Flaccid Paraparesis
Examination
 Complete the LL examination
 Commonly
 HMSN
 Polio
 (infantile hemiplegia)
 Spina Bifida
 Cauda Equina Syndrome
 GBS/CIDP
 MND (see spastic paraparesis)
 Diabetic amyotrophy (See proximal myopathy)
 Concentrate on
 Ataxia – Miller Fisher Variant, Tick Paralysis
 Sensory
 No sensory abnormalities
 Myopathies
 Neuromuscular
 Nerves – certain conditions eg GBS, multifocal motor neuropathy
 Anterior Horn Cell
 Glove and stocking
 Peripheral neuropathy
 HMSN, paraneoplastic
 Mild and patchy = GBS
 Sensory level (Acute)
 Cord compression
 Cord infarction
 Transverse myelitis
 L5 and S1 sensory loss in spina bifida
 Typical features of HMSN
 Pes cavus, clawing of toes, contractures of Achille’s tendon, inverted
champagne bottles (wasting of distally and stops abruptly at the lower one
third of thighs; also similar distal wasting distally in the ULs)
 LMN – reduced tones and no clonus, reduced reflexes and downgoing
plantar response, weakness, bilateral footdrop
 Sensory – no sensory or mild glove and stocking
 Gait – high steppage gait of foot drop
 Marked deformity with minimal disability
 Others
 Feel for thickened nerves (lateral popliteal nerve)
 Examine the hands for small muscle wasting and clawing
 Examine spine for scoliosis
 Feel for thickened Greater Auricular nerves
 Wheelchair, calipers
 Examine
 Back
 Kyphoscoliosis
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 Spina bifida – scars, tuft of hair, dimples, sinus or naevus
Per rectal examination
 Saddle anaesthesia and cauda equina syndrome
 Incontinence – fecal and urinary
Upper limbs
CNs- fatiguibility, GBS (bilateral VII)
Functional aids
Presentation
 Obvious disease
 HMSN
 Sir, this patient has got HMSN/CMT as evidenced by
 Bilateral pes cavus with clawing of toes and distal wasting of the lower
limbs with a inverted champagne bottle appearance; there is hypotonia
with reduced reflexes and downgoing plantar responses a/w weakness of
the lower limbs of power 4/5 with bilateral foot drop; there is no
associated sensory disturbance; she has a high steppage gait form bilateral
foot drop and is able to walk independently inspite of the marked feet
deformity; I also noticed presence of wasting and clawing of the upper
limbs; there is no palpable thickened lateral popliteal nerve.
 I would like to complete my examination by examining the spine back for
scoliosis and palpate for other sites of thickened nerves
 Mention walking aids or wheelchair
 Polio
 Sir this patient has monoparesis of the right LL most likely due to polio
 A shortened right lower limb associated with wasting. It is hypotonic with
reduced reflexes and downgoing plantar response and is flaccid with a
power of 3/5. There is no sensory weakness.
 There is no UMNs or shortened wasted right UL to suggest infantile
hemiplegia
 Examination of the back did not reveal any cutaneous signs of spina bifida.
 Mention any walking aids/wheelchair
 Not so obvious
 Sir, this patient has got flaccid paraparesis as evidenced by
 Presence of hypotonia with reduced reflexes a/w with downgoing plantar
responses bilaterally; I did not detect any fasciculations. There is weakness of
the LLs with a power of 3/5. There is no associated cerebellar signs in the LLs
and no sensory loss to pin prick, propioception and vibration.
 Complete my examination
 Back
 Per rectal
 ULs for ataxia, flaccid paresis
 CNs for cranial neuropathies
Questions
What are the causes of flaccid paraparesis?
 Acute myopathies
 Inflammatory myopathy (polymyositis, dermatomyositis)
 Rhabdomyolysis (extreme exertion, drugs, viral myositis, crush injury etc.)
