Essentials of Neurological History,

Examination and Investigation

Dr Oliver Lily

Consultant Neurologist,

Leeds General Infirmary

By the end of this lecture you will:

• Understand the importance of history taking

and physical examination

• Have a working knowledge of CT/MRI

• Be introduced to EEG

• Be aware of some rare acute neurological

conditions

BRITISH MEDICAL JOURNAL 31 MAY 1975

Relative Contributions of History-taking, Physical Examination, and

Laboratory Investigation to Diagnosis and

Management of Medical Outpatients

J. R. HAMPTON, M. J. G. HARRISON, J. R. A. MITCHELL, J. S.

PRICHARD, CAROL SEYMOUR

History

Examination

Investigation

The importance of history taking

Case 1:

• This 19 year old air stewardess blacked out in

a nightclub

• She remembers feeling hot and went outside to

get some fresh air. Outside she collapsed and

was propped against a wall by the bouncer.

Her eyes were open and she started jerking her

arms and legs. She was incontinent.

Case 1

• She recovered after 15 minutes. She was dizzy and

pale. She vomited. Paramedics found her BP to be

high at 158/90. By the time she reached A+E she

was well and discharged.

• What is the diagnosis?

• What test does she need?

• Can she drive?

• What about work?

Syncope

• Always has characteristic aura (called “pre-

syncope”)

• Is usually postural

• Other types include vaso-vagal or cardiogenic

• Can cause convulsion (now called “convulsive

syncope” rather than “reflex anoxic seizure”)

• Epilepsy driving regulations do not apply

(inform DVLA if recurrent)

Differentiating from epilepsy:

• Postural or activity related

• Aura secs to mins

• Dizziness, heat, sweating, thirst, trembling

• Brief or no convulsion

• Sick and tired afterwards

• Random

• Aura fraction of sec

• Fear, deja-vu, smells,

rising abdominal aura

• Prolonged memory

loss (15 mins plus)

• Brief or no convulsion

• Confused afterwards

The Importance of History taking:

Case 2

• 48 year old man seen on long-stay psychiatric ward,

Referred with falls and dyskinetic movements

• History of paranoia and odd behaviour.

• Chaotic financial affairs. Heavy drinker.

• Little history from patient. Agitated, chain-smoking.

Will not make eye-contact. Muttering. Thin and

unkempt.

• Taking small dose of Haloperidol. No alcohol for 2

weeks.

Case 2

• On examination:

– Loss of vertical pursuit eye movements

– Ataxic gait

– Frequent fidgety movements of legs and jerking

of right shoulder

• CT scan normal

• ? diagnosis

Case 2: Family history

Huntingtons Chorea

• Rare (1 in 20000)

• Frequently presents with dementia or

behavioural problems before chorea

(especially in younger patients)

• Eye movements often abnormal at an early

stage

Case 3

• 45yr old R-handed man presented to A+E

• Normally fit and well, ran 3 miles day before (as

does most days)

• At work about 4pm began feeling lethargic,

muddled and “not himself”

• Family noticed unsteady on feet and slurred speech

when got home. Swaying, jerkiness of limbs.

Vomited twice in the evening. Getting progressively

worse

Admitted to Stroke Unit

– Apyrexial, BP 148/100, RR 20, sats 98%

– Some odd behaviour

– bilateral nystagmus

– cerebellar incoordination L>R

– cerebellar dysarthria

– truncal ataxia

Investigations:

• Bloods:

– Na 147, K 4.8, urea 9, Cr 119

– ALT 35, bil 12, alb 44, Alk phos 218

– Ca 1.82, PO4 71

– PV 1.79, CRP 19, Chol 6.0

• CXR and CT head normal

• Diagnosis: Cerebellar stroke

• Given aspirin

Progress:

• 21/3 (day 5) - consultant WR:

– much better

– MRI scan normal

• 22/3 (day 6)

– feeling breathless and anxious, vomiting, sore throat, diarrhoea

– RR 18, BP 160/80, chest clear

– ECG - peaked T waves

– U&E show acute renal failure

Further deterioration:

• 24/3 (day 8):

– reports difficulty swallowing and choking on

food and fluids. Intermittently pyrexial

• 25/3 (day 9; Good Friday):

– drooling out of L side of mouth. Cannot

swallow tablets. Agitated. Not sleeping. Pupils

not reacting to light. Decreased air entry R lung

base. PR bleeding

Progress:

• Day 10

– transferred to ITU, sedated and

ventilated

• Day 14

– sedation withdrawn, unresponsive, no

brainstem reflexes, no respiratory effort

– flat EEG. MRI remains normal

– ? Can we discuss organ donation

Diagnosis:

• Clinical diagnosis made

• Diagnostic test was performed

• What is missing from the history?

