A CASE OF PSEUDOHYPERTROPHIC MUSCULAR PARALYSIS. By R. K.
MADDOX,
M.B
,
CAPTAIN, I.M.S.,
Officiating
Civil Surgeon, Saran.
A few notes with the accompan}7ing photographs of this case may prove of interest. C., Rajput, aged eight, son of a cultivator, was
admitted to hospital on 3rd July 1899. His father, who brought him, aaid that about three jrears ago he noticed signs of weakness in the boy's lower limbs; the legs were unsteady, and the movements clumsy, and the boy frequentl}fell while walking. About two or three months later he noticed that the muscles of the calves and back of thighs were increasing in size.
This had
difficulty hospital.
gradually increased, with consequent getting about, until admission to
in
Oct.
PSEUDOHYPERTROPHIC
1899.]
Family History.?The patient is the eldest of three children, a sister aged six is alive and well, and one brother died a year ago of diarrhoea aged twelve months. The father is aged about thirty and is quite well, but complains of night blindness ; he had unfortunately gone away before
I had time to examine his eyes. The mother is about twenty-five. When the patient was three months old, his mother became insane, during which time she returned to her father, and the child, who did not go with her, was weaned. The husband cannot give details of the symptoms of his wife at this time, except that she became "paged."
aged
PARALYSIS.
365
with the abdomen projected forward owing to the exaggeration of the antero-posterior curve He balances himself with of the lumbar spine. his arms, and always endeavours to support himself by his hands. He is very easily pushed over when unsupported. He can stoop down, but cannot raise himself without using his hands oin some external object, as a chair or bed; he cannot raise himself by pushing himself up with the hands applied to the legs and thighs. The system generally is normal. Nervous system.?Sensory functions appear normal; muscular sense seems unaffected. Motor functions.?The organic reflexes are normal ; patellar reflex cannot be elicited, and there is no ankle-clonus. The skin reflexes are present. There is no alteration of the surface temperature in any part of the body. Voluntary Muscles.?There is enlargement and unnatural firmness of the following muscles, which is well shown in the photographs:? muscles of the calf on both sides, also of' backs of thighs and gluteal regions. The deltoid on each side is markedly affected, also the supraspinatus and infra-spinatus. To a less degree, the muscles of the forearm and the abductors of the little finger 011 each side. There is wasting of the entire pectoralis major 011 each side; this being specially marked in the sterno-costal portions. There is also some wasting of the biceps, and triceps muscles 011 each side. The enlarged muscles react to the interrupted current.
is difficult. He walks with his and the whole body is swa}Ted from side to side with each step, only the ball of the foot and toes touching the ground at each step. He is able, however, to walk alone by himself all over the hospital building. When sitting or lying down, he cannot raise himself to the erect posture without help; when lying, on a bed and told to raise himself, he first turns right over on to his face and then gradually raises his shoulders, and by a sudden kind of push twists the body round and o-ets himself into a sitting posture; but from this he cannot stand up unaided. When one hand is pulled up for him, he gradually raises himself up by pressing with the other hand on his thigh ; when sitting or lying, the thighs can only just slightly, and with great difficulty, be flexed on the abdomen. This appears to be greatly due to the increase in weight of the lower extremities. He has improved slightly in the power of his limbs since admission to hospital, but I am doubtful if this improvement is more than might be accounted for by good dieting. He has been treated by shampooing of the affected muscles and the application of the interrupted current, together with general tonic treatment and dieting.
Progression
legs separated,
Condition
on
admission.?A fairly healthy
The expression is the photograph by the mouth is kept open, and he has a vacant look, except when spoken to, when his face lights up and puts on His mental capaan appearance of intelligence. city is dull for his age, and he lias a frequently occurring meaningless laugh. He can, however, answer questions correctly and fairly
looking boy; height, 4ft. lin. stupid (this is exaggerated in the bright light at the time);
simple intelligently. When standing
he appears unsteady; the feet with the heels slightly the body resting on the of the feet. The body leans forward
placed widely apart raised, and the weight of are
forepart
MADDOX,
M.B
,
CAPTAIN, I.M.S.,
Officiating
Civil Surgeon, Saran.
