in first 2 weeks = inpatient), AZA/MTX

?Thymectomy

70
Q

What is a Myasthenic Crisis? How would you manage it?

A

Exacerbation requiring mechanical ventilation (FVC < 1.5L)

Rx = mainly supportive, consider early referral to ICU for ventilatory support
- Remove trigger
- IVIg or Plasma exchange

71
Q

Drugs which can trigger a Myasthenic Crisis

A

Gentamicin

Macrolides
Tetracyclines
Quinolones
Procainamide
Beta blockers
Lithium
Phenytoin

72
Q

Mixed UMN and LMN Signs

Differentials of ABSENT ankle jerk and EXTENSOR plantar reflexes

A

MND
Subacute combined degeneration of the cord (vit B12)
Friedrich’s Ataxia
Syphilis

73
Q

Mixed UMN/LMN Signs - Differentials

A

Most likely = dual pathology e.g. cervical myelopathy (UMN) and peripheral neuropathy (LMN)

MND (nil sensory signs)

Subacute Combined degeneration of the Cord (B12)

Syringomyelia
= cyst in central canal of spinal cord = UMN signs initially
- As expands, starts to damage Anterior Horn Cells = LMN signs

74
Q

What is Subacute Combined Degeneration of the Cord?

A

Vitamin B12 deficiency results in damage to:
1) Doral columns (UMN)
- Symmetrical distal sensory neuropathy (legs > arms)
- Loss of light touch, proprio and vibration
2) Lateral corticospinal tract (UMN)
- Muscle weakness and spasycity
- Hyper-reflexia
- Upgoing plantars
3) Spinocerebellar tract (UMN)
- Sensory ataxia
4) Peripheral nerves (LMN)
- Glove and stocking distribution
- Absent ankle reflexes

Rx = B12 supplementation (neuropathy recovers better than myelopathy)

Investigate cause - ?malnutrition ?malabsorption ?pernicious anaemia ?Nitrous oxide abuse

75
Q

Causes of Syringomyelia

A

Syringomyelia = fluid filled cyst in central canal of spinal cord

CAUSES/ASSOCIATIONS
- Chiari malformations
- Spinal cord tumour
- Trauma
- Idiopathic

76
Q

Features of Syringomyelia

A

Reduced pain and temp sensation in “cape-like distribution”
Light touch/vibration/proprio preserved
LMN signs of upper limbs = wasting and fasciculations
UMN signs of lower limbs = brisk reflexes, upgoing plantars
Bladder and bowel dysfunction

77
Q

Differentials for Myopathies

A

Autoimmune
- Dermatomyositis, polymyositis, inclusion body myositis

Connective Tissue Disease
- SLE, Vasculitides, RA, systemic sclerosis

Metabolic
- Mitochondrial disease, glycogen storage disorders (e.g. McArdle’s)

Endocrine
- Thyroid disorders, Cushing’s, Addison’s, Acromegaly, Diabetes

Dystrophies
- Myotonic Dystrophy, Duchenne’s, Becker’s , FSHD

Drugs
- Alcohol, heroin, statins, steroids, amiodarone

78
Q

Investigations for Myopathies

A

Urine dip & PCR (dip for blood could be myoglobin)

Bloods
- Routine
- CK, AST, ALT, LDH
- TFTs, HbA1c, cortisol
- Rheum screen including ACE
- Immunoglobulins
- Myositis antibodies

NCS and EMG

?MRI muscle

Muscle biopsy

Systemic screen = ECG, PFTs, CXR, echocardiogram

Malignancy screen (if dermato/polymyositis) = FOB, mammogram, tumour markers, CT, endoscopy

79
Q

What are Duchenne’s and Becker’s muscular dystrophy?

A

Inherited degenerative condition of muscle
X-linked, mutations on dystrophin gene on X chromosome

Typically occurs in childhood (can have later onset in Becker’s)

Progressive proximal muscle weakness
Calf pseudohypertrophy
Risk of cardiomyopathy (less in Becker’s)

Raised CK, deranged transaminases
Muscle biopsy
Genetic testing

80
Q

What is Facioscapulohumeral dystrophy?

A

Inherited degenerative myopathy
Autosomal dominant

Slowly progressive disease of facial muscles and shoulder girdle

FEATURES
- Facial weakness –> difficulty closing eyes, asymmetric smile, can’t whistle, dysarthria
- Difficulty raising arms above head
- Winging scapulae
- Triceps more wasted than biceps (deltoid spared)
- Waddling gait (pelvic girdle involvement)
- Preserved IQ
- Lung/cardiac involvement rare

81
Q

What are the genetics of Myotonic Dystrophy?

