Where is the damn lesion?

CNS - Brain and spinal cord

PNS – Peripheral nerves (autonomic, sensory, motor), NMJ and muscles.

Thalamus
Cortical
Supratentorial Corona Radiata
Subcortical
Internal Capsule

Basal Ganglia

midbrain

Brain pons

medulla

Brainstem
Infratentorial
Cerebellum

Weakness of brain – hemiparesis, weakness at level of spinal cord causes paraparesis or quadriparesis
Paresis – weakness, Plegia – paralysis

Spastic paraparesis – HAM/TSP, tabes dorsalis, spinal cord compression, multiple sclerosis, SCDC, transverse
myelitis, HIV associated myelopathy.
Flaccid paraparesis – poliomyelitis, guillian barre, ALS (lou gehrigs disease), spina bifida, botulism,
tetrodotoxin, ciguatoxin. SCDC - Subacute Combined Degeneration of the Spinal Cord
- due to Vit B 12 deficiency
Risk factors: Pernicious Anemia, Post gastrectomy, Crohn’s, Tapeworm, Vegans
Combine CNS + PNS so UMN + LMN >
Symmetrical dorsal column loss (sensory + LMN) > joint position and vibration affected first
then Symmetrical corticospinal tract loss (UMN)
Classic Triad > Extensor Plantar + Brisk Knee jerks + absent ankle Jerks
Hematological signs + glossitis, stomatitis, ataxia, confusion, optic atrophy
DDX for UMN + LMN: Tabes Dorsalis, HIV,
 Stroke Mx
IVA + Foley Catheter in situ Acute Stroke Mx
No spontaneous or induced Fasiculations Stabilize - IVF, O2 therapy, Antipyretics if fever
Bulk normal Cardiac monitoring
Tone increased proximal muscle and distal muscle groups bilaterally Monitor for ICP signs
Power 0/5 in proximal muscle and distal groups bilaterally (proximal > distal) rt-PA (only < 3-4 after Sx)
Deep Tendon reflexes are exaggerated bilaterally BP in Ischemic: >140/ 90 mmhg. Only treat > 220 SBP or 120 DBP)
Babinski: up BP in Hemorrhagic: keep SBP 140-160
Clonus seen Order CT
Coordination not assessed due to reduced power VTE prophylaxis - Heparin 5000U SC b.d. (CBC & PTT) or LMWH
Gait not assessed due to reduced power When Heparin C/I then ASA
Anticoagulation (warfarin 5 mg once daily) should be started simultaneously unless C/I
I would like to do a full history and physical exam including detailed neurological exam of and then adjusted when
mental status, cranial nerves, sensory and motor for UL + LL. INR of 2.5 (2.0-3.0), heparin should be stopped.
CLOTS trial data does not support compression stockings
My diagnosis is UMN - Spastic Paraparesis due to: Infections - Prophylactic administration of antibiotics is not recommended
Discharge - Pt stable, family informed
Cortical Causes: No HTN within 48 hrs
Superior Sagital Sinus Thrombosis
Thrombosis of unpaired anterior cerebral artery Secondary Stroke Prevention:
MS of cortex and spinal cord Antiplt - ASA (30-300 mg/day) (Children ASA 3-5 mg/kg per day) or clopidogrel (75 mg
Glioma over motor area OD)
Blood pressure lowering for patients w/ TIA or stroke Hx
Compressive Myelopathies - w/ or w/o HTN Hx - diuretics or the combination of diuretics and an ACEI..
Infectious Causes - HTLV-1, HIV associated myelopathy, HSV, TB, Syphilis, Lyme Disease, Early ischemic stroke (IS), <24 hours from onset;
Dengue high-risk TIA, ABCD2 score ≥4;
Demyelination - MS, NMO, Post infection demylination, low-risk TIA, ABCD2 score <4; dual antiplatelet: ASA + clopidogrel
Vascular - Infarct- Anterior Spinal Artery thrombosis, Superior Sagital Sinus Thrombosis AFib Anticoagulation if no C/I. Heart rhythm monitoring
Embolism - Leriche’s Syndrome
Iatrogenic - after prolonged clamping of aorta during Sx
Nutritional: Pellagra, SACD ASA + clopidogrel > hemorrhage risk and is not recommended unless there is indication
Autoimmune: Lupus Sjorgen, Sarcoidosis for this therapy (i.e., coronary stent or acute coronary syndromes)
Trauma: Radiation,
Congenital - HSP ASA C/I:
Neoplastic - Hemophilia
Drugs - Intrathecal injection of MTX or Penicillin, allergy to NSAIDs and
Idiopathic in patients with asthma, rhinitis, and nasal polyps
Dengue Fever, Yellow Fever
HAM/TSP, tabes dorsalis, spinal cord compression, multiple sclerosis, SCDC, transverse Gastritis, PUD
myelitis, HIV associated myelopathy. Children w/ viral infection to avoid Reye’s Syndrome
General + Specific: Raised ICP signs:
CBC Early- positional headache and confusion
CSF late AMS, papilledema, and
Full XR or CT - bony changes Cushing triad (irregular breathing, wide PP, and HR < 60)
Nitrates are C/I - Raised ICP

