CME INDIA Case Presentation by Dr Ashutosh Chandan Dubey (Dr Strange), DNB Med, Bokaro.

CME INDIA Case Study

CASE: A 50-yr. old male driver by profession, resident of Bokaro with primary education has come with complains of weakness of all 4 limbs which was insidious, gradual and progressive in nature. It all started on 10 Nov when patient noticed neck pain while driving followed by slipping of chappals from rt foot followed by injury to rt toe at multiple times. Weakness was gradual and progressively ascending upwards.

  • After 3-month weakness involved rt upper limb, followed by left lower limb n left upper limb. No associated loc, vomiting, convulsion, back injury, fever, radicular pain and it’s radiation to limb, no ataxia, facial weakness, slurring of speech, nasal intonation of voice, blurring of vision, tingling or numbness in extremities, loss of appetite, wt loss etc. Bowel is normal, pt can feel bladder sensation but he is complaining he can’t hold urine. 2 episodes of urine incontinence but I’m not sure of that.
  • No H/O HTN, DM, Thyroid disorder, old pulm Koch, or rheumatic disorder.
  • No significant personal history except occasional alcohol intake. No significant family or drug history or high-risk history. Vitals stable without any lymphadenopathy, no Neurocutaneous markers.
  • Neurological Examination
  • Normal mental status, normal cranial nerve examination, normal sensory examination (all post column, antero- lateral spino-thalamic tract and cortical sensation), normal cranium and spine.
  • Motor examination atrophy of right shoulder joint and atrophy of hypothenar n thenar eminence more on right compared to left side fasciculations were seen over rt deltoid.
  • Power 3/5 on both right side upper and lower limb, -4/5 in both left side upper and lower limb across shoulder, elbow, wrist, knee, hip, and ankle joint. All DTR exaggerated biceps, triceps, supinator, ankle, knee bilaterally. Even ankle clonus is present. Gait foot drop and spastic gait. Mild motor incoordination due to weakness but no ataxia and negative other cerebellar features. Superficial reflexes like Babinski is positive, exaggerated superficial abdominal and cremasteric reflex. Jaw jerk negative.
  • I’m thinking of High cervical compressive myelopathy but the MRI spine findings are not so significant but disc bulge is there at C4/C5 level and lower lumbar level.
MND or Cervical Cord Compression
  • Foot drop, But the cord compression is not so significant
  • I thought of compressive cervical myelopathy with spastic paraparesis. What’s your take on the case?? EMG is not available.

CME INDIA Discussion:

Dr S. S Lakshmanan, Chennai: MND

Dr Satish, Bokaro: Pure motor insidious onset spastic quadriparesis. They have also noted and recorded fasciculations over shoulder. It is most likely MND: ALS.

Dr Abhishek, DM, Neuro, Patna: As history starts, it looks like myelopathy. But no sensory and bladder bowel is against compressive myelopathy.

On examination, Fasciculations atrophy suggest LMN type.

DTR and spastic gait is UMN, So, LMN+ UMN without sensory bladder bowel with obvious fasciculations = MND, one thing I must say, very nicely presented … after a long time I saw such a detailed presentation.

Well, case clinically is of MND.

Dr Ujjwal Roy, DM, Neuro, Orchid Ranchi: EMG will give the answer. Kindly get it done.

Dr Sudhir Ranjan, DM Neuro, Ranchi: Appear to be ALS variant of MND.

Progressive coarse, combination of umn/lmn sign, insignificant cord compression, preserved sensory system (baring pain which is not against the diagnosis). Certain odds: Incontinence, slipping of chappal, how significant they are – Must do ENMG and paraprotein level.

Dr Ashok Kumar, DM Neuro, Ranchi: It is Motor Neurone Disease (MND). Pure motor weakness.

  • Brisk jerks.
  • Babinski positive – up going Plantar.
  • Fasciculations.
  • Jaw jerk is apparently significant in the video. It is not negative.
  • Superficial reflexes — Abdominal and Cremasteric — are lost in Compressive Myelopathies.
  • But these may be relatively preserved in MND.

Cremasteric reflex should be elicited with mildly Sharp end of the instrument, hammer — not by its side. These factors support a diagnosis of MND. I will suggest 

  • Examination of the tongue.
  • Electrophysiology, especially EMG.
  • Virology – HIV.

Please send the MRI Scan Cervical Spine — all films including Axial cuts. Occasionally we can see signal changes in Corticospinal tract. Though it is better seen in Brain, internal capsule.

CME INDIA Learning Points:

  • The diagnosis of MND depend upon critical clinical observation. Do observe characterization of weakness – asymmetrical versus symmetrical, proximal versus distal, upper versus lower limb predominance, presence, or absence of bulbar symptoms.
  • While a majority of ALS patients present with limb onset disease (60-80 percent), about one third to a fifth present with bulbar involvement in the form of dysarthria and dysphagia (along with emotional lability).
  • Patients may present with predominantly upper motor neuron (UMN) symptoms (hyperreflexia and spastic weakness) with the lower motor neuron (LMN) signs becoming commoner with the progression of the disease. Presentation with lower motor neuron signs of flaccid weakness, fasciculations, and muscle wasting may also be seen.
  • Physical signs in ALS:
    • Fasciculations, if generalized (can be easily missed over the shoulders)
    • Bilateral wasting of the tongues – lateral borders, particularly when there are also fasciculations with a brisk jaw jerk and orbicularis oculi reflexes
    • The “split hand”- preferential wasting of the lateral border of the hand, first dorsal interosseous and abductor policies Brevis, though to reflect the cortical organization.
  • The most common disease that mimics the amyotrophic lateral sclerosis is none other than degenerative spondylotic myeloradiculopathy in the cervical and/or lumbosacral spine, which may present with radicular pain. This may be characterized by progressive symptoms that plateau later with the progression of the disease. Although imaging has been advised to differentiate between the two entities, there may be the presence of co-existent degenerative illness in those with ALS.
  • Presentation with symmetrical findings, prominent extensor plantar responses (cervical myelopathy), presence of sensory deficit, presence of substantial weakness in the absence of wasting (multifocal motor neuropathy and myasthenia gravis) and disproportionate involvement of quadriceps (inclusion body myositis) should prompt further investigation for an alternative diagnosis.
  • An acute presentation of lower motor neuron syndrome should prompt consideration of immune, toxic, metabolic, and infective etiology. The major differentials include MND, multifocal motor neuropathy, and monomelic amyotrophy.
  • Riluzole, an NMDA (N-methyl-D-aspartate) receptor antagonist, reduces glutaminergic transmission by acting on the presynaptic voltage-gated sodium channels. Relative glutamate excess has been shown to lead to upper and lower motor neuron excitotoxicity contributing to neuronal cell death. It has been shown to be associated with a statistically significant improvement in tracheostomy free survival. The standard dose of Riluzole in the management of ALS is 50 mg two times daily.

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