Facial Paralysis OccurringWith HypothyroidismA Report of Two Cases

JERRY M. EARLL, M.D., ANDFELIX 0. KOLB, M.D., San Francisco

NEUROLOGIC COMPLICATIONS of myxedema rangefrom simple headache to convulsion, coma anddeath. This subject was recently reviewed by San-ders,6 who noted that almost 80 per cent of myx-edematous patients complain of paresthesias. Themost frequently impaired cranial nerve is the audi-tory component of the eighth nerve, and 15 to 31per cent of myxedematous patients have dimin-ished auditory acuity.2'4 The vestibular componentof the eighth nerve has been known to be involved,'and vertigo4 and tinnitus2-4 may occur. Althoughhoarseness and dysarthria are commonly noted, itis postulated that local myxedematous infiltrationof the vocal cords and tongue, rather than recur-

rent laryngeal nerve paralysis, is the mechanism.Familial nerve deafness and goiter have been de-scribed, and trigeminal neuralgia has been ob-served in hypothyroid patients.4'6'8 Bell's palsy,associated with hypothyroidism, was mentioned inone patient and no similar association had beenpreviously noted.6 Two patients whose acquiredhypothyroidism was complicated by facial paraly-sis are described in this report.

From the Metabolic Research Unit, University of CaliforniaMedical Center, San Francisco.

Submitted 30 August 1966.Reprint requests to: Metabolic Research Unit, University of

California School of Medicine, San Francisco 94122 (Dr. Earll).

Reports of CasesCASE 1.-A 59-year-old Caucasian man noted

sudden paralysis of the left side of the face in Au-gust 1965. Earlier employment had resulted in ahigh degree of acoustic trauma, and audiogramsin July 1965 confirmed a bilateral high tone lossof 60 per cent. There was pronounced impairmentof speech discrimination. Vestibular function wasintact. When the paralysis did not improve, thepatient was admitted to hospital for decom-pression of the facial nerve in October 1965.The patient had classic myxedematous facies,

low voice, dull wit, a pulse rate of 60 beats perminute and blood pressure of 130/88 mm of mer-cury. The skin was dry and facial features werecoarse enough to suggest acromegaly. There waspitting edema in the lower extremities, and deeptendon reflexes could not be obtained. Completeleft facial nerve paralysis of peripheral type waspresent. Electrical stimulation brought no muscularresponse. The remainder of the examination waswithin normal limits. Ability to taste was nottested.

Results of laboratory studies included hemo-globin 12.6 gm per 100 ml, hematocrit 33 percent and leukocytes 8,950 per cu mm-44 percent neutrophils, 44 per cent lymphocytes, 4per cent eosinophils, 2 per cent basophils, and 6 percent monocytes. Urinalysis showed specific gravityof 1.019 and a 2 plus protein content. Serum elec-trolytes were as follows: Sodium 137, chloride93.5, carbon dioxide 27.3 and calcium 9.2 mEqper liter; phosphorus 3.5 mg per 100 ml. Alkalinephosphatase was 5.2 Shinowara-Jones-Rinehart(SJR) units, and creatinine 1.41 mg per 100 ml.The serum proteins were within normal range,cholesterol 401 mg per 100 ml and triglycerides2,300 mg per 100 ml (normal 50 to 150). Bu-tanol extractable iodine was 0.9 mcg per 100 ml(normal 2.3 to 5.2). Twenty-four-hour radioactive