A

Autosomal dominant (trinucl rep disorder)
- >50 repeats = full penetrance

Most common mutation = DMPK gene on chromosome 19

Genetic anticipation

82
Q

What is Myotonic Dystrophy?

A

Most common adult muscular dystrophy

FEATURES
- Myotonia = failure of relaxation of voluntary contraction
- Frontal balding
- Partial ptosis
- Facial muscle wasting “Hatchet facies”
- Distal muscle weakness
- Dysarthria
- Dysphagia

Usually wheelchair dependent within 15-20 years of onset

NORMAL CK (in comparison to Duchenne’s & Beckers)

83
Q

Name some associated conditions of Myotonic Dystrophy

A

Diabetes mellitus
Testicular atrophy

Cardiomyopathy
Conduction disorders

Cholecystitis and biliary spasm
Constipation
Slow gastric emptying

Hypersomnolence (?cause)

84
Q

Name 2 types of inherited neurocutaneous disorders? What is their mode of inheritance?

A

Neurofibromatosis & Tuberous Sclerosis

Both Autosomal dominant!

85
Q

Features of Neurofibromatosis 1

A

Autosomal dominant (chromosome 17)

Cafe au Lait spots (>6)
Peripheral neurofibromas (>2)
Iris hamartomas (>2)
Axillary/groin freckles
Scoliosis
Phaeochromocytomas

86
Q

Features of Neurofibromatosis 2

A

Autosomal dominant (Chromosome 22)

Bilateral acoustic neuromas/vestibular schwannomas
Multiple intracranial tumours e.g. schwannomas, meningiomas

87
Q

Tuberous Sclerosis

A

Majority autosomal dominant (TsC2 gene on chromosome 16 = most severe form)

CUTANEOUS FEATURES
Ash-leaf spots (fluoresce under UV)
Shagreen patches (rough patches over spine)
Angiofibromas (butterfly over nose)
Subungual fibromata

NEURO FEATURES
Developmental delay
Lower IQ
Epilepsy

ASSOCIATED FEATURES
Retinal hamartomas
Cardiac rhabdomyomas
Polycystic kidney disease
Lung cysts

88
Q

Differentials for Ataxic Syndromes

A

V - Stroke, ICH

I - Multisystem Atrophy C

T - Traumatic brain injury, Alcohol, Carbamazepine, phenytoin

A - MS, Miller-Fisher Syndrome

M - B12 deficiency, Copper deficiency

I - Spinocerebellar Ataxia, Friedrich’s Ataxia, Ataxia Telangiectasia, VHL

N - Cerebellar space occupying lesion, paraneoplastic (small cell lung cancer, breast, gynae, testicular)

89
Q

Outline cerebellar signs

A

D - Dysdiadochokinesis

A - Ataxic (gait, limb or truncal)

N - Nystagmus (gaze-evoked)

I - Intention tremor

S - Slurred or staccato speech
(dysarthria)

H - Hypotonia

Also - Rebound phenomenon!

90
Q

Name 3 inherited ataxia syndromes?
What is their mode of inheritance?

A

Spinocerebellar Ataxias - group of conditions, autosomal dominant trinucleotide repeats

Ataxic telangiectasia - autosomal recessive

Friedrich’s Ataxia - autosomal recessive

91
Q

Friedrich’s

A

Autosomal recessive disorder (trinucleotide repeat, FRATAXIN gene on chromosome 9)

Most common hereditary ataxia in UK

Combination of spinocerebellar signs, corticospinal tract signs and dorsal column loss

FEATURES
Onset in adolescence
Gait ataxia
Kyphoscoliosis
Mix of UMN and LMN - absent ankle reflexes, upgoing plantars
Spasticity
Loss of proprioceptive and vibration sensation

ASSOCIATED FEATURES
HOCM (90%, common cause of death)
Diabetes
High arched palate
High arched feet
Preserved IQ

{Vitamin E deficiency = rare mimic!}

92
Q

Ataxia Telangiectasia

A

Autosomal recessive disorder (ATM gene)

Inherited Combined Immunodeficiency disorder - 10% risk of development malignancy (esp Leukaemias, lymphoma)

FEATURES
Typically presents in childhood as abnormal movements
Cerebellar ataxia
Skin & eye telangiectasia
Recurrent LRTIs (due to IgA deficiency)
Dystonia
Chorea

93
Q

Spinocerebellar Ataxia

A

Group of autosomal dominant disorders - majority trinucleotide repeats

FEATURES
Usually develops in 30-40s
Progressive cerebellar signs
UMN and extrapyramidal signs
Peripheral neuropathy
Ophthalmoplegia

PACES Revision Cards (9 decks)

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