Diagnostic test:

• Plasma ethylene glycol levels:

– 17/3 436mg/l

– 18/3 <100mg/l

• Represents a very large dose

– last drink taken (coke at work) 1pm

– blood taken 8pm

– half life 3 hrs

– estimated peak level 1600mg/l

– >500mg/l indicates severe poisoning

The Importance of examination:

Case 4

• 29 yr lady 11 days post-partum

• Uneventful SVD with epidural

• Headache from first day. Initially postural but

later persistent.

• 17/5 Admitted with sudden onset tingly

numbness on R side of body including face.

• Notes document “CNS grossly intact”

Case 4

• Later the same day had a tonic clonic

seizure.

• Next day: Further seizures without full

recovery. Sedated and ventilated NICU. CT

scan reported as L frontal infarct with

haemorrhagic component. Echo and carotid

dopplers normal.

Case 4

• Day 3 - Sedation lightened. No response.

O/E Deeply comatose. R plantar upgoing.

Normal brainstem reflexes.

• What has been missed on examination?

• What is the diagnosis?

Case 4 – diagnosis..

Case 4 – diagnosis..

Case 4

• Diagnosis – Sagittal sinus thrombosis

• Heparin (IV). Mannitol x3. ICP monitor

• 20/5/03 - pupils fixed and dilated. No

brainstem reflexes. ICP - 65

• Barbiturate coma

• Died day 7

Discussion

• Post-partum pro-thrombotic state

• Cerebral venous thrombosis is rare but

potentially devastating

• Papilloedema can be only physical sign

• No fundoscopy until attending neurology

• Little awareness among

obstetricians/anaesthetists/GPs

The importance of examination:

Case 5

• 67 female

• Retired 2 yrs ago

• 1yr history of personality change, apathy and forgetfulness

• Dizziness and falls

• wieght loss 3 stone

• Aspiration pneumonia

• Non-smoker

• 15u alcohol/day for past 2 yrs

• Generalised seizure 2yrs ago

• O/E AMTS 5/10

cachectic

dysphagia and dysarthria

bradykinesia and truncal rigidity

marked postural instability

staring eyes with lid retraction

Normal Investigations:

• CT

• MRI

• LP

• Diagnosis?

Diagnosis:

• Progressive supranuclear palsy

– subcortical dementia

– early falling (esp backwards)

– supranuclear downgaze palsy

– growling dysarthria

– early dysphagia

PSP – early eye signs:

• Slowing vertical saccades

• lid retraction, eyelid opening or closing apraxia, blepharospasm, or lid lag.

• “masked facies and a startled expression are frequent findings. Retrocollis may be present; with lid retraction, it enhances the astonished, worried appearance “

• Square wave jerks

• Loss of convergence

• Loss of Bells phenomenon

An introduction to MRI:

Case 6

• 46 year-old TV producer. Found wandering in his pyjamas on the hard shoulder of the M62 at 4am. Initially taken into police custody. Unrewarding discussion with duty psychiatrist. He claimed not to remember who he was or what he was doing. Knew nothing about where he was or what day it was.

• Knew his own name and that he was married. Could not give wifes name. Many inconsistencies noted (eg did not know where he was, but could find the mens toilet when needed, remembered he took sugar in his tea etc.)

Case 6

• Kept asking the same questions over and over. Became agitated and needed to be sedated. At one point complained that the furniture in the police station was “wrong” and seemed to be hallucinating.

• On the way to the hospital, had a tonic clonic seizure

• ? diagnosis

Temporal lobe pathology

• Positive phenomena:

– Memory disturbances common (anterograde and retrograde amnesia), fugue, deja-vu, presque-vu, jamais-vu

– hallucinations –

• visual formed eg macropsia/micropsia

• Auditory hallucinations, hyperreligiosity

• Smell

– Psychosis

More MRI:

Case 7

• A 23 year old lady failed to turn up for work as a

hairdresser for two days. Friends broke into her house

and found her in a disorientated and unkempt state.

