PARAPERESIS CASES

This week we were given 3 cases to solve in an attempt to understand the topic of "paraparesis" to develop my competency in reading and comprehending clinical data including history, clinical findings, investigations and come up with a diagnosis and treatment plan.

This may develop my competency
a) reading and comprehending clinical data related to "paraparesis" including history, clinical findings, investigations

b) come up with a diagnosis such as:
1) Anatomical location of the root cause
2) Physiological functional disability
3) Biochemical abnormalities that could be a root cause at a molecular level
4) Pathology that could reflect the root cause at a cellular level

c) a treatment plan for each of these patients of paraparesis that can have a pharmacological and non pharmacological component.

d) learning the scientific basis of diagnostic and therapeutic approach in terms of past collective experiences and experiments (aka evidence based medicine).

PARAPERESIS

PARAPERESIS  is the partial paralysis of both the legs. It is characterised by progressive weakness and spasms in the legs.
True weakness occurs when muscles loose their strength.

Some common Causes of Paraperesis:


  • Arthritis of the spine
  • Herniated disc
  • Spinal cord injury, abscess or tumor
  • Spinal degeneration
  • Spinal stenosis
  • Infections of spinal cord
  • Gullian barre syndrome
  • Multiple sclerosis
  • Muscular dystrophy
  • Myasthenia Gravis
  • Polymyositis
  • Botulinum toxin
  • Organo phosphorus poisoning
  • Stoke 
  • Cerebral palsy
  • Transient ischaemic attacks
  • Vit B12 deficiency
  • Injury to joints, bones and muscles

CASE-1:

 https://srianugna.blogspot.com/2020/05/hello-everyone.htmll
https://youtu.be/3VVH7w3rWSM


An 18 yr old male patient came to the OPD with chief complaints of:

  • Bilateral lower limb weakness since 20 days 
  • HOPI: Weakness in bilateral lower limbs started 2 yrs ago. It started as a proximal muscle weakness and over the years has now progressed to a distal weakness.
  • H/O bilateral lower limb edema- non pitting type.
  • H/O difficulty in squatting position and diificulty in getting up from squatting position.
  • H/O difficulty in wearing and holding footwear

ON EXAMINATION

GENERAL EXAMINATION- NORMAL 

CNS EXAMINATION:
  • Pt is conscious, coherent, co-operative 
  • Higher mental functions- normal
  • cranial nerves- intact
  • sensory system- normal
  • No cerebellar and meningeal signs
  • Motor system: Tone- normal ,Power- LL- 4/5 in both lower limbs, reflexes absent in both lower limbs.
Neurological cause of weakness can be related to following anatomical structures:
Weakness can be due to both upper and lower motor neuron lesions.
Features of upper motor neuron lesion are:
  • Spaticity
  • Hypertonicity
  • Hperreflexia
  • Disuse atrophy (minimal)
  • Positive babinski's sign
  • Features of lower motor neuron lesion are:
  • Flaccidity
  • Hypotonicity
  • Hyporeflexia
  • Denervation atrophy(profound)
  • Negative babinski's sign
  • Fasciculations are present
In this patient there are no features suggestive of upper motor neuron lesion.

LOWER MOTOR NEURON: These are the alpha and gamma motor neurons in the anterior horn of spinal cord.
Axons of lower motor neurons travel peripherally to innervate muscles.Sites of lower motor neuron include:
  • ANTERIOR HORN CELL 
  • SPINAL NERVE ROOT(radical) AND PLEXUS
  • PERIPHERAL NERVE
  • NEUROMUSCULAR JUNCTION
  • MUSCLE
AHC LESION
  • No sensory/autonomic/cerebellar invovement.
  • Different patterns of weakness.
  • Asymmetric distal weakness without sensory loss:ALS(amyotrophic lateral sclerosis),PLS(primary lateral sclerosis),MMA(monomelic amyotrophy).
  • Symmetric weakness without sensory loss:PLS,PMA.
  • Lower motor neuron signs like weakness,wasting and fasciculations with/without UMN findings.
RADICULOPATHY AND PLEXOPATHY
  • Usually ASYMMETRICAL
  • Root pain is present along the distribution of nerve.
  • Sensory,motor systems involved and areflexia seen.
  • Muscles supplied by that particular nerve root are involved.
PERIPHERAL NEUROPATHY
  • Sensory and motor invovement seen.
  • Ususally distal to proximal evolution of the disease.
  • They can be AXONAL or DEMYELINATING neuropathies.
NEUROMUSCULAR JUNCTION LESION
  • Fatiguability.
  • Fluctuating weakness.
  • Ocular/pharyngeal muscle involvement.
MYOPATHY(anatomical location of root cause)
  • Pure motor weakness.
  • Predominant proximal muscle involvement.
  • No fasciculations.
  • Reflexes are burning pain,tight band like pressure and insensitivity to cold)

