Neurology SC Flashcards
CMT
Disorder:
Autosomal dominant, peripheral nerve demyelination (CMT2 = more axonal) resulting in symmetrical distal sensorimotor neuropathy
Sensation: reduced, glove/stocking distribution (dorsal>spinoT)
Reflexes: reduced/absent reflexes.
Weakness: atrophy and weakness of the small muscles of the hand, LL distal muscle weakness & atrophy (inverted champagne bottle legs).
Pes cavus.
High stepping gait (foot drop, proprioceptive deficits)
Management:
Ankle-foot orthoses.
What are the motor functions of the median nerve?
Thumb opposition
Digits 1, 2, 3 flexion
Wrist flexion and abduction
Forearm pronation
What are the motor functions of the ulnar nerve?
Thumb + finger abduction
finger adduction
Digits 4-5 flexion
Wrist flexion and adduction
What are the branches of the sciatic nerve? What are there motor and sensory function?
Common peroneal:
Superficial peroneal - foot eversion; lateral foot + calf
Deep peronal - foot DF, toe extension; patch between toes 1 and 2
Tibial nerve: foot PF, inversion, toe flexion; lateral aspect of foot
Foot drop with loss of ALL foot movements, absent ankle jerk + weak knee flexion
Where is the lesion?
Sciatic neuropathy.
NOTE - peroneal nerve often more affected than tibial; may have sparing of PF despite weak AJ
Lower trunk plexopathy
- signs
- spinal levels
C8, T1
- Claw hand
- Horners
- loss of movement of intrinsic muscles of the hand (T1)+ wrist and finger flexion (C8).
- senosry loss of ulnar side of hand AND forearm
Upper trunk plexopathy
- signs
- spinal levels
“Erbs palsy”
C5,C6
- loss of shoulder movements and elbow flexion = “waiters tip” - limp handing arm + internal rotation of the forearm + wrist and finger flexion.
- sensory loss over lateral aspect of arm AND forearm
Causes of upbeat nystagmus
Pontine lesions - MS, stroke, tumor
Wernickes
Bilateral INO
Causes of downbeat nystagmus
Cervicomedullary junction lesions (arnold chiari malformations)
Cerebellar degeneration
Drug intoxication (PHY, CBZ)
Demyelination
Causes of proximal myopathy
Toxins/drugs - ETOH, steroids
Metabolic - Thyrotoxicosis, diabetic amyotrophy
Muscular - PM/DM, Mitochondrial myopathies, Muscular dystrophy (i.e. scapulohumeral dystrophy)
causes of distal myopathy
IBM (with quadriceps wasting + hand grip weakness)
Myotonic dystrophy
Features of arnold chiari malformation
Lower cranial neuropathies (9/10/11/12), myelopathy (compression of medulla + spinal chord)
Ataxia, dysmetria, downbeat nystagmus (cerebellar compression)
Headache, syringomyelia - central chord syndrome (CSF blockage)
4 differences between critical illness myopathy (CIM) and and polyneuroapathy (CIP)
Reflexes - preserved or mildy reduced in CIM, absent in CIP
Sensation - normal in CIM, reduced in CIP
Facial muscles - weak in CIM, cranial nerves perserved in CIP
NCS CIP: generalized axonal sensorimotor polyneuropathy with low motor AND sensory amplitudes in CIP
NCS CIM: low motor amplitudes and prolonged CMAP in CIM
Features of IBM
Older age (>50 typically)
Typically proximal + distal weakness
Weakness of wrist and finger flexors > extensors
Weakness of quadriceps and knee extensors > flexors
Hyporeflexia
Facial muscle weakness with oculomotor sparing
Muscle atrophy
Dysphagia
Ix - raised CK, muscle biopsy
Features of Adies syndrome
Where is the lesion?
Mydriatic pupil with impaired light reaction and intact (but slowed) accomodation
Often associated with hyporeflexia
Commonly affects females
Usually idiopathic but may be due to inflammation (inc. migraine) or infection
Lesion - efferent parasympathetic pathway
Features of Argyll Robertson pupil
4 causes
Miotic irregular pupils unreactive to light with intact accomodation
- Diabetes mellitus
- Neurosyphillis
- Alcoholic midbrain degeneration
- Midbrain lesions
9 causes of peripheral neuropathy
Drugs/toxins - amiodarone, chemo (vincristine, cisplatinum), anti-infective (isoniazid, nitrofurantoin), heavy metals, phenytoin, alcohol
Infiltrative - amyloidosis, tumor/paraneoplastic
Metabolic - diabetes, hypothyroidism, porphyria, uremia, B12 or B1 deficiency
Immune mediated - GBS (AMSAN, AIDP)
Connective Tissue disorder/vasculitis - SLE, polyarteritis nodosa, sjogrens
Hereditary (i.e. CMT)
Idiopathic
8 causes of predominantly sensory neuropathy
Infective - Syphyllis
Autoimmune - Sjogrens
Metabolic - B6 intoxication, B12 deficiency, Diabetes
Neoplastic - paraneoplastic (lung, ovary, breast), paraproteinemia
Idiopathic
6 causes of painful small fibre neuropathy
Diabetes Alcohol B12 or B1 deficiency Carcinoma Porphyria Arsenic/thallium poisoning
Definition of synkinesis and what disorder is it seen in
Increased rigidity of one arm with voluntary movement of the other
Seen in parkinsons disease
8 causes of parkinonism
Idiopathic parkinsons disease
Parkinsons plus syndromes (PSP, MSA, CBD, LBD)
Toxic - Wilson’s disease, Drugs (antipsychotics, valproate, metoclopramide, methyldopa)
Ischemic - Hypoxic brain injury, Vascular parkinsons
Tumor - Basal ganglia tumors
8 parkinsons plus symptoms
Poor response to dopaminergic therapies Early falls Early hallucinations Early autonomic dysfunction Cognitive impairment within <1 year of symptom onset Symmetry Ocular signs Cerebellar signs
Features differentiating essential tremor from parkinsons tremor
Symmetric
Worse with voluntary movement
Associated head tremor
8 causes of bilateral pes cavus
SCA CMT Hereditary spastic paraperesis Spinomuscular atrophy Muscular dystrophies Cerebral palsy Spinal cord - Syringomyelia, tumors (can also cause unilateral)