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Neurology 1

Retesh Bajaj MRCP (Lond.)Interventional Cardiology SpR

@medicineinasuitFB: Intelligent Finals (for future courses)

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Housekeeping

• Finals Courses – December 14-15 and January 25-26 on Eventbrite

• intelligent.finals@gmail.com

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Limb Exams Tips

• GAIT

• Sensation is always distal to proximal• Start with light touch/pin-prick then vibration and

proprioception

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Power

• 0 = no contractions

• 2 = active movement without gravity• 3 = movement against gravity

• 5 = normal

• More helpful to describe this than using the grade

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How to present• Best to summarise the motor and sensory assessment – ie proximal

vs distal, symmetrical vs assymetrical, pyramidal distribution?

• Split into:

• Describe muscle patterns

• Describe the motor and sensory signs in groups – ideally by ”tracts”

• How would you confirm the diagnosis

• First steps in management (associations and complications)

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Case 1• Patient with blurred vision and poor coordination.

Examine upper limbs

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Multiple Sclerosis

• Female, catheterised, eye signs, cerebellar signs – ATAXIA (unable to heel-toe walk)

• UMN signs – pyramidal weakness and brisk reflexes• Dorsal column disease – reduced vibration, proprioception,

Romberg’s +ive• Isolated VI nerve palsy

• Commonest form – relapsing and remitting, signs disseminated in time and space

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How to present MS• On inspection Mrs X is well. There is a right sided pronator drift and pseudoathetosis of

the fingers (suggesting loss of proprioception). There is disuse atrophy of the muscles of the right arm. There are pyramidal signs evidenced by increased tone, pyramidal weakness and hyperreflexia on the right and posterior column signs with loss of vibration and proprioception distally.

• There are some cerebellar signs as evidenced by rebound phenomenon and an intention tremor.

• The combination of cerebellar, dorsal and pyramidal signs suggest a central pathology and the most likely diagnosis is demyelinating disease such as multiple sclerosis. Mrs X is currently using a wheelchair and there is a urinary catheter in place. She needs a full functional assessment

• I’d like to examine the fundi to complete my examination. The diagnosis can be confirmed on MRI looking for periventricular white matter lesions and VEPs. A full history would be beneficial to assess

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MS – presenting INO

• There is an ataxic nystagmus in the right eye as it fails to adduct. When the abducting eye is covered the other eye is able to adduct normally

• This is intranuclear ophthalmoplegia which is most likely related to MS due to a lesion in the medial longitudinal fasciculus

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Specifics

• Intranuclear opthalmoplegia – demyelination at the MLF –often bilateral• Failure of ADduction of the eyes, nystagmus in normal eye

on lateral gaze

• Lhermitte’s

• Uthoff’s

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Associations

• Optic nerve damage – central scotoma, RAPD, colour and VA loss, optic atrophy

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Investigations

• VEP if single nerve lesion, otherwise

• MRI (T2) to show demyelination

• Periventricular white matter plaques

• CSF – oligoclonal bands

• Bloods – include B12 and TFTs

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Management• MDT• Acute – steroids• Chronic: (drugs + pain/bladder/mobility)

• Disease modifiers: beta-interferon, galatilamir, natalizumab• Anti-spasticity – benzos and baclofen

• Pain – neuropathic agents – gabapentin/pregabalin, CMZ• Bowel (laxatives) and bladder (oxybutynin, LT catheter)• Mobility• Cognitive

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Some differentials

• Neuromyelitis optica – aka Devic’s disease = optic neuritis and TM

• IgG against aquaporin 4 water channel

• Demyelination

• Cervical spondylosis – without the cerebellar signs

• SACD – dorsal columns and cerebellar…

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Give me some causes of demyelination• HIV

• CIDP

• Multiple myeloma

• MGUS

• HMSN

• types 1 & 3

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Cerebellar Signs

• DANISH… = signs

• PASTRIES = causes

• Nystagmus – fast saccades towards the side of the lesion and worse on looking towards the lesion

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Better Cerebellar System

• Head: head tremor (titubation), eyes nystagmus and dysarythria (Speech – British Constitution)

• Arms: rebound phenomenon, intention tremor and past pointing and DDK

• Legs: heel-shin, ataxic gait and Romberg’s negative

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Causes• MS – eye signs (RAPD, INO), spastic paraparesis, catheterised