Patients with TIA, non-disabling stroke, ipsilateral ICA stenosis should be offered
carotid endarterectomy or stenting < 2 weeks unless C/I

Digoxin treatment for AF is a significant risk factor of stroke

General Early Supportive Care

Vitals - BP, HR, RR, O2, GMR, Temp
ECG
Labs - CBC, U&E, PTT, INR, Troponins
Imaging - MRI/ CT to r/o Hemorrhage
ECHO to r/o

Position - bed rest

Swallowing - All conscious patients test ability to swallow w/ 50ml of water
Testing the gag reflex is invalid as a test of swallowing. NG tube for inability to swallow
Patients with AMS- only IVF (Dextrose saline or normal saline) for at least 2-3 days.

Screen for:
HTN
DM
Smoking
Carotid artery stenosis (for those with non-disabling stroke)
AFib or other arrhythmias
Structural cardiac disease
You are examining a patient with vertigo, vomiting, and nystagmus. These are your clinical examination findings:

left-sided limb ataxia:

spinocerebellar pathway localises the lesion to the ipsilateral lateral brainstem.

left-sided alteration of pain and temperature on the face:

Sensory nucleus of the 5th cranial nerve localises the lesion to the ipsilateral lateral brainstem.

left-sided ipsilateral Homer’s syndrome:Sympathetic pathway localises the lesion to the ipsilateral lateral brainstem.
right-sided alteration of pain and temperature affecting the arm and leg:
Spinothalamic pathway localises the lesion to the contralateral lateral brainstem.

dysarthria and decreased gag reflex on the left, with the palate pulling up on the right-side:
localises the lesion to the medulla affecting the ipsilateral CN9 and 10.

Site of the lesion: left lateral medulla.

Also known as Wallenberg syndrome, caused by a left vertebral or left posterior inferior cerebellar artery occlusion (blood supply is variable to this
region).

You are examining a patient with right-sided deafness, that was preceded by tinnitus. These are your clinical examination findings:\

right-sided limb ataxia (predominantly affecting the right upper limb):

spinocerebellar pathway localises the lesion to the ipsilateral lateral brainstem.

right-sided facial numbness with loss of the corneal reflex:

Sensory nucleus of the 5th cranial nerve localises the lesion to the ipsilateral lateral brainstem.

right-sided hemi-facial spasms:

the lesion involves the pons affecting the ipsilateral CN7.

Site of the lesion: The findings indicate a lesion affecting the right lateral pons with evidence of spinocerebellar involvement.
In this case the lesion was not vascular in origin but in fact an example of a cerebellopontine angle lesion – an acoustic neuroma (or schwannoma)

Brainstem Rule of 4s
A medial brainstem syndrome will consist of the 4 M’s and the relevant motor cranial nerves, and a lateral brainstem syndrome will consist of the 4 S’s
and either the 9-11th cranial nerve if the lesion is in the medulla, or the 5th, 7th and 8th cranial nerve if the lesion is in the pons.

A bulbar palsy is a lower motor neuron lesion of cranial nerves IX, X and XII.
Causes:
Motor neurone disease
Syringobulbia
Guillain-Barre syndrome
Poliomyelitis
Subacute menignitis (carcinoma, lymphoma)
Neurosyphilis
Brainstem CVA

Features:
Gag reflex – absent
Tongue – wasted, fasciculations
“wasted, wrinkled, thrown into folds and increasingly motionless”.
Palatal movement – absent.
Jaw jerk – absent or normal
Speech – nasal
“indistinct (flaccid dysarthria), lacks modulation and has a nasal twang”
Emotions – normal
Other – signs of the underlying cause, e.g. limb fasciculations.

A pseudobulbar palsy is an upper motor neuron lesion of cranial nerves IX, X and XII.
Causes:
The commonest cause is bilateral CVAs affecting the internal capsule.

