Multiple Sclerosis (MS) Motor Neurone Disorder (MND)
Type IV hypersensitivity/cell-mediated autoimmune disorder characterised by Neurological disease of unknown cause
demyelination in the CNS Can present w/ both UMN + LMN signs
More common at higher latitudes e.g. Northern + Southern poles (Vit D Rarely presents < 40 yrs
deficiency?) Types
M : F (1:3) Amyotrophic lateral sclerosis
Peak onset 20-40 yrs LMN signs in arms + UMN signs in legs
Pathogenesis Gene responsible lies on Ch21 codes for superoxide dismutase
Myelin produced by oligodendrocytes sensory, motor + cognitive problems Primary lateral sclerosis
T cells infiltrate BBB + release cytokines (IL-1, IL-6, TNF-α, INF-γ B cells + UMN signs only
macrophages join + attack oligodendrocytes Progressive muscular atrophy
Regulatory T cells reduce attacks + cause remyelination
LMN signs only affects distal muscles before proximal, carries best
Signs + symptoms
prognosis
Lethargy (75%)
Visual optic neuritis, optic atrophy, Uhthoff’s phenomenon (rise in Progressive bulbar palsy
temp worsening vision), internuclear ophthalmoplegia Palsy of tongue, muscles of chewing/swallowing + facial muscles
Sensory pins + needles, numbness, trigeminal neuralgia, Lhermitte’s due to loss of function of brainstem motor nuclei, carries worst
syndrome (paraesthesia in limbs on neck flexion) prognosis
Motor spastic weakness: commonly seen in legs Dx
Cerebellar ataxia, tremor - Fasciculations?
Others urinary incontinence, sexual dysfunction, intellectual - Absence of sensory signs/ symptoms?
deterioration - Mixture of UMN + LMN signs
Types - Wasting of the small hand muscles/tibialis anterior is common
Relapsing-remitting (85%) acute attacks (1-2 months) followed by
periods of remission. Residual damage remains after each attack Other features
Secondary progressive describes RRMS patients who develop Doesn’t affect external ocular muscles
neurological signs + symptoms between relapses No cerebellar signs
Primary progressive (10%) progressive deterioration from onset, Abdo reflexes are usually presented + sphincter dysfunction if
common in elderly present (late feature)
Progressive- relapsing (steady decline since onset with Ix Electromyography Low AP + High amplitude/ MRI
superimposed attacks
Mx
Mx (decrease frequency + duration of attacks)
1. Riluzole
Acute relapse: high-dose steroids (oral/IV methylprednisolone)
DMARDs: Reduce relapse rate by 30% Prevents stimulation of glutamate receptor used mainly in
Criteria amyotrophic lateral sclerosis
RRMS + 2 relapses in the past 2 yrs + 100m walk unaided Prolongs life by 3 months
SPMS + 2 relapses in the past 2 yrs + 10m (aided/unaided) 2. Respiratory care
Specific problems Non-invasive (BIPAP) @ night
Tiredness Rx Amantadine
Bladder Rx USS, ACh, intermittent catheter Prognosis 50% die within 3 yrs
Spasticity Rx Baclofen + Gabapentin
Oscillospia Rx Gabapentin
Type IV hypersensitivity/cell-mediated autoimmune disorder characterised by Neurological disease of unknown cause
demyelination in the CNS Can present w/ both UMN + LMN signs
More common at higher latitudes e.g. Northern + Southern poles (Vit D Rarely presents < 40 yrs
deficiency?) Types
M : F (1:3) Amyotrophic lateral sclerosis
Peak onset 20-40 yrs LMN signs in arms + UMN signs in legs
Pathogenesis Gene responsible lies on Ch21 codes for superoxide dismutase
Myelin produced by oligodendrocytes sensory, motor + cognitive problems Primary lateral sclerosis
T cells infiltrate BBB + release cytokines (IL-1, IL-6, TNF-α, INF-γ B cells + UMN signs only
macrophages join + attack oligodendrocytes Progressive muscular atrophy
Regulatory T cells reduce attacks + cause remyelination
LMN signs only affects distal muscles before proximal, carries best
Signs + symptoms
prognosis
Lethargy (75%)
Visual optic neuritis, optic atrophy, Uhthoff’s phenomenon (rise in Progressive bulbar palsy
temp worsening vision), internuclear ophthalmoplegia Palsy of tongue, muscles of chewing/swallowing + facial muscles
Sensory pins + needles, numbness, trigeminal neuralgia, Lhermitte’s due to loss of function of brainstem motor nuclei, carries worst
syndrome (paraesthesia in limbs on neck flexion) prognosis
Motor spastic weakness: commonly seen in legs Dx
Cerebellar ataxia, tremor - Fasciculations?
Others urinary incontinence, sexual dysfunction, intellectual - Absence of sensory signs/ symptoms?
deterioration - Mixture of UMN + LMN signs
Types - Wasting of the small hand muscles/tibialis anterior is common
Relapsing-remitting (85%) acute attacks (1-2 months) followed by
periods of remission. Residual damage remains after each attack Other features
Secondary progressive describes RRMS patients who develop Doesn’t affect external ocular muscles
neurological signs + symptoms between relapses No cerebellar signs
Primary progressive (10%) progressive deterioration from onset, Abdo reflexes are usually presented + sphincter dysfunction if
common in elderly present (late feature)
Progressive- relapsing (steady decline since onset with Ix Electromyography Low AP + High amplitude/ MRI
superimposed attacks
Mx
Mx (decrease frequency + duration of attacks)
1. Riluzole
Acute relapse: high-dose steroids (oral/IV methylprednisolone)
DMARDs: Reduce relapse rate by 30% Prevents stimulation of glutamate receptor used mainly in
Criteria amyotrophic lateral sclerosis
RRMS + 2 relapses in the past 2 yrs + 100m walk unaided Prolongs life by 3 months
SPMS + 2 relapses in the past 2 yrs + 10m (aided/unaided) 2. Respiratory care
Specific problems Non-invasive (BIPAP) @ night
Tiredness Rx Amantadine
Bladder Rx USS, ACh, intermittent catheter Prognosis 50% die within 3 yrs
Spasticity Rx Baclofen + Gabapentin
Oscillospia Rx Gabapentin
