Brian's Cards Flashcards
Pes Cavus (Description + Pathology)
Description:
- High arch of the sole of foot, claw toes
- Some degree of atrophy of muscles
Pathology
- Long standing LMN peripheral neuropathy (think CMT)
- Freidrich’s ataxia
- Spinocerebellar problems
Charcot Marie Tooth
PES Cavus, foot drop, distal muscle atrophy in legs > arms, diminished reflexes, sensory neuropathy
Autosomal dominant
Suspect in people in 20s/30s
Type of Hereditary motor and sensory neuropathy
- Pes Cavus
- Foot drop due to weakness (high steppage gait, orthotics)
- Distal muscle weakness and distal atrophy in legs > arms (inverted champagne bottle) - below elbow and middle third of thigh
- Peripheral sensory neuropathy
- Diminished reflexes in the limbs
Neuropathies - Motor>Sensory
- ADIP/CIDP
- Neuromuscular junction disorders
- Motor neurone disease
- Hereditary motor and Sensory neuropathy (CMT)
- Diabetes Mellitus
Rarer: Lead poisoning, acute intermittent porphyria, multifocal motor neuropathy
CIDP
Chronic Inflammatory demyelinating polyradiculoneuropathy
Neuropathies - Predominantly Sensory
- Diabetes Mellitus
- Paraneoplastic
- Paraproteinaemia
- Vitamin B6 intoxication
- Sjogren’s syndrome
Painful Peripheral Neuropathy
- Diabetes
- Alcohol
- B12 or B1 deficiency
- Drugs - chemotherapy, arsenic, thallium
Mononeuritis Multiplex Causes
Acute (Usually vascular)
1. Diabetes
2. Polyarteritis nodosa or Connective tissue disease
Chronic
1. Multiple compressive neuropathies (joint-deforming arthritis)
2. Sarcoidosis
3. Acromegaly
Fasiculations Causes
- Benign idiopathic
- Motor neurone disease
- Motor root compression
- Spinal Muscular Atrophy
Upper Trunk Palsy (C5/C6)
Loss of Shoulder movement and elbow flexion, hand in waiter’s tip position
Sensory loss over lateral arm and forearm, and over thumb
Lower Trunk Palsy
True claw hand with paralysis of all intrinsic muscles
Sensory loss along ulnar side of hand and forearm
Horner’s Syndrome
Ataxia + UMN signs
- Spinocerebellar ataxia
- Friedrich’s ataxia
- Demyelination such as MS
- Stroke or injury to brainstem and spinocerebellar tracts
Mixed Upper and Lower MN SIgns
Motor Neurone Disease
Syringomyelia
Multiple pathologies
Cervical myelopathy
Wasted Hand
- Peripheral Nerve lesions - Median, Ulnar, Brachial Plexus, CMT
- Anterior Horne Cell Disease (MND, Polio, SMA)
- Myopathy
- Spinal Cord Lesions
- Nutritional, disuse, ischaemia
Proximal Myopathy
- Drugs - Steroids, statins, fibrates
- Toxins - Alcohol
- Diabetic amyotrophy
- Thyroid disease
Myotonic Dystrophy
- Frontal Baldness
- Wasting of fascial musculature - temporalis/masseter, sternomastoid atrophy
- Partial ptosis
- Difficulty opening eyes after forceful closure
- Palatal, pharyngeal and tongue involvement - dysarthria, nasally speech
- Neck flexion weakness
- Impaired handshake (grip myotonia w/ impaired relaxation)
- Generalised weakness in voluntary muscles of arms and legs
- Forearm muscle wasting
- Thenar tap causes contraction + slow relaxation (percussion myotonia)
May have subtle peripheral sensory neuropathy/foot drop
Fascioscapulohumeral dystrophy
- Expressionless face
- Similar to myotonic dystrophy without ptosis or frontal balding
- Muscle weakness in face, shoulder, distal legs
- Muscle atrophy in face - difficulty with eye closure, smiling, pursing lips
- No ophthalmoplegia
- Shoulder weakness due to scapula winging - get patient to press on wall
- Biceps + triceps weakness but deltoid spared
- Spared wasting of the forearm
- Tibialis anterior muscle weakness is characteristic
Inclusion Body Myositis
Symmetrical proximal weakness. Asymmetrical distal weakness
- Classically - upper limb distal weakness, lower limb proximal weakness
Quadriceps particularly weak compared to other groups in legs
Forearm flexor weakness, handgrip weakness
Scalloping weakness in medial forearm
Distal DIP finger flexion weakness is often first sign
Can have weakness in eye closure. No oculomotor involvement
UMN Weakness: Face and arms > Legs
MCA Territory
UMN Weakness: Legs > Face and arms
ACA territory
Subcortical signs
Face/Arms/Legs equally effected
Pure motor stroke = lacunar lesion
Pure sensory = thalamic lesion
Mixed motor and sensory - Thalamus and Internal Capsule
Brown-Sequard
Ipsilateral UMN weakness. Ipsilateral vibration + proprioception deficit
Contralateral pain + temperature deficit
Ipsilateral LMN weakness and complete sensory loss at level of lesion
Syringomyelia
Begins asymmetrically
Atrophy in the neck, shoulder, arms, small muscles of hand
Sensory deficit in ‘cape’ distribution over shoulders, neck, trunk, distal hands
Clasically segmental LMN at level of lesion, UMN below lesion
Spinothalamic
Pain and Temperature
Corticospinal Tract
Muscle power
Spinocerebellar
Unconscious proprioception
Dorsal Column
Conscious vibration, proprioception
Anterior Spinal Artery Infarct
Only dorsal comuln is intact (vibration + propcrioception)
Motor, pain, temeprature deficity below lesion
Posterior Spinal Artery Infarct
Light touch, vibration, proprioception deficit below the lesion