Multiple sclerosis
Motor neurone disease
High brainstem tumours
Head injury

Features:
Gag reflex – increased or normal
Tongue – spastic
“it cannot be protruded, lies on the floor of the mouth and is small and tight”.
Palatal movement – absent.
Jaw jerk – increased
Speech – spastic: “a monotonous, slurred, high-pitched, ‘Donald Duck’ dysarthria” that “sounds as if the patient is trying to squeeze out words from
tight lips”.
Emotions – labile
Other – bilateral upper motor neuron (long tract) limb signs.
CAUSES OF HEMIPLEGIC
Acute stroke: ischemic, hemorrhagic, or subarachnoid hemorrhage
Intracranial mass
Meningitis/encephalitis
Hypoglycemia/hyperglycemia
Postictal Todd’s paresis
Hemiplegic migraine
Brown-Sequard syndrome

Quadriparesis/Paraparesis ± Sensory Level

Spinal cord compression

Spinal cord infarction
Transverse myelitis

PROXIMAL WEAKNESS
Acute myopathy
Guillain–Barré syndrome (GBS)
Diabetic lumbosacral radiculoplexus neuropathy (DLRN)
Myasthenia gravis
Lambert-Eaton myasthenic syndrome (LEMS)

DISTAL WEAKNESS
Vasculitic neuropathy
Toxin induced peripheral neuropathy
Nerve compression syndromes

INVESTIGATIONS
Laboratory
glucose, electrolytes, Ca, Mg, PO4, UEC, LFTs, and coags
TFTs, CK, ESR
others according to suspected etiology: e.g. CSF, vasculitis screen
Imaging (according to suspected etiology)
CTB, CTB +/- contrast +/- angiogram +/- venogram
MRI brain +/- angiography
CT or MRI spine

MANAGEMENT
Airway and respiratory management

Assess for intubation (see factors to consider, above)

key life threats are:
airway obstruction due to oropharyngeal collapse
respiratory failure due to diaphragmatic weakness
respiratory failure due to aspiration from inadequate airway protection
Non-invasive ventilation
Consider as a temporizing measure in a neurologically stable patient with a neuromuscular condition expected to have rapid resolution (e.g., myasthenia gravis exacerbation)
Consider use for pre-oxygenation prior to intubation
Avoid suxamethonium if there is evidence of underlying progressive neuromuscular disease (e.g., Guillain-Barre Syndrome, chronic muscular weakness, or prolonged immobility) —
use rocuronium (1.2 mg/kg IV IBW) for rapid sequence intubation

Myasthenia gravis
Succinylcholine is relatively ineffective, double the standard dose if used (e.g. 3 mg/kg IV)
Alternatively, use half-dose of non-depolarizing agents (e.g. rocuronium 0.6 mg/kg IV IBW)
If autonomic instability is present or anticipated
Prepare atropine/glycopyrrolate, fluids, and vasopressors prior to intubation
anticipate swings and avoid overshoot (i.e. don’t chase hypotension too aggressively with vasopressors, as there may be a dramatic swing to severe hypertension as a result of
autonomic instability)
Specific therapy - according to underlying cause
Supportive care and monitoring

MCA - CL weakness and parathesia in UL > LL, Gaze deviates toward the side of infarction
Contralateral homonymous hemianopia without macular sparing or superior/inferior quadrantanopia
Aphasia if dominant hemisphere (Lt MCA)
Hemineglect if nondominant hemisphere ( Rt MCA)

ACA - CL weakness and parathesia in LL > UL , Abulia, Urinary incontinence, Dysarthria, Transcortical motor aphasia, Frontal release signs, Limb apraxia

AICA - Horner syndrome, facial paralysis and droop, decreased taste anterior ⅔ tongue, decreased facial sensation for pain and temperature vertigo, audiovestibular dysfunction,
and ataxia.

PICA Lateral medullary syndrome (Wallenberg syndrome - ataxia, vertigo, nystagmus, dysphagia, dysphonia, IPSI Horner syndrome and facial paresthesia, and CL limb
paresthesia.

