• Users Online: 89
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 352-353

Acquired postganglionic cholinergic dysautonomia – A rare clinical entity

1 Department of Ophthalmology, Little Flower Hospital, Angamaly, Kerala, India
2 Department of Neurology, Little Flower Hospital, Angamaly, Kerala, India

Date of Submission09-Jul-2020
Date of Acceptance08-Oct-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Meena Chembil Kakkitampara
“Lakshmi” P T Usha Road, Ernakulam - 682 011, Kerala
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2214_20

Rights and Permissions

Acute dysautonomia or dysfunction of the autonomic nervous system is characterized by severe postganglionic sympathetic and parasympathetic dysfunction, with a relative or complete sparing of motor and sensory function. Acquired cholinergic dysautonomia, a very rare condition of failure of the parasympathetic system leads to complete bilateral internal ophthalmoplegia, impaired secretion of tears, and may present with visual symptoms. Herein, we report a rare case of acute cholinergic dysautonomia in an 11-year-old girl.

Keywords: Cholinergic, dysautonomia, internal ophthalmoplegia, postganglionic

How to cite this article:
Kakkitampara MC, Mohan M, Raju AS. Acquired postganglionic cholinergic dysautonomia – A rare clinical entity. Indian J Ophthalmol Case Rep 2021;1:352-3

How to cite this URL:
Kakkitampara MC, Mohan M, Raju AS. Acquired postganglionic cholinergic dysautonomia – A rare clinical entity. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Aug 3];1:352-3. Available from: https://www.ijoreports.in/text.asp?2021/1/2/352/312363

Acquired acute dysautonomia presents dramatically and is often misdiagnosed initially. Dysautonomia refers to dysfunction of the autonomic nervous system, either partially or completely. The autonomic nervous system has a craniosacral parasympathetic, cholinergic division and a thoracolumbar sympathetic, noradrenergic component. Pure pandysautonomia, first described by Young et al. in 1969,[1] is characterized by severe postganglionic sympathetic and parasympathetic dysfunction, with relative or complete sparing of motor and sensory function[2] Initial symptoms are often gastrointestinal (nausea, vomiting, abdominal pain, and diarrhea), followed by orthostatic syncope, bladder dysfunction, impotence, and hyporeactive pupils.[3] Visual symptoms may rarely be the presenting manifestation.[4] Acquired cholinergic dysautonomia, limited to the cholinergic system, causes impaired lacrimation, bilateral internal ophthalmoplegia, and visceral dysfunction.[5] Symptoms of excessive cholinergic activity (diarrhea, hyperhidrosis, and hypersalivation) may briefly precede more sustained cholinergic hypofunction.

Herein, we report a case of acquired cholinergic dysautonomia, rarely encountered in the published ophthalmic literature.

  Case Report Top

An 11-year-old healthy girl presented with defective vision and photophobia of 3 months duration. She described a hot, burning sensation in both eyes while outdoors and absent tear secretion. Difficulty in swallowing and dry mouth were also reported. Her symptoms began acutely, a day after hospitalization with severe vomiting and diarrhea. The abdominal complaints were ascribed to gastroenteritis and treated with intravenous cefotaxime, ranitidine, ondansetron, lactobacillus, digestive enzymes, and camylofin dihydrochloride, a synthetic antimuscarinic smooth muscle relaxant. Her gastrointestinal (GI) disturbances improved gradually but ocular symptoms persisted. An ophthalmologist was consulted and topical pilocarpine was prescribed.

Her past ocular and medical histories were insignificant. She denied consumption of canned food, recent travel, and exposure to drugs, toxins, or medications other than those mentioned. No similar complaints had been described among family members or contacts concurrently or earlier.

She was alert, moderately built, and nourished. Pulse was regular at 100/min and supine blood pressure 100/60 mmHg, with no abnormal postural variation of either. Her skin and mouth were dry. Visual status showed: Right eye- 20/80, near vision N12 with +1.75DS/+0.50DC @ 100 degree 20/20 N6 and Left Eye- 20/80, near vision N12 with +1.75DS/+0.75DC @ 70 degree to 20/20, N6. Pupils were 5 mm, not constricting to direct or consensual light or attempted accommodation [Figure 1] but constricting to 0.1% pilocarpine [Figure 2]. Tear meniscus was reduced with tear-film breakup time shortened to less than 5 s. Schirmer's test gave a value of 2 mm in both eyes without paracaine. The anterior segment and fundus were otherwise normal. Ocular motility was full, including convergence.
Figure 1: 5 mm pupil not responding to light or accommodation

Click here to view
Figure 2: Hypersensitivity to 0.1% pilocarpine instilled in the right eye

Click here to view

Cognition, sensory and motor examination, and gait were normal. Knee jerk was normal but other deep tendon reflexes were hypoactive. Bedside autonomic function tests were negative. Hemogram, erythrocyte sedimentation rate (ESR), renal and liver function tests, serological testing for syphilis and porphyrins, antinuclear SS-A, SS-B, and acetylcholine receptor antibodies were all normal. Magnetic resonance imaging (MRI) of brain and nerve conduction studies showed no abnormality. Based on her ocular signs and systemic symptoms a diagnosis of acquired postganglionic cholinergic dysautonomia was made.

