How can the brain go wrong? Flashcards
What is the central nervous system divided into?
Brain (encephalopathy)
Spine (myelopathy)
What is the peripheral nervous system divided into?
-Roots (radiculopathy)
-Plexus (plexopathy)
-Nerves (neuropathy)
-Neuromuscular junction
-Muscle (mypoathy)
What is involved in neurological localisation?
-Where is the lesion?One location, multi-focal or diffuse?
-Central vs peripheral?
-more precise location
-Based on examination and findings
Outline the acronym VITAMIN C DEF (the ‘surgical sieve’)
Vascular
Infective
Trauma
Autoimmune
Metabolic
Iatrogenic
Neoplasm
Congenital
Degenerative
Endocrine/Environment
Functional
What could be the cause of sudden disease processes?
-Vascular
-Trauma
What could be the cause of subacute disease processes?
-Infection
-Inflammation
What could be the cause of chronic (progressive) disease processes?
-Degenerative
-Neoplastic
What could be the cause of chronic (fluctuating) disease processes?
-Metabolic
-Inflammation
What are paroxysmal disease processes?
-Vascular
-Migrainous
-Epileptic
What would investigations include?
-Bloods
-Imaging (CT/MRI)
-neurophysiology
-CSF examination
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?
Dx: Vascular
Stroke
Right thalamic infarct caused by vertebral artery dissection
“Stroke” = sudden onset of neurological signs with no cause other than vascular
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?
Dx: VDRL positive in
serum and CSF
Tertiary syphilis
Treated with IV penicillin
Some improvement but left with severe memory deficit
Origin: infectious
Case study: 63 year old man
* Few weeks of headache, confusion, fever
* 2 days facial droop, double vision
What is the cause/origin?
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
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?
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
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?
Dx: Multiple Sclerosis
- Immune-mediated disorder causing inflammation in the CNS
- “Dissemination in time and space”
- New disease modifying therapies
Origin: autoimmune
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?
Dx:
NMDA receptor encephalitis
Treated with steroids and plasma exchange
Ovarian teratoma (Oophorectomy)
Origin/Cause: Autoimmune
What are examples of autoimmune diseases?
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
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?
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
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?
Dx: Iatrogenic disease
Common, drugs can cause almost any neuropsychiatric symptom:
Psychotic symptoms
Depression
Parkinsonism
Anxiety
Seizures
Non-pharmacological
Radiotherapy
Surgery
Psychological
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?
Dx: T cell lymphoma
Skin and brain biopsies
Chemotherapy
Origin/cause: neoplasm
Cancer and the nervous system: what are primary tumours of the nervous system?
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
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?
Dx: Focal onset epilepsy
Developmental anomalies * Acquired brain lesions
Genetic
Cryptogenic
What are congenital disorders in neurology?
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
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?
Dx: Atrophy with frontal
predominance on MRI
Genetic testing for C9ORF72 positive
Origin/cause: degenerative