VA- Lateral medullary syndrome, Medial medullary syndrome, Neck pain, Signs of PCA infarction

CCA - Horner syndrome, Signs of MCA infarction

ICA - Ipsilateral amaurosis fugax (transient vision loss), Dysphagia, tongue deviates to ipsilateral side (CN XII)
Numerous contralateral symptoms can occur (e.g., hemiparesis, hemisensory loss, homonymous hemianopsia).
s a branch of the intracranial internal carotid, arising distal to the ophthalmic artery but below the circle of Willis. Its size compares with
that of cerebral surface artery branches, but its location makes it an uncommon site of occlusion that causes stroke.
 Cortex
The hallmark of a cortical lesion are Cortical deficits

1. Loss of executive function, memory, planning, judgement, neglect, graphaesthesia, astereognosis, vision loss
2. Language dysfunction/aphasia {FRENCH – fluency, reading/repeating, naming, comprehension, handwriting)
Broca (expressive aphasia) – repeating, naming
Wernicky (receptive aphasia) – unable to name or repeat words, neologism, word substitution
3. seizures
4. altered level of consciousness – can also be due to a lesion of the reticular activating system of the midbrain.
5. Face, arm and leg weakness all on the opposite (contralateral) side of the lesion { based on homunculus and
vascular territories MCA – face/arm weakness > legs and ACA – leg weakness > face weakness}
6. visual field defect and gaze preference – left frontal eye field destruction -> unopposed action of the right
frontal eye field (responsible for the eye looking left) so in ischemic stroke the frontal eye field is destroyed
and = the eye on the opposite side looks toward the lesion. In hemorrhagic stroke, blood irritates the frontal
eye field and causes overactivity of the frontal eye field so eyes look away from the lesion.

Involvement of cortical sensation – involvement of the dominant parietal lobe. MCA divides into two branches so a
global aphasia must involve MCA before division. haemorrhage, infarct, infection, tumour

SubCortex
The hallmark of a subcortical lesion absence of cortical deficits (patient is wide
awake and can converse well ie higher mental function is preserved but there is
face and leg weakness)
Small vessel disease (usually lacunar infacts from HTN)

Corona radiate fibres are splayed out and have lots of supplying arteries so usually produces only mild weakness

Internal capsule fibres are closely packed together and very dense producing a dense hemiparesis. There are small
end arteries which supply the area (poor supply) as well as poor collateral circulation to the area.

Corticobulbar – upper motor neuron goes down to cranial nerve nuclei. Upper face has dual representation on cortex
CN VII (frontalis and orbicularis oculi muscle) – so if there is stroke on one side, the other side has responsibility for
the upper face. LMN pattern – final output to muscle so if the lesion is here then everything supplied by this will not
work.
therefore UMN CNVII palsy – weakness of the lower face (buccinators and orbicularis oris muscle has single rep while
the ones mentioned above are spared). In a LMN CVII palsy there is weakness of the entire side of the face
unilaterally.
CNVII is found in the pons and a lesion here or at any other point that the nerve travels can give LMN from brainstem
to cerebellopontine angle to vestibular fossa (acoustic schwanoma), internal acoustic meatus (basal skull fracture),
meningitis (inflames vessels supplying nerve) and parotid gland tumors and swellings can cause CNVII injury not just
bells palsy.
Real bells palsy is an ipsilateral LMN of CNVII due to viral clearing of HSV after nerve infection
Ramsay Hunt syndrome type II is the reactivation syndrome of herpes zoster in the geniculate ganglion. It has variable
presentation which may include a lower motor neuron lesion of the facial nerve, deafness, vertigo, and pain.

Brainstem
motor (corticospinal pathway) localises the lesion to the contralateral medial brainstem
loss of vibration and proprioception in the left upper and lower limbs:
confirms localisation of the lesion to the contralateral medial brainstem
The hallmark of a brainstem lesion are crossed signs. Ipsilateral cranial nerve
neuropathy with contralateral hemiparesis

(CN system is ipsilateral and does not cross)

sparing of CN3 and CN6 means the midbrain and pons are not involved
sparing of the face (CN7) means the lesion must be below the upper pons. involvement of the face means the lesion must be at or above the upper pons
tongue deviation indicates CN12 involvement, localising the lesion to the ipsilateral medulla
Symptoms of cranial nerve disease are telling us about the brainstem
haemorrhage, infarct, infection, mass lesion

Cortex, Subcortex, brainstem lesions etiology vascular(stroke), tumors (glioma), trauma, infection (meningitis,
encephalitis), inflammatory (CNS casculitis),
CN3 involvement, localising the lesion to the ipsilateral midbrain
(sparing of CN6 and CN12 means the pons and medulla are not involved)

Spinal Cord
Hallmark is distal, symmetrical, spastic, weakness with a sensory level (UMN)
Sphincter dysfunction – bowel incontinence, bladder incontinence, urinary retention (autonomic system off). Once
there is involvement of the corticospinal system, the GABAergic inhibitory modulating mechanisms are decreased but
the peripheral nerve is spared with normal resting depolarization and growth factors so keeps muscle bulk and tone.
Unless the injury is longstanding there is no significant atrophy or fasciculations.