 Acute alcoholic necrotizing myopathy
 Periodic paralyses (hypokalemic, hyperkalemic)
 Metabolic derangements (hypophosphatemia, hypokalemia,
hypermagnesemia)
 Thyroid or steroid myopathy
 Neuromuscular
 Myasthenia gravis
 Botulism
 Tick paralysis
 Other biotoxins (tetradotoxin, ciguatoxin)
 Organophosphate toxicity (can also cause neuropathy)
 Lambert-Eaton Myasthenic Syndrome (LEMS)
 Nerve
 Diphtheria
 Porphyria
 Drugs & Toxins (arsenic, thallium, lead, gold, chemotherapy – cisplatin /
vincristine)
 Vasculitis (incl. Lupus, polyarteritis)
 Paraneoplastic and Paraproteinemias
 Multifocal motor neuropathy
 Nerve roots
 Guillian Barre Syndrome
 Lyme disease
 Sarcoidosis
 HIV
 other viruses (CMV, VZV, West Nile)
 Cauda equina syndrome (lumbar disc, tumour, etc.)
 Plexus lesions (brachial plexitis, lumbosacral plexopathy)
 Anterior Horn Cell (motor neuron diseases):
 Amyotrophic lateral sclerosis (ALS) – with UMN findings
 Poliomyelitis
 Kennedy’s disease (spinobulbar atrophy / androgen receptor gene)
 other spinomuscular atrophies (inherited)
 Anterior spinal artery syndrome (with grey matter infarction)
 Spinal Cord (corticospinal tract diseases):
 Inflammatory (Transverse myelitis)
 Subacute combined degeneration (B12 deficiency)
 Spinal cord infarction
 other myelopathies (spondylosis, epidural abscess or hematoma
 Brain
 Pontine lesions (eg. Central pontine myelinolysis, basis pontis infarct or bleed)
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Multifocal lesions (multiple metastases, dissemination encephalomyelitis
[ADEM], multiple infarcts or hemorrhages – eg. DIC, TTP, bacterial
endocarditis)
What is Charcot Marie Tooth disease?
 Hereditary sensory motor neuropathy
 Consisting of 7 types of which types 1,2 and 3 are the most common types
 Type 1 – A demyelinating neuropathy, aut dominant, absent tendon reflexes,
enlarged nerves; Chr 17
 Type 2 – An axonal neuropathy, aut dominant (mild and present later), normal
deep tendon reflexes, nerves not enlarged; Chr 1
 Type 3 – rare, hypertrophic neuropathy of infancy, thickened nerves, aut
recessive (Dejerine Sottas disease)
 Physical findings
 Above plus
 Others – optic atrophy, retinitis pigmentosa and spastic paraparesis
 Ix
 Rule out other causes of neuropathies
 EMG/NCT
 Biopsy
 Genetic testing
 Mx
 Eductaion and counselling and family screening
 PT/OT, AFOS
 Medical Rx – pain relief, avoid obesity
 Surgical treatment
 Px
 Normal life expectancy
 Disease usually arrest in middle life
 Disability varies
 Dy/Dx of hereditary disease
 Hereditary amyloidosis
 Refsum’s disease – accumulation of phytanic acid
 Fabry’s disease – deficiency of alpha galactosidase
What is poliomyelitis?
 Enterovirus, picorna virus, with IP of 5-35 days, oro-fecal route or contaminated
water, 3 serotypes
 Replicate in the nasopharynx and GIT and then to lymphoid tissue and then
hematological spread with predilection to the anterior horn cells of the spinal cord
or brainstem with flaccid paralysis in spinal or bulbar distribution
 4 forms
 Inapparent infection
 Abortive – nauseas, vomiting and abdominal pain
 Nonparalytic – above plus meningeal irritation
 Paralytic – paralysis and wasting; bulbar or spinal distribution
 Occasionally, can get postpolimyelitis syndrome which results in weakness or
fatigue in the initially involved muscle groups 20-40 years later
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Ix
 Viral c/s from stool, throat and CSF
 Antibodies
Mx
 Educationa and counselling
 Non-medical
 PT/OT
 Care of limbs
Medical
 Rx complications
 Pain
 Respiratory failure
 Clear bowels
Prevention
 Inactivated polio vaccine – Salk vaccine which is administered parenterally
 Oral live vaccine – can result in poliomyelitis in immunodeficient individuals
Dy/Dx
 Spina bifida
 Infantile hemiplegia – hypoplasia of the entire side of the left side with UMN
sign on the affected side
What is Spina Bifida?
