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 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 1999  |  Volume : 47  |  Issue : 2  |  Page : 130-2

Acute dysautonomia following mumps.


Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, 695011, India.

Correspondence Address:
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, 695011, India.

  »  Abstract

Pure acute or subacute dysautonomia is a rare entity. Its etiology is as yet unknown. However, majority of these cases have a preceding viral infection such as herpes simplex, infectious mononucleosis, rubella or coxsackie B. A unique patient in whom acute dysautonomia followed mumps is reported.

How to cite this article:
Mathuranath P S, Duralpandian J, Kishore A. Acute dysautonomia following mumps. Neurol India 1999;47:130


How to cite this URL:
Mathuranath P S, Duralpandian J, Kishore A. Acute dysautonomia following mumps. Neurol India [serial online] 1999 [cited 2017 Nov 21];47:130. Available from: http://www.neurologyindia.com/text.asp?1999/47/2/130/1637




   »   Introduction Top


Autonomic dysfunction as a component of peripheral neuropathy is a common occurrence. However, a pure acute autonomic neuropathy is rare. [Table I] lists the causes of autonomic dysfunction complicating neurological disorders. We report here a case of pure acute dysautonomia following mumps and a brief review of the entity.


   »   Case report Top


A 30 year old lady developed fever and bilateral parotid swelling with dryness of mouth. She was clinically diagnosed to have mumps. Seven days after the swelling subsided she acutely developed transient burning paraesthesias extending below the neck. It was followed by incapacitating postural syncope. A day later she developed severe abdominal colic, associated with vomiting and intermittent diarrhoea. The following day she had intermittent profuse generalized diaphoresis and urinary retention requiring catheterisation. At the time of admission she was conscious and complained of increased thirst. Higher mental functions, optic fundi and cranial nerve examination were unremarkable except for small pupils, sluggishly and symmetrically reacting to both light and accommodation. Horner's sign was not detected. Muscle bulk, tone and power in all the limbs were normal. Objective sensory examination did not reveal any superficial or deep sensory loss, hyperaesthesias or trunkal sensory level. All deep tendon jerks were absent. There were no cerebellar signs or peripheral nerve thickening. There was marked symptomatic postural hypotension unaccompanied by cardioacceleration. The supine blood pressure was 150/100mm of Hg which fell on standing to 60 mm of Hg (systolic) with unrecordable diastolic pressure. The heart rate remained unchanged at 88 beats/min irrespective of the postural alterations.

The blood biochemistry, haemogram and serum protein electrophoresis were normal. Urinary porphobilinogen was negative. Cerebrospinal fluid (CSF) cytology and biochemical studies, done in the 1st and 3rd week of neurological illness were within normal limits. CSF viral cultures were negative for mumps virus. Complement fixation tests for mumps `S' and `V' antigens were not performed. Motor and sensory nerve conduction studies done on the median, ulnar, peroneal, tibial and sural nerves during the 1st and 3rd week of neurological illness were essentially normal except for an absent F-response on peroneal nerve stimulation in the 3rd week's study. Electromyographic examination was not done.

The bedside autonomic tests revealed absent sympathetic skin response (SSR) ; RR ratio of 1.1 in response to valsalva maneuver, (valsalva ratio) (normal > 1.4); expiration : inspiration RR ratio of 1.0 (normal > 1.2); no response in heart rate to standing (normally increases by 5-30 beats/min); documentation of postural drop (mentioned earlier). All the tests thus showed abnormal responses. Secretomotor and gastrointestinal motility tests were not performed.

The patient was initiated on symptomatic treatment with fludrocortisone (0.1mg daily) and omeprazole to which she showed good response. In view of this response and the stable course of her illness, plasmapheresis was not initiated. Six weeks later she was asymptomatic except for occasional postural giddiness and continued to require fludrocortisone. Her supine blood pressure was 110/70 mm of Hg. On standing it fell to 100/56 mm of Hg though she remained asymptomatic. There was no cardioacceleration.


