ANTERIOR HORN CELL DISORDERS Flashcards
⚡⚡ MOST COMMON type of MND
AMYOTROPHIC LATERAL SCLEROSIS
UMN ➕ LMN MND
Amyotrophic Lateral Sclerosis
Pure LMN MND
⭐ Spinal Muscle Atrophy
⭐ Multi focal Motor Neuropathy
Pure UMN MND
Primary Lateral Sclerosis
Paraproteinemias is ASSOCIATED with
MMN
Lou Gehrig’s Disease
Amyotrophic Lateral Sclerosis
On an ATROPHIC or WASTED LIMB, if Reflexes are ⬆️ ⬆️
Amyotrophic Lateral Sclerosis
Weakness in ALS
Late feature
DISTAL Weakness
Bulbar Palsy vs Psuedo Bulbar palsy
🧠⚡JGT SO madarchod ⚡
🧠⚡ JG ⬆️ in Psudobulbar Palsy ⚡
🧠⚡ Emotional liability (incontinence) seen in Pseudobulbar palsy⚡
Basic Difference between Bulbar and PseudoBulbar palsy
Bulbar is LMN LESION of CN 9,10,11,12 (Cranial Nerve nuclei in Brainstem undergoes atrophy)
PseudoBulbar is U/L UMN LESION of CN 9,10,11,12 (Cortico-bulbar fibers involvement)
1st SYMPTOM OF BULBAR PALSY
Nasal twang and Nasal Regurgitation
⬇️
Flaccid Tongue and Fasiculations
⬇️
Flaccid Dysarthria
Bulbar DYSARTHRIA
Flaccid Dysarthria
PseudoBulbar Dysarthria
✨ Spastic Dysarthria
✨ Donald Duck speech
✨ Hot potato speech
Bulbar Onset Dysarthria
1st SYMPTOM is DYSARTHRIA
Never involved in ALS
✨ Sensory symptoms
✨ ANS
✨ BOWEL & BLADDER
✨ Cognition
✨ Extraocular muscles
EMG in ALS
Long duration Polyphasic Fasciculation potentials ➕ Fibrillation
MRI in ALS
B/L symmetrical Hyper intensity in Corticospinal Tract
Kennedy’s disease
X-linked Spinobulbar Muscle Atrophy
✨ Pure LMN lesion
✨ Progressive weakness of limbs (Distal > Proximal) & Bukhar Muscles
✨ Androgen insensitivity
✨ Tremors ➕
✨ Trinucleotide Repeats ➕
Hirayama Disease
Benign Monomelic Amyotrophy
✨ Dura comes anteriorly & compresses the spinal cord
✨ C8-T1
✨ Upper Limb wasting, weakness & Fasiculations
✨ Presents like MND
Hirayama Disease MRI
- Atrophy (OR) Thinning of Middle & Lower Cervical Spinal Cors
- ⬆️ Intensity in Epidural Space
Madras MND
LMN > > UMN
PROXIMAL = DISTAL Involvement
Bifacial Weakness
Severe Tongue Involvement
Hearing Loss ➕