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iodine uptake was 4 per cent and no increase oc-curred after two days of thyroid stimulating hor-mone, 10 units a day. A Burdick photomotogramT 1/2=600 milliseconds at the ankle (normal 250to 380). X-ray films showed borderline enlarge-ment of the cardiac silhouette. The sella was nor-mal in size, but thinning of the bone at the poste-rior inferior aspect of the sella with minor shapingof the clinoids was suggestive of increased intra-sellar pressure. Views of the hands showed largebone structure with prominent tendon insertions.Heel pads measured 22 and 23 mm. Baseline 24-hour urinary steroids were low (17-hydroxycorti-costeroids 3.3 mg and 17-ketosteroids 2.5 mg).These levels increased to 7.4 and 4.7 mg respec-tively with intravenous infusion of 25 units ofcorticotropin. Total gonadotropins were negativeat 5 mouse units.The patient was treated with 12.5 mcg of L-tri-

iodothyronine daily and the amount was increasedto 50 mcg daily within six days. Because the exactstatus of the pituitary-adrenal axis was not estab-lished, supplementary cortisol was administeredfor the facial nerve decompression, which was per-formed in November 1965. The proximal portionof the vertical part of the nerve was somewhatatrophic, but there appeared to be moderate bal-looning of the nerve from its sheath at the stylo-mastoid foramen, indicating increased pressure.The manifestations of myxedema steadily regressedand when last observed the patient was euthyroidwhile taking 300 mg of thyroxin daily. There wasdefinite improvement in the paralysis, with returnof about 50 per cent function in six months. Audi-tory function improved only a little. The serumlipids decreased significantly, cholesterol to 230mg and the triglycerides to 985 mg per 100 ml.Gonadotropins were positive at 5 and 80 mouseunits, and the serum growth hormone was normalat less than 1 mfug per ml when measured by radio-active immunoassay.

CASE 2.-A 23-year-old Caucasian womannoted the first symptoms of thyrotoxicosis duringher first pregnancy. These symptoms progressedafter delivery in February 1964, and when she con-sulted a private physician in April 1964, a diffusetoxic goiter was confirmed by laboratory studies.She was treated at first with 10 mg of methimazoleevery 8 hours, and later with 100 mg of propyl-thiouracil every 8 hours. The hypermetabolicsymptoms rapidly subsided over a period of sixweeks and she was referred to the thyroid clinic

for further evaluation in July 1964. Right facialparalysis developed suddenly before she enteredthe clinic. The goiter had been enlarging slightly,and she had been feeling cold, depressed and fa-tigued for several weeks. Physical examination re-vealed complete right facial nerve paralysis ofperipheral type. The thyroid gland was twice nor-mal size, the pulse rate was 60 beats per minute,the skin was dry, the hands were cold and reflexeswere definitely delayed. Taste acuity was nottested. The protein-bound iodine was 2.9 mcg per100 ml (normal 3.5 to 8), Lawson kinemometryvalue was 310 milliseconds (normal 200 to 280)and radioactive iodine uptake was 11 per cent in24 hours. The dose of propylthiouracil was re-duced to 50 mg every 8 hours, and there wasprompt improvement in symptoms of hypothyroid-ism, the goiter diminished in size, and the facialparalysis rapidly improved. There was no residualfacial paralysis in October 1964 when the patientwas last seen. She was being maintained on propyl-thiouracil. The pulse rate was 80 beats per minute,she appeared euthyroid and kinemometry revealeda normal value of 240 milliseconds.

DiscussionIn these two patients classic unilateral Bell's

palsy developed in association with acquired hypo-thyroidism. Both experienced the usual rather sud-den onset of paralysis, although in Case 1 there wasrather chronic and severe primary myxedema andin Case 2 a relatively acute iatrogenically-producedhypothyroid state. Prompt recognition and correc-tion of the hypothyroid state in Case 2 was associ-ated with rapid and complete recovery from theparalysis. In Case 1, the hypothyroid state was notrecognized at first and the facial paralysis did notcompletely abate in spite of thyroid replacementand surgical decompression. It is unlikely that thetherapeutic levels of cortisone briefly used in Case1 played a significant role in the patient's improve-ment.The anatomical course and confinement of the

facial nerve, which make it subject to the usualBell's palsy, could account for its vulnerability tomyxedematous infiltration and swelling of soft-tis-sues (Figure 1). Increases in soft-tissue mass oc-cur in both myxedema and acromegaly, and inboth conditions paresthesias and neuropathicchanges are frequently noted. The carpal tunnelsyndrome has been reported in association withmyxedema,7 and in two cases reported by Purnell5

CALIFORNIA MEDICINE 57

Mors Huma Antoy B. J.:Anson coyih'1.66..