A few notes with the accompan}7ing photographs of this case may prove of interest. C., Rajput, aged eight, son of a cultivator, was
admitted to hospital on 3rd July 1899. His father, who brought him, aaid that about three jrears ago he noticed signs of weakness in the boy's lower limbs; the legs were unsteady, and the movements clumsy, and the boy frequentl}fell while walking. About two or three months later he noticed that the muscles of the calves and back of thighs were increasing in size.
This had
difficulty hospital.
gradually increased, with consequent getting about, until admission to
in
Oct.
PSEUDOHYPERTROPHIC
1899.]
Family History.?The patient is the eldest of three children, a sister aged six is alive and well, and one brother died a year ago of diarrhoea aged twelve months. The father is aged about thirty and is quite well, but complains of night blindness ; he had unfortunately gone away before
I had time to examine his eyes. The mother is about twenty-five. When the patient was three months old, his mother became insane, during which time she returned to her father, and the child, who did not go with her, was weaned. The husband cannot give details of the symptoms of his wife at this time, except that she became "paged."
aged
PARALYSIS.
365
with the abdomen projected forward owing to the exaggeration of the antero-posterior curve He balances himself with of the lumbar spine. his arms, and always endeavours to support himself by his hands. He is very easily pushed over when unsupported. He can stoop down, but cannot raise himself without using his hands oin some external object, as a chair or bed; he cannot raise himself by pushing himself up with the hands applied to the legs and thighs. The system generally is normal. Nervous system.?Sensory functions appear normal; muscular sense seems unaffected. Motor functions.?The organic reflexes are normal ; patellar reflex cannot be elicited, and there is no ankle-clonus. The skin reflexes are present. There is no alteration of the surface temperature in any part of the body. Voluntary Muscles.?There is enlargement and unnatural firmness of the following muscles, which is well shown in the photographs:? muscles of the calf on both sides, also of' backs of thighs and gluteal regions. The deltoid on each side is markedly affected, also the supraspinatus and infra-spinatus. To a less degree, the muscles of the forearm and the abductors of the little finger 011 each side. There is wasting of the entire pectoralis major 011 each side; this being specially marked in the sterno-costal portions. There is also some wasting of the biceps, and triceps muscles 011 each side. The enlarged muscles react to the interrupted current.
is difficult. He walks with his and the whole body is swa}Ted from side to side with each step, only the ball of the foot and toes touching the ground at each step. He is able, however, to walk alone by himself all over the hospital building. When sitting or lying down, he cannot raise himself to the erect posture without help; when lying, on a bed and told to raise himself, he first turns right over on to his face and then gradually raises his shoulders, and by a sudden kind of push twists the body round and o-ets himself into a sitting posture; but from this he cannot stand up unaided. When one hand is pulled up for him, he gradually raises himself up by pressing with the other hand on his thigh ; when sitting or lying, the thighs can only just slightly, and with great difficulty, be flexed on the abdomen. This appears to be greatly due to the increase in weight of the lower extremities. He has improved slightly in the power of his limbs since admission to hospital, but I am doubtful if this improvement is more than might be accounted for by good dieting. He has been treated by shampooing of the affected muscles and the application of the interrupted current, together with general tonic treatment and dieting.
Progression
legs separated,
Condition
on
admission.?A fairly healthy
The expression is the photograph by the mouth is kept open, and he has a vacant look, except when spoken to, when his face lights up and puts on His mental capaan appearance of intelligence. city is dull for his age, and he lias a frequently occurring meaningless laugh. He can, however, answer questions correctly and fairly
looking boy; height, 4ft. lin. stupid (this is exaggerated in the bright light at the time);
simple intelligently. When standing
he appears unsteady; the feet with the heels slightly the body resting on the of the feet. The body leans forward
placed widely apart raised, and the weight of are
forepart