REMEMBER THAT THE SENSORY LEVEL OCCURS ON THE TRUNK!

The dermatomal level is 2 levels lower than the spinal level (ie derm T8 corresponds to spinal level T6)
etiology of spinal cord lesions – trauma, tumor(primary vs mets), inflammation (transverse myelitis), degenerative
disk disease (cervical spondylosis), non compressive myelopathy (TSP, tabes dorsalis, SCDC, hiv associated
myelopathy). (General rules from Gilbert for the Motor exam. However not always true e.g in brown sequard syndrome with hemisection of the
cord corticospinal tract findings are not symmetrical. And also TSP affects spinal cord and causes proximal weakness. Always
weird and wonderful exceptions in all things medicine.)

Lower Motor Neuron Disease

LMN stars at the anterior horn cells to the actual muscle
Disorders of the anterior horn cell (AHC) can either be acquired or inherited.
spinal muscular atrophy, poliomyelitis (to the Piccornaviridae family, in the genus Entrovirus serotype 1 2 3 ) and amyotrophic lateral sclerosis.
Muscular atrophy, severe weakness, and fasciculations without sensory deficit are signs of anterior horn cell disease. These signs may be seen alone
or in combination with upper motor neuron signs (hyper-reflexia, upgoing toes) in the case of ALS. Sensory disturbances are absent. When sensory
function is impaired in conjunction with anterior horn cell disease, dysfunction of adjacent tracts of the spinal cord or the peripheral nerves may be
present.
 Nerve Root
Intervertebral disc herniation and spinal stenosis
Chronic compression produces severe demyelination and fibrosis of the nerve root. Similar
lesions can result from exposure to irritant substances released from the nucleus pulposus.
The hallmark is radiculopathy/pinched nerve – lancing shooting pain down the
distribution of the nerve and asymettrical weakness.
Loss of reflex served by the myotome of the nerve involved. Commonest is L5/S1 and C5/C6
motor weakness and altered sensibility or pain due to ischemia & intraneural edema

Peripheral Neuropathy
The hallmark is distal limb weakness and sensory loss. May be symmetrical or
ipsilateral. Fasciculations, atrophy, hyporeflexia/absent reflexes. Can be
autonomic, sensory or motor nerve involved.
There are positive and negative sensory signs positive is paraesthesia, burning pain. Negative signs – loss of sweating

Autonomic -> gastroparesis (bloating, feeling full and food coming back up/regurgitation), erectile dysfunction,
postural hypotension.

Can be in a glove and stocking distribution (commonest type) Guillain-Barre Syndrome

Etiology – DM and any endocrinopathy (hypo/hyperthyroidism), B12, B6 neuropathy, chemotherapy, alcohol,

paraprotienemia (multiple myeloma), end organ failure (renal and hepatic failure) and heavy metals,

Neuromuscular Junction
The hallmark is proximal weakness with fatiguability. CHAIR HAIR STAIRS!
Have difficulty getting out of a chair, have difficulty combing hair, walking up stairs, hanging clothes on a line.

Patient is OK in the morning but gets weaker as the day progresses and is better with rest but worsens with activity.
Early involvement of the facial and bulbar muscles. Prototype is myasthenia gravis
MG Types:
Congenital - AChE deficiency Ocular MG
Acquired - Botulism, LEMS, Organophosphates, tick bite, MG, Snake Venom Generalized MG

Myopathy Thymomatous MG
Seronegatuve MG

The hallmark is proximal limb weakness, muscle pain or tenderness, preserved

sensation and preserved bulk

Can be symmetrical or non symmetrical.

Congenital
Acquired
Etiology – inflammatory (polymyositis and dermatomyositis), Infectious (viral associated myopathy), Autoimmune
(SLE), Drug Induced (Alcohol, statins, steroids), Endocrinopathy (hypothyroidism DM, cushings disease, addisons
disease, acromegaly, Conns syndrome), muscular dystrophies, metabolic (hypocalcemia, hypokalemia)

in the acute phase, UMN lesions may be difficult to differentiate from a LMN lesion
acute UMN lesions may result in flaccid paralysis, normal or reduced tone, and unreliable reflexes
in acute LMN lesions there may be insufficient time for atrophy to be evident and fasciculations are rarely seen