• Her boyfriend reported an argument the night before

and she was admitted as ? Overdose.

• She had a history of mild depression and took

fluoxetine tablets only.

Case 7

• In hospital she was mute or monosyllabic. Took no interest in her surroundings. She ate little and slept a great deal.

• No neurological signs.

• Drug screen was negative in urine. CT scan and EEG were normal.

• The on-call psychiatrist wondered if she had severe depression and suggested ECT

Features of Medial Thalamic

Stroke:

• Confusion, coma, neuropsychiatric

problems.

• Somnolence

• Mutism

• Abulia

• vertical gaze ophthalmoplegia

• Loss of convergence

Arterial supply:

• Posterior thalamosubthalamic paramedian

artery

• Arises from PCA

• In 30% of people, bilateral supply from the

same PCA (artery of Percheron)

Advanced MRI:

Case 8

• 20 year old female student. Felt tired and unwell,

difficulty sleeping and concentrating in lectures.

Diagnosed with depression and started on citalopram.

• Six months later, complaining of burning pain in both

feet. Nerve conduction studies normal. Neurologist

suggested stress. Very emotional and tearful. Several

sessions with psychologist.

Case 8

• 2 months later stopped attending clinic.

Dropped out of university. Mother reported

profound personality change and jerking of

arms.

• CT scan and LP normal

Creutzfeld-Jacob disease

• New-variant

– mean age 29 (15-73)

– depression, withdrawal, anxiety

– persistant painful sensory symptoms

– after 6m - ataxia, chorea, dystonia, myoclonus

– progressive dementia late stage

– survival mean 14 months (some 3yrs+)

– MRI - pulvinar sign in 90% (high signal in posterior thalamus)

– Tonsil biopsy

REFERRALS OF

SUSPECT CJD

DEATHS OF DEFINITE AND PROBABLE CJD

Year Referral

s

Year Sporadic Iatrogeni

c

Familial GSS vCJD Total

Deaths

1990 [53] 1990 28 5 0 0 - 33

1991 75 1991 32 1 3 0 - 36

1992 96 1992 45 2 5 1 - 53

1993 79 1993 36 4 5 2 - 47

1994 119 1994 54 1 5 3 - 63

1995 87 1995 35 4 2 3 3 47

1996 133 1996 40 4 2 4 10 60

1997 163 1997 60 6 4 2 10 82

1998 155 1998 64 3 3 2 18 90

1999 170 1999 62 6 2 0 15 85

2000 178 2000 50 1 2 1 28 82

2001 179 2001 58 4 4 2 20 88

2002 163 2002 72 0 4 1 17 94

2003 162 2003 79 5 4 2 18 108

2004 114 2004 50 2 4 2 9 67

2005 124 2005 67 4 8 5 5 89

2006 112 2006 69 1 6 3 5 84

2007 119 2007 64 2 9 1 5 81

2008 150 2008 88 5 2 3 2 100

2009 153 2009 79 2 3 5 3 92

2010 150 2010 85 3 6 1 3 98

2011 153 2011 90 3 11 2 5 111

2012* 50 2012 32 0 2 0 0 34

Total

Referral

2937 Total

Deaths

1339 68 96 45 176 1724

Creutzfeld-Jacob disease

• Sporadic

– peak 70s

– rapidly progressive dementia, ataxia,

myoclonus

– occasionally cortical blindness

– 50% survive 4 months, 5% survive 2 years

– EEG 2/3 periodic sharp wave complexes

– LP +ve 14-3-3 protein

EEG

F

C

B

Typical MRI appearances

in CJD

s CJD nv CJD

An introduction to EEG:

Case 9

• 42 year old R handed housewife

• Previous alcohol dependance with withdrawal

seizures. Reported to be abstinent for 3 yrs

• Admitted with generalised tonic-clonic seizure

24 hrs before.

Case 9

• Husband says she had been behaving

strangely for a few days. One episode 2

days before where she had turned up at the

train station in her pyjamas, not really

knowing what she was doing there. She had

also complained of some headaches.

