There is a 49 year old patient who started exercising for the first time in her life. She said that she notices that her left half of the face does not sweat at all whereas the right half of the face sweats quite normally. She is unsure whether the same happens on the rest of her body. This symptom is not prominent enough for the others to have noticed it but she has not asked anyone else to specifically look for it. This is not causing her any problems but she is just concerned that this might be a symptom of an underlying problem. She also has a history of idiopathic trigeminal neuralgia 2 years back. She is also a pre-diabetic with well-controlled blood glucose levels. There are no associated symptoms. What do you think it could be? Diabetic autonomic neuropathy? Menopause? Normal variation? Subjective symptom she just noticed because she exercised for the first time in her life? Something else? Thanks in advance for your help!
This is most likely Harlequin syndrome. See e.g.
http://en.wikipedia.org/wiki/Harlequin_syndrome
for more information (and the references therein).
Which kind of sport is she doing? Maybe she has a new onset horner syndrome due to a carotid artery dissection? Did she noticed an ipsilateral ptosis and an ipsilateral miosis? If yes, then I suggest she goes urgently to the emergency room...
I agree with Nicola Maggio. Which exercises or actual manual therapy of the neck are known? Anhidrosis only in the left half of the face means lesion of the 3. sympathic neuron 1. in the Ganglion cervicale superius or 2. in the sympathic fibers of the plexus caroticus, most often as result of a dissecion of the left A. carotis interna. If you see an ipsilateral miosis and use Phenylephrin, you will not get a mydriasis!. There ist danger of stroke!
I aggree with Michel, Nicola and Karl. It sounds like a lesion in the sympathic nervus system. As she recognises the anhidrosis only in the left have of her face a lesion in the upper part of the sympathicus is likely. The most likely cause of a this carotis dissection. In your description I miss the neurological examination. Was there any sign of Horner´s Syndrom? The is still in the age where a dissection is the likely cause of a stroke.
But...in your case description there was no hint for typical pain due to ICA dissection (behind the eye ball etc...), which I know is not obligatory....there are a asymptomatic ICA dissection as well...
I would be interesting to know what kind of sport she did. Did involve rapid and wide head moving like when you fasten your seat belt in a car?
Domingo´s suggestions is very interesting. The Ross syndrome is a familial anhydrosis which involves anhydrosis mainly at chest and stomak, myotonic pupils and absent deep tendon reflexes. It is very rare.
So Asfandyar...we need more information.
You have to check parotis grandula. In addition, both symptoms (neuralgia and lack of sweathing) are signs of damage of n trigeminus
It could be Ross syndrome. To look the distribution of sweat/anhidrosis you cam make Mirror test (easy and cheap). The distribution of abnormal sweating is essential to make a diagnosis
My wife is suffering from anihydrosis of the enitire body since birth and has not noticed till two years back. For the past two years she has been suffering from fibromyalgia like symptoms following an attack of Chikungunya . All investigations towards fibromyalgia turned out to be negative including electro myo graphy. Now her symptoms of fibromyalgia is worsening. The doctors are of the opinion that her current symptoms and health problems are complex because of a) toxins accumulations because of anihydrosis and b) chikungunya. Could any one help me what investigations need to be done to pin point the problem?
Yuvaraj, in my opinion it could be Fabry-Anderson-disease with connatal anhidrosis and late onset of neuropathic pain; could be treated (by i.v. -encymsubstitutes and carbamacepine or pregabaline per os).
I propose 1. an opthalmoscopic and 2. a dermatologic examination, to look for small corneal lesions and small angiomas.
@ Yuvaraj Jayaraman: get your wife reviewed by a decent Rheumatologist - if you are based in New Delhi, try Prof Anand Malaviya or Dr Rohini Handa (AIIMS).
...........the time period of onset is unclear! I would attempt to rule out Horner's first & then look for uncommon causes as mentioned above.......
A patient with the same problem had a sympathetic ganglion tumour in thorax. He preferred not to treat it with surgery. He was the same, without changes, ten years later.
Anderson fabry is an X linked disorder and is unlikely in this female patient, and a lesion in the Carotid artery is not necessarily dissection and could be for e.g. Carotid body tumour. simply do a TCD.
A lesion in the sympathetic chain would present not only with hemianhidrosis, but also with ptosis and miosis (Horner Syndrome).
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