Myopathy (Becker variety)

Myopathy (Becker variety)

4 Personal history

جمال زكي إبراهيم male 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.

4 c/o

inability to stand up from sitting position, 20 years duration.

4 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.

4 Past history

-    No past history of drugs known to cause myopathy (e.g. Corticosteroids, Chloroquine,…).

-    No past history of D.M, HPN, or operations.

4 Family history

-          No similar condition in family.

-          No consanguinity.

-          No common disease in family.

4 General exam

-          Temperature: 37.2^o 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.

-          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.

4 Examination of Speech: Normal.

4 Examination of Cr. Nerves: Normal (special care to exam of ocular group and facial nerve ).

4 Examination of Motor System :

4 Inspection__

-          Bilateral symmetrical Proximal wasting in upper & lower limbs with pseudohypertrophy in the calf muscles.

-          No skeletal deformities, no trophic changes, no involuntary movement.

4 Examination of Tone__

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

4 Percussion__

No fasciculation or mechanical myotonia.

4 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 à Beevor's sign.

4 Coordination

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

4 Reflexes

-          Bilateral areflexia in upper and lower limbs

-          Babinski : plantar flexion on both sides (normal).

-          Abdominal reflex : diminished below the level of the umbilicus .

4 Sensory:

-          Superficial sensations: intact.

-          Deep sensation: intact.

-          Cortical sensation : intact.

4 Back: Exaggerated lumbar lordosis.

4 Gait: waddling gait.

4 No affection in other system examination.

4 Investigations

-          EMG, Muscle Biopsy, enzymes .

4 Diagnosis :

Muscle disease, Progressive muscular dystrophy, Pseudohypertrophic pelvic girdle type, Becker variety.

So the lesion is LMNL	v      Wasting v      Hypotonia v      Areflexia v      Weakness
So the case is Muscle disease	4 Not A.H.C: No Fasciculation. 4 Not Peripheral Nerve: No sensory or         sphincteric disturbance.
So the case is myopathy	4 Not myoneural junction: No diurnal variation, no descending march course. 4 Not Myotonia v      No Mechanical Myotonia. v      No Voluntary Myotonia. v      Sternomastoid Muscle is spared.
So the case is either pelvic or shoulder girdle	4 Not Ocular: ocular muscles are intact. 4 Not distal type of Gower: distal muscle are less affected that proximal.
So the case is either atrophic or pseudohypertrophic	4 Pelvic Girdle v      LL > UL. v      Beevor's Sign. v      Diminished abdominal reflex below the level of umbilicus.
So the case is either Duchenne or Becker	4 Pseudo-hypertrophy: calf muscles are hypertrophied but weak. To make sure ask for EMG and muscle biopsy.
	4 Becker v      3rd Decade. v      Slowly progressive. v      No skeletal deformity. v      No C.V.S affection.

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

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)