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Myopathy (Becker variety)

 Personal history
‫ جمال زكي إب راهيم‬male patient, 48 years old, from Cairo, married with 4 off spring, the
youngest is 15 years old, Teacher, mild smoker with no other special habits of medical
importance, Rt. handed.

 c/o
inability to stand up from sitting position, 20 years duration.

 HPI
The condition started 25 years ago by gradual onset and slowly progressive course of
weakness associated with flaccidity and proximal wasting, with no muscle twitches.
This weakness affected both lower limbs and progressed 2 years later to involve the upper
limbs. It affects proximal more than distal muscles, extensor more than flexor muscles,
adductor more than abductor muscles. No diurnal variation, no descending march
course, no fever, no trauma, no history suggesting cushing, acromegaly, hypo or
hyperthyroidism with no history of drug intake.

5 years later, the weakness became more severe and the patient was unable to
stand up from sitting position at all.

No symptoms of sensory affection.

No symptoms of sphincteric affection.
No symptoms of cranial nerves affection.
No symptoms of increased I.C.T.
No symptoms of speech disorders.
No symptoms suggesting pulmonary or systemic congestion or any other symptoms
suggesting CVS affection.
No symptoms suggesting other system affection.

 Past history
- No past history of drugs known to cause myopathy (e.g. Corticosteroids, Chloroquine,…).
- No past history of D.M, HPN, or operations.

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

- No common disease in family.

 General exam
- Temperature: 37.2o c.
- Bl. Pressure: 130/70.
- Pulse: regular, 70 beat/minute, average volume, no special character, vessel wall
not felt, equal in both sides with intact peripheral pulsation.
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- 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 Cr. Nerves: Normal (special care to exam of ocular group and facial nerve ).
 Examination of Motor System :
 Inspection__
- Bilateral symmetrical Proximal wasting in upper & lower limbs with
pseudohypertrophy in the calf muscles.
- No skeletal deformities, no trophic changes, no involuntary movement.

 Examination of Tone__
- Bilateral symmetrical Hypotonia in both upper and lower limbs. It's proximal more
than distal.

 Percussion__
No fasciculation or mechanical myotonia.

 Examination of Muscle Power

- Bilateral symmetrical Weakness in both upper and lower limbs. It is proximal more
than distal, adductors more than abductors, extensors more than flexors.
- Deltoid, Serratus anterior, Latismus Dorsi, Lower head of pectoralis major, lower
fibers of trapezius are affected while Sternomastoid, Upper fibers of trapezius, Upper
head of pectoralis major are spared.
- Power of abdominal muscles  Beevor's sign.

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

 Reflexes
- Bilateral areflexia in upper and lower limbs
- Babinski : plantar flexion on both sides (normal).
- Abdominal reflex : diminished below the level of the umbilicus .

 Sensory:
- Superficial sensations: intact.
- Deep sensation: intact.
- Cortical sensation : intact.

 Back: Exaggerated lumbar lordosis.

 Gait: waddling gait.
 No affection in other system examination.

 Investigations
- EMG, Muscle Biopsy, enzymes .
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 Diagnosis :

Muscle disease, Progressive muscular dystrophy, Pseudohypertrophic pelvic girdle

type, Becker variety.

 Wasting
 Hypotonia So the lesion is
 Areflexia LMNL
 Weakness

 Not A.H.C: No Fasciculation.

So the case is
 Not Peripheral Nerve: No sensory or
Muscle disease
sphincteric disturbance.

 Not myoneural junction: No diurnal variation,

no descending march course.
 Not Myotonia So the case is
 No Mechanical Myotonia. myopathy
 No Voluntary Myotonia.
 Sternomastoid Muscle is spared.

 Not Ocular: ocular muscles are intact.

So the case is either
 Not distal type of Gower: distal muscle are less
pelvic or shoulder girdle
affected that proximal.

 Pelvic Girdle
 LL > UL.
So the case is either
 Beevor's Sign.
atrophic or pseudohypertrophic
 Diminished abdominal reflex below the level
of umbilicus.

 Pseudo-hypertrophy: calf muscles are

So the case is either
hypertrophied but weak.
Duchenne or Becker
To make sure ask for EMG and muscle biopsy.

 Becker
 3rd Decade.
 Slowly progressive.
 No skeletal deformity.
 No C.V.S affection.

:: ‫◄ حالة "قرنى" تختلف عن حالة "جمال" في اآلتي‬

1. Wasting, weakness & hypotonia are more in upper limb.
2. Facial muscles are affected.
So the case is shoulder girdle type, facio-scapulo-humeral variety (landouzy and dejerren's)