How can the brain go wrong?

Question 1

What is the central nervous system divided into?

Answer 1

Brain (encephalopathy)
Spine (myelopathy)

Question 2

What is the peripheral nervous system divided into?

Answer 2

-Roots (radiculopathy)
-Plexus (plexopathy)
-Nerves (neuropathy)
-Neuromuscular junction
-Muscle (mypoathy)

Question 3

What is involved in neurological localisation?

Answer 3

-Where is the lesion?One location, multi-focal or diffuse?
-Central vs peripheral?
-more precise location
-Based on examination and findings

Question 4

Outline the acronym VITAMIN C DEF (the ‘surgical sieve’)

Answer 4

Vascular
Infective
Trauma
Autoimmune
Metabolic
Iatrogenic
Neoplasm
Congenital
Degenerative
Endocrine/Environment
Functional

Question 5

What could be the cause of sudden disease processes?

Answer 5

-Vascular
-Trauma

Question 6

What could be the cause of subacute disease processes?

Answer 6

-Infection
-Inflammation

Question 7

What could be the cause of chronic (progressive) disease processes?

Answer 7

-Degenerative
-Neoplastic

Question 8

What could be the cause of chronic (fluctuating) disease processes?

Answer 8

-Metabolic
-Inflammation

Question 9

What are paroxysmal disease processes?

Answer 9

-Vascular
-Migrainous
-Epileptic

Question 10

What would investigations include?

Answer 10

-Bloods
-Imaging (CT/MRI)
-neurophysiology
-CSF examination

Question 11

Case study:21yo woman
* Doing military fitness on Hampstead Heath
* Sudden onset of tingling and pain down left side of body
* Very emotional
* Examination: reduced sensation, left extensor plantar

What is the origin/cause?

Answer 11

Dx: Vascular
Stroke
Right thalamic infarct caused by vertebral artery dissection
“Stroke” = sudden onset of neurological signs with no cause other than vascular

Question 12

Case study: 55 year old man, living in
Spain
* Subacute onset altered behaviour and poor memory
* Grandiose
* Elated
* Amnestic
* No problem with retrograde
memory
* Working memory normal
* Unable to learn new information

What is the origin/diagnosis?

Answer 12

Dx: VDRL positive in
serum and CSF
Tertiary syphilis
Treated with IV penicillin
Some improvement but left with severe memory deficit

Origin: infectious

Question 13

Case study: 63 year old man
* Few weeks of headache, confusion, fever
* 2 days facial droop, double vision

What is the cause/origin?

Answer 13

Dx: Basal meningitis
Infective: bacterial (including TB) fungus
Neoplastic
CSF
Opening pressure 35
Protein 2.4g
Glucose 1.5 (5.2)
WCC 150 (lymphocytes)

Origin: infectious, often subacute onset, fever, systemic symptoms

Question 14

Case study:

Fall from own height onto
concrete Feb 13
* Loss of consciousness 5 minutes, ?PTA
* Traumatic SAH, small frontal contusion
* Managed conservatively
* Problems Apr 17
* Headaches
* Possible seizures
* Cognitive and behavioural symptoms
* Vestibular symptoms
* Anxiety

What is the cause/origin?

Answer 14

Dx: TBI
Severity
* Conscious level at presentation
* GCS
* Duration of post- traumatic amnesia
* Mechanisms of injury * Bleeding
* Contusion
* Diffuse axonal injury
* Functional and psychological symptoms

Origin: Trauma

Question 15

Case study:

23 year old woman
* Aug 16: Numbness and pain R arm * Nov 16: Double vision 2/52
* Feb 17
* Spreading numbness L arm
* Blurred vision L eye, colours less
bright
* Feeling extremely anxious

What is her diagnosi/cause of disease?

Answer 15

Dx: Multiple Sclerosis

• Immune-mediated disorder causing inflammation in the CNS
• “Dissemination in time and space”
• New disease modifying therapies

Origin: autoimmune

Question 16

Case study:

25 year old woman
* Acute onset of behavioural disturbance and hallucinations
* Then became less responsive with unusual movements

What is her diagnosi/cause of disease?

Answer 16

Dx:
NMDA receptor encephalitis
Treated with steroids and plasma exchange
Ovarian teratoma (Oophorectomy)

Origin/Cause: Autoimmune

Question 17

What are examples of autoimmune diseases?

Answer 17

R-Systemic autoimmune diseases
SLE (“lupus”)
Sjogren’s syndrome
Rheumatoid
-Parainfectious processes e.g. ADEM
Autoimmune diseases of the nervous system
Antibody mediated * NMDAR encephalitis
VGKC encephalitis
Neuromyelitis optica
-Complex immune- mediated
-Multiple sclerosis
-Primary angiitis of the CNS
-Paraneoplastic processes -extracellular antigens e.g. NMDA -onconeural antigens e.g. anti-Hu

Question 18

Case study:

55 yo man under medical team
* Alcoholic liver disease * Hypertension
* Diabetes
* Developed odd movements
* Confused
* Fluctuating attention * “Delirium”
Bloods: liver enzymes normal but albumin low
* Ammonia 80

What is the cause/origin?