On examination:

• Agitated, confused and uncooperative

• Apyrexial, no neck stiffness

• Bilateral mild papilloedema

• No focal neurological signs

• Bloods normal except CRP 49

• CXR/ECG normal

Investigations

• CT head showed L temporal haemorrhage

Investigations

• CT head showed L temporal haemorrhage

• EEG showed diffuse slowing with unilateral

temporal periodic epileptiform discharges

Investigations

• CT head showed L temporal haemorrhage

• EEG showed diffuse slowing with bilateral

temporal periodic epileptiform discharges

• LP (neurosurgeons) showed lymphocytic

pleocytosis and elevated protein. PCR for

HSV I was positive

Investigations

• CT head showed L temporal haemorrhage

• EEG showed diffuse slowing with bilateral

temporal periodic epileptiform discharges

• LP (neurosurgeons) showed lymphocytic

pleocytosis and elevated protein. PCR for

HSV I was positive

• DIAGNOSIS: Herpes simplex encephalitis

Progress

• Became comatose and developed a swinging

pyrexia. On ITU for 2/52

• Further imaging showed bilateral temporal lobe

changes and diffuse brain swelling (actually

visible on original scan)

• Not given aciclovir until 5/7 after her admission

• Left with permanent severe memory and cognitive

problems including global aphasia

Herpes Simplex Encephalitis

• Commonest cause of encephalitis (still rare)

• Difficult to diagnose with CT

• If in doubt, treat! (High dose aciclovir)

• Disability correlates well with time left

untreated. Mortality 70% without treatment

(20% with).

• Long term memory problems are common

MRI appearances:

New Immunological tests:

Case 10

• 21-year old college student and keen horse-

rider

• Feeling unwell for 2 weeks with headaches

and low mood. Became nervous and

withdrawn. Stopped attending college. Became

paranoid and confused.

• Admitted to hospital after prolonged tonic-

clonic seizure

Case 10

• CT scan normal

• LP showed 34 lymphocytes. –ve PCR for

HSV. Negative culture.

• MRI scan normal

Case 10 – progress:

• Over next few days was confused and

agitiated. Did not recognise father. Tried to

escape the ward on a number of occasions.

Tried to climb onto roof. Tearful and

anxious. Little meaningful communication.

• Lapsed into catatonia with frequent

stereotyped arm movements mistaken for

epilepsy (but EEG normal).

Diagnosis?

• Anti-NMDA antibodies positive

• Treated with Intravenous high dose steroids follwed by 5 days of immunoglobulin

• CT of Chest/abdo/pelvis normal at presentation, 6 months and 1 year

• Full recovery within 2 weeks of treatment. Back to full-time college and horse-riding within 3 months

Diagnosis: Case 10

• Sporadic anti-NMDA receptor antibody

mediated limbic encephalitis

Autoantibody Effect Cause Treatment

Anti-NMDA Behavioural disorders,

psychosis, seizures, movement

disorders

Sporadic or ovarian

teratoma

Steroids, IVIG,

cyclophosphamide

Tumour resection

Anti-GAD Amnesia, epilepsy and

cognitive impairment.

Stiff person syndrome

Sporadic Steroids, IVIG,

cyclophosphamide

Voltage-gated potassium

channels

Amnesia, seizures and

psychiatric disturbance

Sporadic Steroids, IVIG,

cyclophosphamide

Anti-Hu

Anti-CV2

Anti-CRMP5

amnesia and neuropsychiatric

disturbances,

Neuropathy

Small cell lung

carcinoma

Tumour resection

Anti-Ma2 Limbic or brainstem

encephalitis

Testicular teratoma Tumour resection

Anti-Ri Opsoclonus/ myoclonus Lung, Breast

carcinoma

Tumour resection

Anti-basal Ganglia

antibodies

Encephalitis lethargica,

Catatonia, Adult onset tic

disorder, Sydenhams chorea,

PANDAS

Post streptococcal

infection

Not established

Summary

• History taking is the most useful part of the assessment. Should always include occupation, family history etc.

• Examination important in every case (don`t forget the eyes!)

• Investigations useful in patients with acute neurological symptoms include MRI, EEG and serology