 Incomplete closure of the bony vertebral canal with similar anomaly of the spinal
cord
 Usually in lumbosacral region, can also involve the cervical region and is
associated with hydrocephalus
 Look for
 Scars, tuft of hair, dimples, sinus, naevus, lipoma
 Asymmetric LMN signs of LLs
 L5 and S1 dermatomal sensory loss
 Bladder involvement
 X-ray: sacral dysgenesis, laminar fusion of the vertebral body. Scoliosis
 Multifactorial aetiologies, with folic deficiency and use of Na Valproate, siblings
with spina bifida has higher risk
 Prevented with use of folic acid early in pregnancy
 Can be tested with amniotic serum AFP, serum AFP or USS
What is cauda equina syndrome?
 The cauda equina refers to the nerve roots that are caudal to the termination of the
spinal cord; any lesion below the 10th Thoracic vertebrae
 Low back pain, unilateral or bilateral sciatica, saddle anaesthesia, bladder and
bowel disturbances and variable motor and sensory LL abnormalities
 Causes – trauma, PID, spondylosis, abscess, tumor (ependymoma and NF)
 Anatomy
 Spinal cord starts from the foramen magnum to the level of L1 vertebrae
 Add 1 to Cx vertebrae
 Add 2 to Tx vertebrae 1-6
 Add 3 for Tx vertebrae 7-9
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T10 and T11 vertebrae = lumbar segments
T12 and L1 = sacral and coccygeal
Conus medullaris = T9 to L1 vertebrae
Conus Medullaris
Presentation
Acute
Reflexes
Knees preserved; ankle absent
Motor
Spastic para; symmetrical
Sensory
More LBP, less radicular
Sensory
Perianal
Impotence
Frequent
Sphincter
Occurs early
Cauda equina
Chronic
Both knees and ankles absent
Flaccid para; asymmetrical
Less LBP, more radicular
Saddle
Less frequently
Occurs late
What is Guillain Barre Syndrome?
 Auto immune, antecedent Campylobacter infection
 Bimodal – young adults or the elderly
 Motor, sensory and autonomic dysfunction
 Progressive ascending muscle weakness, variable patchy sensory loss,
hyporeflexia and autonomic disturbances such as tachycardia and labile BP
 Post GI or resp infection, 2-4 weeks of onset of symptoms which may progress
over hrs to days and recovery over months; complicated by respiratory failure
 Subtypes
 Acute inflammatory demyelinating neuropathy
 Acute motor axonal neuropathy
 Acute motor-sensory axonal neuropathy
 Miller Fisher Syndrome (Ataxia, areflexia and ophthalmoplegia; antiGQ1b
Ab)
 Acute panautonomic neuropathy
 Ix
 CSF shows albuminocytologic dissociation (<10 mononc cells and high prot)
 AntiGQ1B Ab in MFS and anti GM1 implies poorer Px
 NCT – demyelination
 FVC (15-20ml/kg < or NIF 25cmH2O<) = may require ventilation
 Mx
 Emergency
 ABCs, and pacing maybe required
 IVIG
 Plasma exchange
 Steroids
 PT/OT
 Prevention Cx – DVT prophylaxis
 Px
 Most 85% will have full recovery by 6-12 months
 Complications from respiratory failure and cardiac dysrythmias and labile BP