smitha
Forum Elite
Topics: 53
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CASE:· a 35yo black male is seen in clinic with a 3 month history of weakness and muscle cramps, first felt in the left arm, but then progressing to both legs. His voice is not as loud as it used to be, and the tone has changed. Sometimes, he complains having trouble eating and swallowing on one side of his mouth, and he noticed he can do so if he moves it with his finger. Has has no sensory loss. Reflexes are brisk, including a jaw jerk. His toes are equivocal. Fasciculations are present in the tongue at rest, and all four proximal extremities. One year later, he has difficulty swallowing, is short of breath, and appears emaciated. Can you localize the lesion?
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| smitha
Forum Elite
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and added to my above q of localising the lesion, i wud also add" wat's ur diagnosis?"................. 
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| shirish_sss
Forum Senior
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GREAT QUESTION SMITHA.
YOUR QUESTIONS REALLY SIMULATE THOSE ON BOARDS.
WELL THIS IS A CASE OF MOTOR NEURON DISEASE OR AMYOTROPHIC LATERAL SCLEROSIS OR LOU GEHRIGS DISEASE.
THIS IS BECAUSE THE PATIENT IS AN ADULT WITH SIGNS OF BOTH UPPER MOTOR AND LOWER MOTOR NEURONAL DEGENARATION WITHOUR ANY SENSORY LOSS.
IF IT WOULD HAVE BEEN A CHILD THEN MY DIAGNOSIS WOULD BE WERDNIG-HOFFMAN DISEASE- A CHILDHOOD VERSION OF ALS.
SINCE YOUR CASE HAS SYMPTOMS OF BOTH LOWER MOTOR NEURON LOSS( FASCICULATIONS) AND UPPER MOTOR NEURON LOSS (WEAKNESS AND DYSARTHRIA) THIS CLASSIFIES AS ALS.
IF IT WAS AN ENTIRELY UMN CASE WITHOUT ANY SIGNS OF LOSS OF LMN THEN I WOULD SUGGEST FAMILIAL SPASTIC PARAPLEGIA AS MY DIAGNOSIS.
ALS CAN BE INHERITED IN VARIOUS MANNERS.
AUTOSOMAL DOMINANT- DEFICIENCY OF SUPEROXIDE DISMUTASE (SOD)
AUTOSOMAL RECESSIVE- UNKNOWN MECHANISM
MITHOCHONDRIAL - DEFECT IN CYTOCHROME C OXIDASE GENE.
THANKS SMITHA FOR THIS GOOD QUESTION.
___________________
good
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| smitha
Forum Elite
Topics: 53
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That was gr888888 xplanation & answer shirish........... =D> =D>
Nothing cud be better................keep it up & keep posting!!!!!!!!
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| smitha
Forum Elite
Topics: 53
Posts: 236
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source: step3notes, usmle.net, first aid fro step2
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| smitha
Forum Elite
Topics: 53
Posts: 236
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i was going thru this, again.........and i wanted to say or add here......werdnig hoffman, which we can say to a certain extent ,resembles ALS in adults, but it involves only LMN ..........and so is called "floppy baby"....with that flaccid paralysis..........
So wat's the drug for this ALS now...........??????anybody???????
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| tamriko
Forum Elite
Topics: 23
Posts: 296
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Hi Smitha, if i am correct, the primary treatment of the ALS is Riluzol. After this symptomatic and supportive care.
Tamar
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| smitha
Forum Elite
Topics: 53
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YES EXACTLY, TAMAR...........
IT'S RILUZOLE..........WHICH REDUCES PRESYNAPTIC GLUTAMATE RELEASE AND HELPS IN REDUCING THE PROGRESSION OF DISEASE!!!!!!!!!!!
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| tamriko
Forum Elite
Topics: 23
Posts: 296
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What is it a Plummer- Vinson's syndrome and when it occurs?
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| smitha
Forum Elite
Topics: 53
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hey tamar!!!!!!!!
seems like ur new to this forum..........u cud've started a NEW POST for urself with this q.............
anyways, PVS includes, atrophic glossitis, anemia and esophageal webs.........seen in iron deficiency ..............
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| smitha
Forum Elite
Topics: 53
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and most importantly.......it's associated with SQUAMOUS CELL CARCINOMA OF ESOPHAGUS...............
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| tamriko
Forum Elite
Topics: 23
Posts: 296
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WELL,YES, I AM NEW IN THIS FORUM AND I FIND IT VERY HELPFUL. I AM GOING TO TAKE STEP 2 IN DECEMBER,THUS,IHAVEN'T TAKEN STEP 1 YET.
YOUR ANSWER IS CORRECT(AS ALWAYS) SMITHA.
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hi guys.......
do you all know this ........
the most sought after proffessor of KAPLAN......Dr. GOLJAN is suffering from ALS..............LOU GEHRIG'S DISEASE.................
of course poppy(as people call him) has recovered from it and is doing good now.............
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