More neuro Qs Flashcards
Name all 4 clinical courses of MND and what part of the CNS they affect and therefore what symptoms develop
- ALS - Amyotrophic Lateral Sclerosis: UMN + LMN
Disease of lateral corticospinal tracts, cause weakness with UMN signs and LMN wasting; progressive spastic tetraparesis with LMN signs and fasciculation - PMA - Progressive Muscular Atrophy: LMN ONLY
Wasting begins in muscles of hands and spreads to anterior horn cells; no UMN signs; lost reflexes and widespread wasting and weakness - PBP - Progressive Bulbar Palsy: UMN & LMN OF LOWER CRANIAL NERVES
Affects cranial nerves 9 – 12, develop dysarthria, dysphagia, and nasal regurgitation of fluids and choking, can be mixed UMN/LMN signs, progresses to ALS - PLS - Primary Lateral Sclerosis: UMN
Loss of Betz cells in motor cortex, mainly UMN signs, progressive tetraparesis with terminal pseudobulbar palsy
Regardless of the body part that is first affected by ALS disease, weakness and
atrophy spread to other parts of body with varying degrees of upper motor
neuron (UMN) symptoms (e.g., spasticity) and eventually involve the muscles
of all 4 extremities and the trunk, as well as bulbar muscles.
Rectal and bladder sphincters and oculomotor muscles are usually spared.
No sensory symptoms.
Gene associated with MND
SOD1
What is the El Escorial Criteria?
El Escorial Criteria for the diagnosis of Amyotrophic Lateral Sclerosis
The diagnosis of ALS requires the presence of:
- Signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathology examination,
- Signs of upper motor neuron (UMN) degeneration by clinical examination, and
- Progressive spread of signs within a region or to other regions,
together with the absence of:
Electrophysiological evidence of other disease processes that might explain the signs of LMN and/or UMN degenerations; and
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs.
Probable = LMN and UMN signs in 2 regions
Probably + lab support = LMN and UMN signs in 1 region
Suspected = LMN or UMN signs in 1 region
Tx of MND
- Riluzole - supresses glutamate activity
- MDT approach – Neurologist, speech and language therapists, occupational therapists, Specialist Nurse, Physiotherapist etc.
- Symptomatic: e.g. baclofen for spasms, Non-invasive ventilation when needed, PEG tube for feeing when needed
UMN SX
Weakness Brisk reflexes Hypertonia Upgoing Plantars (positive Babinski) Clonus
LMN SX
Weakness Depressed/Absent reflexes Decreased tone Wasting Fasciculations
What organisms may trigger Guillian-Barre syndrome?
Campylobacter jejuni, CMV, Mycoplasma,
Zoster, EBV, HIV but no obvious infectious cause is found in 40%
What symptoms are seen in Guillian-Barre syndrome?
- 1-3 weeks post infection: symmetrical, ascending muscle weakness.
(can advance quickly and cause total paralysis) and/or numbness - Weakness begins in distal limb muscles and progresses to more
proximal muscles over 4 weeks - recovery follows this - Loss of reflexes, neuropathic pain and autonomic dysfunction
(sweating, tachycardia, arrhythmias) - In 20%, respiratory muscles and facial muscles are affected respiratoy involvement requires ITU admission.
What Ix for Guillian-Barre syndrome?
- Confirmed with nerve conduction studies (slow nerve conduction, and
prolonged distal motor latency +/- conduction block) - Lumbar Puncture: CSF- increased proteins, WCC normal
- Consider spirometry to monitor FVC - a decreased forced vital
capacity can indicate the need to admit to ITU to maintain airways.
Mx of Guillian Barre?
IV immunoglobulin (IVIg) for 5 days: decreases duration and severity of paralysis. Plasma exchange also effective but seldom used.
24 Monitor ventilation. Ultimately self-limiting but need to prevent respiratory
failure.
Encephalitis - what can cause?
Viral infection
- Herpes simplex virus 1 + 2 (most common cause in the UK), Varicella zoster virus, Epstein Barr, Cytomegalovirus, HIV, Mumps,
Measles
Non-viral causes – secondary to bacterial meningitis, TB, toxoplasma, malaria, listeria, Cryptococcus etc.
What is encephalitis?
Infection and inflammation of the brain parenchyma.
