Intractable Case


Report and

Schenley Co,

Literature Review


pharynx open. This results in a highly atomized naso-oral discharge with droplets 0.1 to 0.2 mm in diameter expelled at a velocity of 30 m/s or

\s=b\ Intractable

sneezing is rare; the 12th is reported here. Various causative factors have been identified in the literature; these include psychologic problems, cervical lymphadenitis, epilepsy, and allergy. The sneeze reflex consists of two phases, nasal and respiratory, which are mediated by cranial nerves V and VII and by brain-stem respiratory centers. Hypotheses that have been proposed to explain the multiple causation of sneezing are the concept of the trigeminal system as a central neuronal pool, optic-trigeminal summation, and parasympathetic generalization. (Arch Neurol 36:111-112, 1979) case

a distance of 0.5 to 1.6 m.1 ·-' The Talmud calls it "a pleasure sent from God." Pope Gregory (540-604) believed it to be a prodromal symptom of the plague and popularized the exclamation "God bless you!" And public health is concerned about it as a possible mode of disease transmission. However, little is found in medical literature about sneezing as a neuro¬ logical problem. Only 11 cases of intractable sneezing have been re¬ ported (Table). Following is a report of intractable sneezing in an 11-yearold girl, the 12th documented case.

more, to

neezing is a reflex that results in a spasmodic inspiration followed by a spasmodic expiration. Closure of the nasopharynx momentarily occurs during expiration until increased inO ^

trathoracic pressure forces the

ty of New Mexico School of Medicine, and the Veterans Administration Hospital, Albuquer-

Reprint requests to Department of Neurology, University of New Mexico School of Medicine, Albuquerque, NM 87131 (Dr Co).

Reported Cases Source



of Intractable


Onset After transfer to




After flu




yr 40

Murray and Bierer,"


Kammermier,5 1955


3 yr

Birch et al,2 1959




Elkins and Milstein,* 1962 Kofman,' 1964






700/30 min 8-10 each




job bicycle in country

fever and "nervousness." Examination disclosed




Outcome Cured

Sent home




rate of 10 to 20

times per minute. Tickling the nose resulted in a greater frequency of sneez¬ ing. The turbinâtes were swollen. There were no other abnormal findings. The CBC count, serum electrolyte values including Ca and PO„ blood gases, thyroid function tests, CSF examination, serum and CSF VDRL, roentgenograms of the chest, skull, and sinuses, computed tomographic scan, and audiogram were all normal. The trigeminofacial reflex latencies were nor¬ mal bilaterally. A Schirmer's test disclosed tearing of 10 mm/5 min on the right and 40


Shilkret,3 1949

3V2 wk

ultrasonic nebulized inhalations, tetracycline, and phénobarbital, all to no avail. The patient and her family had histories of hay

Diagnosis Psychogenic

20 to 26 times per minute. The other symp¬ toms subsequently resolved, but the sneez¬ ing continued until admission, interrupted only during five to six hours' sleep at night.


yzine hydrochloride (Atarax), diazepam (Valium), diphenylhydramine hydrochlo¬ ride (Benadryl), lidocaine (Xylocaine) ap¬ plication to the nasal mucosa, intravenous fluids, vitamins, corticotropin injections,

girl sneezing

An 11-year-old girl was admitted to the University of New Mexico Medical Center, Albuquerque, on Jan 27, 1977, for sneezing of 22 days' duration. She had been asymp¬ tomatic until she slept with two cats. The next morning she developed rhinorrhea, tearing, and started sneezing at a rate of

Accepted for publication Dec 2, 1977. From the Department of Neurology, Universi-

house dust, and cat hair. The CBC count, eosinophil count, serum electrolyte values, skull and chest roentgenograms, urinalysis, and ear, nose, and throat examination were normal. She had been treated with dexa¬ methasone (Decadron) nasal spray, hydrox-




This resulted in an aching chest wall, inability to eat, weight loss, and poor school performance. She had been seen by several specialists, and skin tests were positive for pollen,