Answer 18

Dx: Metabolic disturbance
Common: metabolic disturbance
in systemic disease, e.g. sepsis,
electrolyte disturbance, liver and kidney failure

Rare:
Inherited metabolic diseases Congenital
e.g. Wilson’s and Mitochondrial disease

Origin/Cause: metabolic

Question 19

Case study:

60 yo man with schizophrenia, on risperidone
* Tremor of right hand
* Slowing, shuffling gait
* Rigidity and bradykinesia R>L
* Akinetic rigid syndrome = basal ganglia
* Parkinson’s disease versus extra-pyramidal side effects
* Dopamine transporter SPECT

What is the cause/origin?

Answer 19

Dx: Iatrogenic disease
Common, drugs can cause almost any neuropsychiatric symptom:
Psychotic symptoms
Depression
Parkinsonism
Anxiety
Seizures
Non-pharmacological
Radiotherapy
Surgery
Psychological

Question 20

Case study:
70 yo woman with severe coeliac disease
2 weeks of subtle cognitive symptoms, e.g. struggling with crossword, mis-spelling words
skin rash, clouding of vision

What is origin/cause of disease?

Answer 20

Dx: T cell lymphoma
Skin and brain biopsies
Chemotherapy

Origin/cause: neoplasm

Question 21

Cancer and the nervous system: what are primary tumours of the nervous system?

Answer 21

Benign-meningioma
malignant-glioma
-Spread of cancer to the nervous system: solid metastases and malignant meningitis
-Paraneoplastic processes -extracellular antigens e.g. NMDA -onconeural antigens e.g. anti-Hu

Question 22

Case study:

24 year old woman
* Attacks since childhood
* Unresponsive
* Chewing and fiddling movements
* Sometimes collapses with jerking and incontinence
* Epilepsy: complex partial seizures with secondary generalisation
* No response to medication

What is the origin/cause?

Answer 22

Dx: Focal onset epilepsy
Developmental anomalies * Acquired brain lesions
Genetic
Cryptogenic

Question 23

What are congenital disorders in neurology?

Answer 23

Congenital disorders in neurology
* Neurodevelopmental disorders * Genetic disorders
* Monogenic / Mendelian * Autosomal recessive
* Autosomal dominant
* X-linked
e.g. Wilson’s disease e.g. Huntington’s disease e.g. Fragile X
Metabolic Degenerative
* Complex genetic disorders

Question 24

Case study:

50 year old man, wife concerned about altered behaviour
* More obsessional, “rigid” * Irritable
* Inappropriate remarks
* Sweet tooth
* ACE-III
* Grasp reflex but no other signs

What is the origin/cause?

Answer 24

Dx: Atrophy with frontal
predominance on MRI
Genetic testing for C9ORF72 positive

Origin/cause: degenerative

Question 25

What are neurodegenerative diseases?

Answer 25

Neurodegenerative diseases
Progressive degeneration and death of nerve cells
* Clinical syndromes
* Dementia
* Alzheimer’s
* Dementia with Lewy Bodies * Fronto-temporal dementia
* Neuropathology * Tau
* Amyloid beta
* Alpha synuclein * TDP43
* etc
* Parkinsonian disorders * Parkinson’s disease
* MSA
* PSP
* Huntington’s disease
* Motor neurone disease

Question 26

Case study:

33 year old woman
* Involuntary fidgety movements over last few months
* Also some weight loss, anxiety, palpitations, feeling hot and itchy
* Mild chorea, pulse 110, goitre

What is the origin/cause?

Answer 26

Dx: endocrine disorders
Uncommon but important
Systemic features often provide a clue
Usually easily diagnosed with blood tests
Corticosteroid treatment can cause almost any neurosychiatric symptom

Question 27

Case study:

21 year old woman
* 7 months ago
* Episodic dizziness, flashing lights,
numbness.
* 5 months ago
* Gradually worsening pain and fatigue
* Unsteadiness, involuntary jerks, muscle spasms, slowness of thinking
* Worse with exertion
* Housebound, not in touch with any
friends
* 1 month increasing weakness
* Confined to bed
* Eventually unable to feed herself, needing help with toileting

What is the cause/origin?

Answer 27

Dx: Functional neurological symptoms
Common
Real, not imagined or feigned
Not caused by neurological damage but by altered processing in complex brain networks
Can be diagnosed using specific features on examination

Question 28

What is diagnosis based on?

Answer 28

History (NB time course, systemic symptoms)
Examination (localising the lesion)
Investigations (including blood tests, imaging, neurophysiology…)