  Discussion Top

Acute acquired dysautonomia presents with dysautonomic symptoms in an otherwise healthy young subject. Immune etiology has been hypothesized, with 20% reporting a viral prodromal and others having coexistent autoimmune diseases. Axonal degeneration on sural nerve biopsies with a decrease in unmyelinated and small myelinated fibers and elevated CSF protein supports the theory that it may be a variant of Guillain-Barre syndrome (GBS).[1],[6],[7]

Barring borderline resting tachycardia and hypotension, dysfunction was restricted to the cholinergic system. Since the lower GI symptoms resolved relatively rapidly in contrast to other reports of cholinergic dysautonomia,[5],[7] GBS with a GI prodrome and autonomic manifestations would enter the differential diagnosis but the significant weakness was not reported. Adie's pupils may rarely be bilateral,[8] especially when associated with Sjogren's syndrome, but the tonic contraction of the pupils was not evident. Pediatric Sjogren's is rare and she had no other manifestations or serological evidence.

Pupillary abnormalities were studied by Bremner and Smith in 150 patients with generalized autonomic failure.[9] Relative mydriasis of the pupil in the light with attenuation of light and near responses was seen with parasympathetic dysfunction while sympathetic dysfunction caused relative miosis in the dark, re-dilatation lag, and attenuation of the startle reflex. The denervated pupil develops supersensitivity to dilute agonists (e.g., 1% phenylephrine for the dilator muscle, 0.1% pilocarpine for the sphincter muscle).

The treatment of acute pandysautonomia is controversial and disappointing. Immunomodulation may be indicated in the acute stage. In the previous few case reports on acquired postganglionic cholinergic dysautonomia, a complete recovery of symptoms was not reported.[7] Corticosteroids show mixed results[1],[6] but good response to intravenous immunoglobulin (IVIg) has been reported.[10] Recovery is often slow and incomplete with persistent gastric motility disturbances and orthostatic hypotension.[7] Since the patient presented 3 months after onset, immunomodulation was not considered. Glasses along with lubricants were prescribed to combat visual symptoms and follow-up monitoring for visual or systemic manifestations advised.

  Conclusion Top

We report a rare case of acute cholinergic dysautonomia in an 11-year-old girl and discuss the diagnosis and possible management strategies in such patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Young RR, Asbury AK, Adams RD, Corbett JL. Pure pan-dysautonomia with recovery. Trans Am Neurol Assoc 1969;94:355-7.  Back to cited text no. 1
Appenzeller O, Kornfeld M. Acute pandysautonomia. Clinical and morphologic study. Arch Neurol 1973;29:334-9.  Back to cited text no. 2
Hart RG, Kanter MC. Acute autonomic neuropathy: Two cases and a clinical review. Arch Intern Med 1990;150:2373-6.  Back to cited text no. 3
Yee RD, Trese M, Zee DS, Kollaritis CR, Cogan DG. Ocular manifestations of acute Pandysautonomia. Am J Opthalmol 1976;81:740-4.  Back to cited text no. 4
Inamdar S, Easton LB, Lester G. Acquired postganglionic cholinergic dysautonomia: Case report and review of the literature. Pediatrics 1982;70976-8.  Back to cited text no. 5
Low PA, Dyck PJ, Lambert EH, Brimijoin WS, Trautmann JC, Malagelada JR, et al. Acute pan autonomic neuropathy. Ann Neurol 1983;13:412-7.  Back to cited text no. 6
Baron R, Engler F. Postganglionic cholinergic dysautonomia with incomplete recovery: A clinical, neurophysiological and immunological case. J Neurol 1996;243:18-24.  Back to cited text no. 7
Saha I, Shroff CM, Gupta C, Verma R. An interesting case of simultaneous bilateral Adie's tonic pupil. Oman J Ophthalmol 2018;11:82-4.  Back to cited text no. 8
[PUBMED]  [Full text]  
Bremner F, Smith S. Pupil findings in a consecutive series of 150 patients with generalized autonomic neuropathy. J Neurol Neurosurg Psychiatry 2006;77:1163-8.  Back to cited text no. 9
Mericle RA, Triggs WJ. Treatment of acute pandysautonomia with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 1997;62:529-31.  Back to cited text no. 10


  [Figure 1], [Figure 2]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded20    
    Comments [Add]    

Recommend this journal