Associated Press report, 1967" Kaplan and Lanoff," 1970

Present case, 1977

20 yr





15 12




in school Cervical TB adenitis



Psychogenic "Pseudo"


1 mo


After moving house



3 wk


Separation from publicity Hypnosis

Cured Cured

Psychogenic Epilepsy






Opérant conditioning



Psychiatric therapy


Psychogenic Psychogenic


days 1/s



days mo





After cold After


with cats


Epileptiform EEG


Psychiatric therapy Conditioned response to normal saline


Densensitization, antihistaminics, anticonvulsants,


psychotherapy of mother

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mm/5 min on the left (normal, 10 to 15 mm/5 min). A waking EEG, recorded with the patient sneezing every five seconds, showed delta transients over both posterior regions, unrelated to the sneezing. An EEG recording during sleep, while sneezing was absent, showed activation of generalized atypical polyspike and wave complexes prominent over the anterior regions. Phenylephrine (Neosynephrine) nasal spray, diphenylhydramine (Benadryl), phenytoin, and carbamazepine were tried, without effect. The patient sneezed more frequently in the presence of her doctors and parents, and as infrequently as once in 15 minutes when left alone. She was extremely submissive, often seeking ap¬ proval of parents and physicians. The mother was overprotective. The patient left the hospital in nine days. Carbamazepine was not helpful. The mother was persuaded to seek psychiatric help. After the mother underwent therapy, the patient sneezed less and stopped alto¬ gether in mid-April 1977. COMMENT

Of the 11 previously described patients with intractable sneezing, three were male and eight were female. Their ages ranged from 11 to 40 years; eight of the 11 were less than 20 years old. Six of the 11 patients had a background of allergy. The sneezing was

attributed to seizure disorder in

patient and to tuberculous cervical lymphadenitis in another. The re¬ maining nine cases were thought to be of psychogenic origin. Eight of those patients experienced remission with hypnosis, opérant conditioning, or psychotherapy. The patient who had temporal lobe spikes improved with anticonvulsants.7 The patient with tuberculous adenitis stopped sneezing after lymph node resection and treat¬ ment with antituberculous drugs.7' The anatomic pathways and physi¬ ology of the sneeze reflex are complex. The sneeze consists of two phases, the nasal and the respiratory. The nasal phase is usually initiated by nasal irritants, which produce an afferent impulse conducted via the fifth cranial nerve to the trigeminal sensory neu¬ one

in the brain stem and upper cervical cord. The efferent component is carried by the autonomie portion of the seventh cranial nerve to the lacri¬ mal glands and the vessels of the nasal mucosa via the nervus intermedius, rons

greater superficial petrosal


vidian nerve, sphenopalatine gan¬ glion, and postganglionic palatine nerves.1- The nasal phase results in congestion of the nasal mucosa and secretion by its glands of slightly viscid mucus. Its duration is variable, depending on the intensity, duration, and type of the stimulus and the speed

which the efferent impulse produces glandular secretion and vas¬ cular congestion. The congestion and secretions initiate the more rapid respiratory phase, with afferent im¬ pulses again carried by cranial nerve V to the trigeminal sensory neurons. Brain-stem centers for inspiration, closure of the nasopharynx, and expi¬ ration are then activated, and the efferent arc of the respiratory phase with

is initiated.1·8 The multisynaptic sneeze reflex is susceptible to various influences, and stimuli other than nasal irritation can produce a sneeze. The list by Holmes et al10 of various causes of nasal engorgement and sneezing includes not only local nasal factors but also light, chilling, sexual excitement, menstruation and pregnancy, and resentment and frustration. This can be explained by Crue et al's11 concept of the trigeminal system as a central sensory neuronal pool in the brain stem and upper cord, receiving input from the cerebrum, various cranial nerves, visceral afférents of the autonomie nervous system, and upper cervical nerve roots. Thus, tickling of the external ear canal; altered sensory afférents from the neck (as in tuber¬ culous cervical lymphadenitis); altered autonomie tone during chilling, men¬ struation, pregnancy, and sexual ex¬ citement; and cerebral activity asso¬ ciated with psychologic problems or epilepsy have been recognized as factors that can contribute to sneez¬