HISTORY RELATED TO PRESENT CASE: (physiological functional disability)                          
  • history of symmetrical weakness of lower limbs
  • history of proximal muscle weakness followed by distal muscle weakness 
  • no history of loss of sensation( no H/O numbness,tingling,pin and needles sensation,burning pain,tight band like pressure and insensitivity to cold)
  • areflexia is present
DIFFERENTIAL DIAGNOSIS:
  • Myopathy
  • Demyelinating peripheral neuropathy
ON INVESTIGATION:
  • CBP - normocytic normochromic with leucocytosis
  • RFT - increases creatinine and uric acid levels
  • CUE- albumin + and pus cells present
  • Muscle biopsy sugestive of polymyositis and Muscalar dystrophy
  • EMG and NCS-latency and decreased conduction velocit

  • As NCV is normal there is no invovement of peripheral nerves(CIPD ruled out)
  • Acquired myopathies are ruled out due to absence of antibodies and no H/O of exposure to any toxic substance
  • Metabolic Myopathies are ruled out due to absence of any clinical signs or lab investigations consistent with metabolic disorders 
  • Increased levels of creatine kinase (biochemical abnormality)and muscle biopsy point towards a possibility of a MUSCULAR DYSTROPHY.(pathology)
  • Inherited muscle disorders are called muscular dystrophies:
    Dystrophinopathies(Duchenne and Becker's) are likely in this case.
    • Duchenne's muscular dystrophy
    • Becker's muscular dystrophy
    • Limb girdle muscle dystrophy
    • Emery Dreifuss muscle dystrophy
    • Fascioscapulohumeral muscle dystrophy
    • Myotonic dystrophy
    • Mitochondrial myopathies
    • Both have X-linked recessive inheritance due to a mutation in dystrophin gene.
    • Duchenne muscular dystrophy is a more severe form usually presenting before the age of 5. Proximal muscles of lower limbs are predominantly involved with positive gower's sign There is pseudohyperthrophy of calf muscles.They do not live longer because of serious cardiac conduction abnormalities and dilated cardiomyopathy.Therefore,Duchenne is unlikely in this patient.
    Becker's usually survive into their 40's and have highly variable disease onset.

    FURTHER INVESTIGATIONS REQUIRED:
    • Electromyography
    • Genetic testing

    TREATMENT: Presently there is no cure for muscular dystrophy.
    • corticosteroids- increase muscle strength and slow progression
    • Heart medications if associated with any heart conditions
    • Physiotherapy.

    CASE-2:


    An 18 yr old male presented with complain
    • Difficulty in walking since 1 month
    • Bilateral lower limb weakness since 1 month
    • Pain in the lower limb calf muscles since 1 month.
    • h/o difficulty in standing from sitting position.
    • h/o difficulty in climbing stairs
    • h/o difficulty in holding footwear
    • h/o slipping of footwear without knowledge
    • h/o muscle wasting (LL>UL)

    Patient is a known alcoholic

    GENERAL EXAMINATION:

    pallor present. patient is conscious and coherent.

    CNS EXAMINATION:

    • Pt is conscious and cranial nerves intact
    • Sensory system - normal
    • Cerebellar and meningeal signs - absent
    • Motor system:
                  bulk- decreased in UL and LL
                  Tone: normal in UL. Hypotonia in lower limbs
                  Plantar reflex- absent
                  Deep tendon reflexes- Absent







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