Risk Factors: Extremes of age, and immunocompromised patients
Pathophysiology: disease which mostly affected the frontal and temporal
lobes, so causes decreased consciousness, confusion, and focal signs
Clinical features of encephalitis
- Begins with features of a viral infection
- Fever, headache, myalgia, fatigue, nausea. - Progresses to
- Decreased consciousness/confusion, drowsiness
- Behavioural change
- Focal Neurological Deficit
- Seizures
- Coma - May have signs of meningitis (meningo-encephalitis= inflammation of
the brain lining + parenchyma)
Diagnosis of encephalitis
- LP (cultures + PCR study, moderate increase in protein level , lymphocytes, +/- decreased glucose)
- Others : FBC and blood film- leucocytosis
- Blood cultures, Viral PCR, U+E’s, LFT’s, glucose, ESR, CRP, May need other cultures e.g.- throat swabs, stool culture
20 - Contrast-enhanced CT/MRI scans: rule out space-occupying lesions, strokes. Identify raised intracranial pressure (a contra-indication for an LP).
MRI can show oedematous changes seen in encephalitis. - Electroencephalogram (EEG) – diffuse abnormal slow wave changes
Mx of encephalitis
- If viral - Urgent antiviral treatment (acyclovir 1st line- given high dose
IV usually for 14 days). Untreated mortality 70%, therefore
sometimes started empirically. - Supportive treatment +/- anti-seizure medications
- If meningitis is suspected- emergency IM benzylpenicillin.
What organisms can cause meningitis?
- Adults and Children : Neisseria meningitides, Streptococcus
Pneumoniae/ Pneumococcus
- Less common: Haemophilus Influenza (due to introduction of
vaccine) - Pregnant women/older adults- Listeria monocytogenes
- Neonates: Escheria coli, Group B Haemolytic streptococcus
- Immunocompromised: Cytomegalovirus, Cryptococcus (in HIV, stains with India ink), TB
* aseptic meningitis - caused by tumour
What clinical features are seen in meningitis?
Fever, headache “worst of life” , meningism - photophobia, neck stiffness, Kernigs and Burdzinski’s signs +- altered mental state, seizures
Non-blanching rash (bacterial)
What investigations are used in meningitis? what results
LP
- Microscopy
- Culture
- Glucose
Bacteria: neutrophils, raised protein, low glucose
Viral: lymph, normal protein, normal glucose
Treatment of meningitis
*Suspected Bacterial meningitis – Start Abx even before tests
- Treatment: IV Cefotaxime (3rd Generation Cephalosporin)
If in community e.g. GP, give IM Benzylpenincillin - If over 50, add Ampicillin to cover Listeria
- Consider dexamethasone to reduce cerebral oedema
Prophylaxis – Rifampicin or Ciprofloxacin
Give the 2 subtypes of high grade brain tumuors
Grade III and IV
Gliomas are the most common type - Astrocytoma and oligodenroglioma are the most common subtypes
ALSO primary cerebral lymphoma and medulloblasta
Name 2 benign/low grade brain tumours
Grade I and II
Meningioma (benign), acoustic neuroma (arise from nerve
sheath of CN VIII) Neurofibromas - from schwann cells, Pituitary tumour, Pineal tumour
What are the common sites of meningiomas?
Parasagittal region, sphenoidal region, subfrontal region, pituitary fossa and skull base
What signs and symptoms are associated with brain tumours
- Increased intracranial pressure headache (54%) - typically worse in
mornings or lying down or coughing/sneezing - Nausea and vomiting
- Seizures (26%) – more common in low grade
- Neurological focal symptoms (68%)– more common in high grade
tumours - Personality change, cognitive or behavioural symptoms, confusion
- Symptoms of raised intracranial pressure- unilateral ptosis, pupillary
changes, papilloedema on fundoscopy - Coning may occur, i.e. herniation of cerebellar tonsils through
foramen magnum - Resp depression, bradycardia, death
Where do brain mets most commonly come from?
Lung, breast, prostate, colorectal, melanoma and kidney
Mx of brain tumours
Diagnosis/Investigations: CT or MRI scan - determine size and location of
lesions (high grade have irregular edges and high growth rate)
Management: Refer to Neurosurgery (Surgical resection, radiotherapy and
supportive treatment – very dependent on tumour type)
If someone is referred to you with suspected Horner’s syndrome, what do you expect to find on examination?
Unilateral papillary constriction with slight ptosis and enophthalmos, loss of sweating on same side