• Alcoholism – peripheral neuropathy, liver signs

• Hypothyroidism

• Paraneoplastic – bilateral signs

• Friedrich’s – pyramidal signs, pes cavus, k-s

• Posterior fossa space occupying lesion: VI CN palsy

• Phenytoin and lithium

• COELIAC

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Charcot Marie Tooth• Scoliosis• Motor and sensory losses (sensory milder)• Motor loss: antero-lateral compartments – ankle dorsiflexion and toe

extension• Absent reflexes• Pes cavus• Papable lateral popliteal nerve. Thickened nerves at medial malleolus• High stepping and ataxic gait• NO scars over fiblula – looking for signs of popliteal nerve damage

from trauma

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How to Present CMT• On inspection of Mr X there is symmetrical wasting of the muscles of the lower limbs

distally with relative preservation of the thigh muscles with pes cavus. There is also wasting of the distal small muscles of the hand

• There is a LMN pattern of weakness in the extensors of the toes and feet and a loss of sensation to light touch in a stocking distribution in both legs. The ankle jerks are absent and the plantar reflexes show no response

• The gait is high-stepping.

• There is a combined sensory and motor peripheral neuropathy - likely hereditary motor and sensory neuropathy

• Mr X is currently using foot splints as mobility aids. He needs a full functional assessment. The diagnosis can be confirmed with a family history, nerve conduction studies and genetic testing

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CMT

• Type 1 – demyelination - - - inherited AD but also others

• Type 2 – axonal - - - AD/AR

• Type 3 – demyelination - - - presents in infancy (AR)

• DDx – other causes of a peripheral neuropathy involving both sensory and motor

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DDx• Common peroneal nerve palsy

• Inversion of foot is normal

• L5 root lesion

• Inversion of foot weaker

• B12 Def

• DM

• Alcohol

• Hypothyroid

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Investigations and Management

• NCS

• PT

• Walking aids

• Ankle and foot supports

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Some causes of peripheral neuropathy

• Sensory/Motor/Autonomic

• Mainly sensory: alcohol, diabetes, hypothyroidism (vitiligo?), B12 deficiency, HIV, drugs

• Mainly motor: CIDP, HMSN, lead poisoning

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Pes Cavus

• High foot arch that does not flatten on weight bearing • Causes

• Bilateral: Friedrich’s ataxia, spinal cord tumours, syringomyelia• Unilateral: polio, compartment syndrome, spinal trauma/tumour

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Epilepsy Drug SEs

• Sodium valproate: tremor

• Carbamazepine: enzyme inducerneutropaenia, SIADH

• Phenytoin: cerebellar syndrome (in toxicity), gum hypertrophy, facial coarsening

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Cerebral Venous Sinus Thrombosis • Headache and papilloedema. Signs of raised ICP (6th nerve

palsy)

• Seizures

• Remember Cushing’s reflex

• HTN, bradycardia and irregular breathing

• MRI with MR venography is usually used in diagnosis

• Give LMWH

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BIH

• Obese, female, papilloedema, postural headache worse with straining

• Ass. w/ OCP

• Opening pressure > 25 cm H20

• Rx: Acetazolamide

• V-P or L-P shunt

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Myotonic Dystrophy

• Face: myopathic facies, frontotemporal balding, bilateral ptosis

• Wasting of facial and muscles of mastication

• Distal wasting and weakness with loss of ankle jerks

• Unlike most muscular disorders which cause proximal myopathy first

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Myotonia

• Percussion myotonia: hypothenar eminence depression visibly fills slowly after percussion

• Slow-releasing grip – remember to SHAKE HANDS

• Difficulty opening eyes after closing tightly

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How to present Myotonic Dystrophy• This gentleman has evidence of myopathic facies, frontotemporal hair loss with

bilateral ptosis, wasting of the facial muscles and sternocleidomastoids.• There is also symmetrical wasting of the forearm muscles and small muscles of

the hands bilaterally. There is a reduction in power distally in both arms in all muscle groups tested with normal sensation and coordination. The reflexes are reduced.