Everett,12 in his article sneezing, discusses




two other

hypotheses by which various stimuli produce a sneeze. The first is optictrigeminal summation, a vague con¬ cept postulated to explain photopho¬

bia, by which visual afférents also stimulate the trigeminal sensory sys¬ tem. The second is parasympathetic generalization, a phenomenon by which stimulation of one branch of the

parasympathetic leads to spread of



excitation to other branches. This has been observed in the association between urination, shivering, and lacrimation. Similarly, light, in addition to producing miosis, may also stimulate the nervus inter¬ medius and produce nasal engorge¬ ment and a sneeze. Several factors may have contrib¬ uted to our patient's intractable sneezing: her peculiar psychologic pro¬ file, epileptiform EEG activity, strong history of allergy, and perhaps an abnormal parasympathetic tone of the nasolacrimal glands as indicated by the abnormal Schirmer's test. The

CBC count and nasal secretions, how¬ ever, did not show increased eosin-

ophils, and there did not appear to be any significant abnormality of cranial nerves II, VIII, IX, or X, of upper

cervical roots, or of local nasal struc¬ tures. Our patient's waking EEG did not show spikes coincident with her sneezing. It was only during sleep, when sneezing was absent, that the

epileptiform discharges appeared. Moreover, phenytoin and carbamaze¬ pine therapy did not alter the sneez-, ing. Probably the only demonstration of a direct relationship between seizures and sneezing, aside from Kofman's case,7 is the description Penfield and Jasper171 of a man with psychomotor seizures secondary to an arteriove¬ nous malformation who, during brain surgery, sneezed and exhibited chew¬ ing movements when point 11 of the

right temporal lobe




Although psychological problems

and positive allergic histories have been present in many cases, the complex anatomic and physiologic substrate underlying the sneeze makes many other factors at least potentially significant. A thoughtful and methodical evaluation of nasal, neurological, and psychological func¬ tion is essential to the understanding and management of intractable sneez¬ ing in the individual patient. References 1. Brubaker A: The physiology of sneezing. JAMA 73:585-587, 1919. 2. Birch C, et al: Sneezing. Practitioner 182:122-124, 1959. 3. Shilkret H: Psychogenic sneezing and yawning. Psychosom Med 11:127-128, 1949. 4. Murray N, Bierer J: Prolonged sneezing. Psychosom Med 13:56-58, 1951. 5. Kammermier R, cited by Shapiro SL: Parox-$ ysmal sneezing. Eye Ear Nose Throat Mon 46:1532, 1967. 6. Elkins M, Milstein J: Hypnotherapy of pseudosneezing: A case report. Am J Clin Hypn 4:273-275, 1962. 7. Kofman 0: Paroxysmal sneezing. Can Med Assoc J 91:154-157, 1964. 8. School girl sneezes but feels fine, cited by Kaplan M, Lanoff G: Intractable paroxysmal sneezing: A clinical entity defined with case reports. Ann Allergy 28:26, 1970. 9. Kaplan M, Lanoff G: Intractable paroxysmal sneezing: A clinical entity defined with case reports. Ann Allergy 28:24-27, 1970. 10. Holmes T, Goodell H, Wolf S, et al: The Nose. Springfield, Ill, Charles C Thomas Publisher, 1950, pp 4, 16, 52. 11. Crue B, Todd E, Carregal E, et al: Cranial neuralgia, in Vinken P, Bruyn G (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1968, vol 5, p 287. 12. Everett H: Sneezing in response to light. Neurology 14:483-490, 1964. 13. Penfield W, Jasper H: Epilepsy and the Functional Anatomy of the Human Brain. Boston, Little Brown & Co, 1954, p 453.

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Intractable sneezing: case report and literature review.

Intractable Case Sneezing Report and Schenley Co, Literature Review MD pharynx open. This results in a highly atomized naso-oral discharge with...
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