Blakiston Division, McGraw-Hill Book Co. U-sed by per-mission. Modified from the original. B, Schematic illus-tration of facial nerve before and after decompression(Case 1).

incision of the transverse carpal ligaments revealedan edematous, noninflammatory thickening of theflexor synovalis about the nerve. In Case 1 hereinreported, the ballooning of the facial nerve fromits sheath during surgical operations suggests asimilar problem with edema and swelling.

SummaryTwo hypothyroid patients in whom reversible

facial paralysis developed are reported. This asso-ciation has not been previously emphasized. Theanatomical course and confinement of the facialnerve make its situation similar to the mediannerve's confinement in the wrist; both may be vul-nerable to the profound metabolic and soft-tissuechanges of hypothyroidism. Prompt recognitionand treatment of the associated hypothyroidismshould offer the best chance for complete recovery.

REFERENCES1. Barlow, R. A.: The study of vestibular nerve func-

tion in myxedema, Amer. J. Med. Sci., 164:401, 1922.

2. Bloomer, H. A., and Kyle, L. H.: Myxedema: Are-evaluation of clinical diagnosis based on eighty cases,Arch. Intern. Med., 104:234, 1959.

3. Hollender, A. R.: Hypometabolism in relation toear, nose, and throat disorders, Arch. Otol., 63:135, 1956.

4. Nickel, S., and Frame, B.: Neurologic manifesta-tions of myxedema, Neurology, 8:511, 1958.

5. Purnell, D. C., Daly, D. D., and Libscomb, P. R.:Carpal tunnel syndrome associated with myxedema,Arch. Intern. Med., 108:751, 1961.

6. Sanders, V.: Neurologic manifestations of myx-edema, New Engl. J. Med., 266:547, 599, 1963.

7. Schiller, F., and Kolb, F. O.: Carpal tunnel syn-drome in acromegaly, Neurology, 4:271, 1954.

8. Watts, F. B.: Atypical facial neuralgia in hypothy-roid state, Ann. Intern. Med., 35:186, 1951.

Subcutaneous Fat NecrosisWith Acute HemorrhagicPancreatitisA Case in a Child with Steroid-Resistant

Nephrosis Treated with 6-Mercaptopurine

EVA HEUSER, M.D., ELLIN LIEBERMAN, M.D.

GEORGE N. DONNELL, M.D., AND

BENJAMIN H. LANDING, M.D., Los Angeles

PANCREATITIS has frequently been described insteroid-treated patients who come to autopsy.* Thehistologic process is frequently mild and is an in-cidental finding, pancreatitis not having been man-ifested clinically. 2'412,15,22 The following casereport illustrates the occurrence of clinically inap-parent acute hemorrhagic pancreatitis in a six-year-old Negro boy with steroid-resistant nephrosis. Thepancreatitis was associated with massive dissemi-nated fat necrosis which extensively involved sub-cutaneous tissues. A final complication in thispatient was generalized fungal infection.

Report of a Case

The patient, a six-year-old Negro boy, was ad-mitted to Childrens Hospital of Los Angeles with

From the Departments of Pathology and Pediatrics, Universityof Southern California School of Medicine, and the Departmentof Pathology and Division of Renal-Metabolic Disease, ChildrensHospital of Los Angeles.

Submitted 16 August 1966.Reprint requests to: Childrens Hospital of Los Angeles, 4614

Sunset Boulevard, Los Angeles 90027 (Dr. Heuser).*Reference Nos. 2, 4, 12, 15, 22.

58 JANUARY 1967 * 106 * 1