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MND (Motor Neuron Disease)

 Personal history
As Usual.
 c/o
Heaviness of both upper and lower of 25 years duration.

 HPI
The condition started 25 years ago by gradual onset of weakness associated with
flaccidity, marked distal asymmetrical wasting in both UL and LL and twitches. Both
weakness and wasting started in both ULs but they are noticeably more on the Rt. Side.
Then, they progressed ,one year later, to affect both LLs being more on Lt. side. Weakness
was distal more than proximal, in abductor more than adductor muscles, in extensor
more than flexor muscles in U.L, while in L.L, it is more in flexors than extensor muscles
with no involuntary movements.

No symptoms of sensory affection.

No symptoms of Bulbar or Cranial Nerve affection.
No symptoms of sphincteric affection.
No symptoms of increased I.C.T.
No symptoms of speech disorders.
No symptoms suggesting other system affection.

 Past history
- No past history of DM, HPN, fever, Bilharziasis , drugs or operations.

 Family history
- No similar condition in family.
- No consanguinity.

- No common disease in family.

 General exam
- Temperature: 37.2o c.
- Bl. Pressure: 120/70.
- Pulse: regular, 70 beat/minute, average volume, no special character, vessel wall
not felt, equal in both sides with intact peripheral pulsation.
- Mentality: The patient is fully conscious, well oriented for time, place and person.
Average mood and memory. The patient is co-operative with average intelligence.

 Examination of Speech: Normal.

 Examination of Cranial Nerves:
- Bulbar Cr. Nerves  free.
- Other Cranial Nerves  free.
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 Examination of Motor System :

 Inspection__
- Bilateral asymmetrical distal wasting in ULs and LLs. It's more in right UL and left LL.
- Bilateral Pes cavus, no trophic changes, no involuntary movement, no specific posture.

 Examination of Tone__
- Bilateral asymmetrical Hypotonia in upper limbs and lower limbs. It's more in right UL
and left LL.

 Percussion__
Few fasciculation scattered in right UL and left LL with No myotonia.

 Examination of Muscle Power

- Bilateral asymmetrical Weakness in upper limbs and lower limbs. It's more in right UL
and left LL. It is distal more than proximal, in abductor more than adductor muscles, in
extensor more than flexor muscles in UL while in LL, It's more in flexors than extensor
muscles.

 Coordination
Coordination cannot be examined on both upper and lower limbs because of weakness.

 Reflexes
- Deep reflexes: Bilateral asymmetrical hyper-reflexia in upper limbs and lower limbs.
It's more in right UL and left LL with +Ve pathological reflexes.
- Superficial reflexes: +Ve Babiniski on both sides. Abdominal Reflex  Lost.

 Sensory:
- All sensations (Superficial, deep, cortical) are intact.

 Back: Normal (No deformities, no pigmentation, no scars, no swellings).

 Gait:
 No affection in other system examination.

 Diagnosis :

A case of motor neuron disease, most probably Amyotrophic Lateral Sclerosis .

 N.B:
1) Why MND?
- Purely motor with no sensory or sphincteric manifestations
- Unknown etiology.
- It affects motor neuron only inside CNS.
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2) Why spinal type?

Bulbar and spino-bulbar types are excluded because bulbar cranial nerves are free.

3) Why ALS?
Because the patient has wasting and fasciculation which are features of LMNL and at the
same time, It has weakness of pyramidal tract distribution with +ve planter reflex which are
features of UMNL.
On the light of these confusing data, the case is either:
1- ALS.
2- Cervical spondylosis.
3- Cervical syringomyelia.
But because the patient's sensations are intact, It's most probably ALS.

4) Criticism to the diagnosis :

- Asymmetrical  may be attributed to the patient's life style as he is manual worker
using his right UL and left LL and MND is a disease of exhausted muscles.
- Long survival while MND is of short duration  prognostic criteria are:
Bulbar affection.
Fasciculation.
Our patient has few fasciculation with no bulbar affection which account for
good prognosis and prolonged survival.
- Early onset of the disease while MND is a disease of old age  ALS can affect
young age.

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