• Mr X had difficulty in releasing my hand from a squeeze and opening his eyes when closed firmly – suggesting myotonia

• This is likely to be myotonic dystrophy. To complete my assessment I would like to examine the cardiovascular system, check the blood sugar and perform a full functional assessment. The diagnosis can be confirmed with a family history, EMG and genetic testing

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Associations

• Cataracts

• Dysphagia

• Cardiac – both cardiomyopathy and heart block (may need PPM)

• DM

• Hypogonadism

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Investigations• LFTs should be checked for NM resp insufficiency

• ECG for conduction block

• Echo for CM

• Fasting glucose for DM

• Slit lamp for catatracts

• SALT• EMG: ‘dive-bomber’ potentials

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Rx

• Rx – MDT; phenytoin for myotonia, weakness has no Rx

• Genetic counselling

• AD – with genetic anticipation: worsening severity of the condition and earlier age of presentation within successive generations

• Trinucleotide repeat. DM1: Chr 19. DM2: Chr 3

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Friedrich’s ataxia

• Cerebellar signs

• Pes cavus

• <25 when cerebellar signs

• Loss of ankle jerks and upgoing plantars – dorsal column loss

• CST degeneration

• Axonal damage on NCS

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Associations and Complications

• Diabetes

• Cardiomyopathy

• Kyphoscoliosis

• SN deafness

• Trinucleotide repeats – GAA on Chromosome 9 causing spinocerebellar degeneration

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Parkinson’s - Diagnosis

• Demonstrate rigidity, tremor, postural instability

• Synkinesis

• Cogwheeling

• Asymmetry

• Festination

• Head/chin tremor, expressionless face, monotonous voice, lack of blinking, loss of arm swing

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How to present PD

• This gentleman has signs on inspection of a resting pill-rolling tremor, expressionless face and decreased blink rate

• The gait is stooped, slow to initiate and the steps are shuffling with decreased arm swing

• On examination of the arms there are assymetric UMN signs including increased tone and lead-pipe rigidity in the left compared with right and some loss of muscle power in all groups tested

• There is bradykinesia to all movements. There is no sensory involvement

• This gentleman has signs consistent with Parkinsonism (or Parkinson’s syndrome) – he needs a full functional assessment and MMSE. He is currently limited in his mobility as he uses a walking stick

• I would like to take a drug history, look for a postural BP drop and examine the cranial nerves for limited upgaze

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DDx

• Drug induced

• MSA – including Shy-Drager (autonomic), olivopontocerebellar(cerebellar), striatonigral

• PSP – loss of downward gaze, backward falls and axial rigidity. Associated with fronto-temporal dementia

• CBD – single arm signs, myoclonus, alien limb syndrome. Associated with fronto-temporal dementia

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What features make PD more likely?

• Poor family history

• Response to levodopa

• Unilateral onset

• Tremor

• Progressive not stepwise deterioration

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Complications

• Falls

• Cognitive impairment

• Depression

• SEs of medications

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PD Drugs• Levodopa + decarboxalase inhibitor: Sinemet, Medapar

• Dyskinesias, on-off phenomena, postural hypotension• DA – ergot (cabergoline, bromocriptine, pergolide) cause

retroperitoneal & pulmonary fibrosis• Non-ergot: ropinorole, pramipexole – avoids the above: now first line

over ergots• Dopamine dysregulation syndrome – hypersexuality and gambling• Apomorphine: sc for advanced disease

• Amantadine: for dyskinesias secondary to levodopa• COMT inhibitors: entacapone for tailing off between doses of

levadopa

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Migraine

• With or without aura

• May cause hemiplegia

• Triggers: alcohol, chocolate cheese, oestrogens (OCP)

• Sumatriptan, ergotamine

• Prophylaxis: pizotifen, propranolol, verapamil

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MND

• ‘UMN in lower limbs and LMN in upper limbs’

• Combination of upper and lower MN signs with no sensory deficits

• Sparing of eyes

• Wasting and fasciculation of muscles

• Increased tone and reflexes (ankle clonus and Hoffman’s sign)

• Tongue fasciculations, palatal paralysis, nasal speech

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Associations and Complication

• Respiratory compromise

• Frontal lobe dementia (2%)

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What do you know about MND?

• Multiple subtypes:

• ALS – Upper and LMN

• Progressive muscular atrophy – mainly LMN

• Primary lateral sclerosis – mainly UMN

• Progressive bulbar palsy

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Ix and Mx• Clinical diagnosis – investigate to rule out treatable causes

• SALT

• Anticholinergics – hyoscine patches: for drooling

• Spasticity – dantrolene and baclofen

• Emotional lability – lithium

• Fasciculations and cramps – magnesium, diazepam, phenytoin

• Depression

• Pain

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Normal Pressure Hydrocephalus

• Hydrocephalus = enlarged ventricles on brain imaging

• Failure to absorb CSF in subarachnoid space

• Dementia + gait abnormality + incontinence

• Shunt will treat 70%

• LP – normal pressure

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