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 Achilles 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
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
Kennedys 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)
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
Refsums disease accumulation of phytanic acid
Fabrys 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

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
T10 and T11 vertebrae = lumbar segments
T12 and L1 = sacral and coccygeal
Conus medullaris = T9 to L1 vertebrae
Conus Medullaris
Cauda equina
Presentation
Acute
Chronic
Reflexes
Knees preserved; ankle absent Both knees and ankles absent
Motor
Spastic para; symmetrical
Flaccid para; asymmetrical
Sensory
More LBP, less radicular
Less LBP, more radicular
Sensory
Perianal
Saddle
Impotence
Frequent
Less frequently
Sphincter
Occurs early
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