   »   Discussion Top


Young et al briefly reported a patient in 1969 and described the results of autonomic tests on him in 1975,[1] giving the first detailed description of pure pandysautonomia. Autonomic dysfunction associated with various conditions mentioned in [Table I], are excluded from the entity of pure pandysautonomia[2],[3]

The additional characteristics which qualify for the diagnosis of pure pandysautonomia include acute or subacute onset; monophasic course; and gradual partial or complete recovery.[3] Majority of patients presenting with pandysautonomia have a preceding infection,[3],[4] which includes herpes simplex, infectious mononucleosis, rubella and coxsackie B infection.[4],[5]

A variety of neurological sequelae to mumps have been reported including meningitis, encephalitis, myelitis and rarely polyneuropathy of a Guillain-Barre syndrome type.[6] Muscle weakness associated with a lymphocytic pleocytosis mimicking poliomyelitis has also been reported to accompany the acute illness.[7] Polyneuropathy reported with mumps develops one to three weeks following the viral infection[8] and in the majority of cases is preceded by mumps orchitis.[9]

To the best of our knowledge, there is no previous report of mumps preceding acute pure dysautonomia. Our patient, by clinical consideration, had mumps, though CSF viral culture was negative. It is well known that the virus is detectable only upto 4 days after the infection. Our patient also had sensory symptoms and absent F-response in the peroneal nerve which raises the possibility of a proximal involvement of the somatic nerve. In other postinfectious acute pure dysautonomias, mild sensory symptoms have been reported though objective sensory signs or electrophysiological evidence for somatic nerve involvement were extremely rare.

The definite aetiopathogenesis of acute pure dysautonomia remains uncertain, though a possible postganglionic immune-mediated inflammatory mechanism has been suggested by Suarez et al.[3] Some authors have reported electroencephalographic abnormality and a transient hypothalamic dysfunction.[10] Okada and Shintomi reported abnormal eye movements in a few patients. Based on all these reports, they postulates an affection of cholinergic neurons within the CNS that share antigenic similarities to the postganglionic autonomic neurons.[11]

As a general rule, almost all patients slowly recover over months, though some may show only partial recovery. Management is essentially symptomatic. On the basis of their findings, Suarez et al[3] have suggested a trial of plasmapheresis or steroids as a specific management during the acute phase, if symptoms are severe.

 

  »   References Top

1.Young RR, Asbury AK, Corbett JL et al : Pure pandysautonomia with recovery. Description and discussion of diagnostic criteria. Brain 1975; 98 : 613-636.   Back to cited text no. 1    
2.Hopkins A, Neville B, Bannister R : Autonomic neuropathy of acute onset. Lancet 1974; 1 : 769-771.   Back to cited text no. 2    
3.Suarez GA, Fealey RD, Camilleri M et al : Idiopathic autonomic neuropathy : Clinical, neurophysiological and follow-up studies on 27 patients. Neurology 1974; 44 : 1675-1682.   Back to cited text no. 3    
4.McDougall AJ, McLeod JG : Autonomic Neuropathy II : specific peripheral neuropathies (review). J Neurol Sci 1996;   Back to cited text no. 4    
5.Pavesi G, Gemignani G, Macaluso GM et al : Acute sensory and autonomic neuropathy : possible association with coxsackie B virus infection. J Neurol Neurosurg Psychiatry 1992; 55 : 613-615.   Back to cited text no. 5    
6.Ross Russell R, Donald JC : The neurological complication of mumps. BMJ 1958; 2 : 27-30.   Back to cited text no. 6    
7.Lennette EH, Caplan GE, Magoffin RL : Mumps virus infection simulating paralytic poliomyelitis. Pediatrics 1960; 25 : 788-797.   Back to cited text no. 7    
8.Bhatia B, Misra S : Acute peripheral neuropathy following mumps. Neurol India 1994; 42 : 108-110.   Back to cited text no. 8    
9.Duncan S, Will RG, Catnach J : Mumps and Guillain-Barre syndrome (letter). J Neurol Neurosurg Psychiatry 1990; 53 : 708-712.   Back to cited text no. 9    
10.Harik SI, Ghandour MH, Farah FS et al : Postganglionic cholinergic dysautonomia. Ann Neurol 1977; 1 : 393-396.   Back to cited text no. 10    
11.Okada F, Shintomi Y : The central sites of autonomic nervous system may also be disturbed in some cases with acute pandysautonomia (letter). Arch Neurol 1990; 47 : 127-128.   Back to cited text no. 11    
12.Mathias CJ : Disorders of Autonomic System. In : Neurology in Clinical Practice, Vol. II, Bradley WG, Daroff RB, Fenichel GM et al (Eds.) 2nd edition. Butterworth-Heinemann, Boston 1996; 1957.   Back to cited text no. 12    

 

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