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Floppy head syndrome.

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LETTERS
13 1
(1). However, physicians measuring the finger to floor
distance must be aware that hypermobile or even normal hip movement may mask loss of spinal movement.
This patient had HLA-B27 negative spondylitis,
psoriasis, anterior uveitis, and Crohn’s disease with generalized joint hypermobility. She had developed the low
backache and stiffness characteristic of ankylosing
spondylitis 30 years prior to diagnosis (3). Her complaints had been repeatedly minimized because of her
ability to touch the floor with her fingers.
Objective assessment of her spine revealed virtual absence of movement in all planes. Radiologic examination confirmed definite evidence of psoriatic
spondylitis.
It is important to measure accurately spinal
movement in patients with spondylitis. Less specific
measurements such as finger-to-floor distance may be
misleading in the presence of generalized joint hypermobility.
M. L. LEBLANC,MD
WM. G. BENSEN,MD, FRCP (C)
W. M. GOLDBERG,
MD, FRCP(C)
St. Joseph’s Hospital
McMaster University
Hamilton, Ontario, Canada
REFERENCES
1. Neustadt DH: Ankylosing spondylitis. Postgrad Med
61:124-135, 1977
2. Moll JMH, Wright V: Normal range of spinal mobilityan objective clinical study. Ann Rheum Dis 30381-386,
1971
3. Calin A, Porta J, Fries JF, Schurman DJ: Clinical history
as a screening test for ankylosing spondylitis. JAMA
273~2613-2614, 1977
Floppy head syndrome
To the Editor:
In an article that appeared in your journal entitled “Hypothyroidism Presenting as a PolymyositisLike Syndrome,” by Hochberg et a1 (l), W. M. Ord was
cited for the first description in 1879 of the muscular
weakness of hypothyroidism (2). In reviewing Ord’s
study, it is noted that his patient had severe neck weakness to the point that:
. . . she could not keep her head erect; in spite of all
her efforts to the contrary it would fall forward, and
the chin, resting on the lower part of the neck, would
hinder swallowing and even embarrass her breathing.
After a careful review of the literature, we were
unable to find any subsequent report in the literature resembling that of Ord. However, we would like to present a very similar case, the first since the original description of this unusual presentation of hypothyroidism
100 years ago.
Case report. LA, a 67-year-old woman, was admitted to St. John Medical Center for evaluation of extreme neck muscle weakness of one year’s duration. The
patient had been in good health until she began having
progressive inability to hold her head erect. At the time
of admission she said that she was unable to raise her
head without the use of her hands. She denied any other
areas of muscle weakness, though she had mild, generalized fatigue. No cold intolerance was noted.
Physical examination revealed a slender white
woman in no distress with pulse 72/minute, blood pressure 142/70, and normal temperature. There was mild
alopecia noted in the temporal areas. The thyroid gland
was normal size. All muscle groups had normal strength
except the paracervical group which was markedly
weak and rigid to touch. She was unable to raise her
chin off her chest without the help of her hands. Deep
tendon reflexes were interpreted as normal throughout.
The remainder of the examination showed normal results.
Laboratory studies included normal hematologic
findings except for an hematocrit of 26.2. Admission T3
Ru was 15.4% (normal: 16.4-31.4%), T4 1.0 mEq/dl
(normal: 5.1-13.3 mEq/dl), and T7 0.2 (normal: 1.42.9). Westergren sedimentation rate was moderately elevated at 60 mm/hour. Antithyroglobulin antibodies
were not found. Thyroid-stimulating hormone was 47.4
microunits/ml which was essentially four times the upper limits of normal. Elevated muscle enzymes included
an elevated SGOT at 5 1 units/liter (normal: 0-40 units/
liter), LDH 377 units/liter (normal: 100-225 units/
liter), and CPK 686 units/liter (normal 0-145 units/
liter). CPK isoenzymes confirmed muscle origin. No
antinuclear antibodies or rheumatoid factor were found
by the latex fixation test. Cervical x-rays showed degenerative changes at the C5/C6 interspace. An EMG on
the muscles of her extremities gave normal resplts.
A muscle biopsy of the right deltoid muscle did
reveal nondystrophic, noninflammatory degenerative
muscle disease with irregular, patchy areas of individual
basophilic degeneration of muscle fibers. This was considered to be characteristic of a metabolic disease such
as hypothyroidism.
The patient was started on thyroid replacement,
LETTERS
132
initially with Synthroid 0.1 mg daily, then gradually increased to 0.2 mg daily. Within one week after the initiation of treatment her neck muscles had strengthened
greatly, and when seen as an outpatient one month
later, her muscle strength had completely returned to
normal. Her hematocrit and muscle enzyme abnormalities had also corrected within 2 months after therapy
was started.
The case is unique in that the patient had little
clinical evidence of hypothyroidism. We present this report as a reminder of Ord’s earlier observation that hypothyroidism be considered in the differential diagnosis
of severe neck muscle weakness, an observation still
valid a century later.
ARNOLDL. KATZ,MD
Assistant Professor of Medicine
DAVIDPATE,MD
Senior Resident
Oklahoma University
TulsaMedical College
Tulsa, Oklahoma
REFERENCES
1. Hochberg MC, Koppes GM, Edwards CQ, Barnes HV,
Arnett FC. Hypothyroidism presenting as a polymyositislikesyndrome. ArthritisRheum 19:1363-1366,1976
2. Ord WM: Cases of myxoedema. Trans Clin SOCLondon
13:15-19.1880
Malnutrition does not ameliorate systemic
lupus erythematosus
To the Editor:
Studies of mouse models of lupus erythematosus
(LE) indicate that malnutrition prolongs survival (1)
and ameliorates nephritis (2) even though effects on immunologic measures of disease vary (3-5). In human
beings malnutrition markedly reduces immune response
(6,7). These data have suggested that “therapeutic”
trials of induced malnutrition may be helpful in human
LE (2).
Natural experiments offer clinical tests of this
hypothesis which bypass the moral conundrum posed
by offering starvation as “therapy.” We report three
such experiments in which malnutrition did not appear
to lessen LE activity.
Details of the patients are given in Table 1. All 3
patients had active nephritis while malnourished. Patients l and 2 had clinically obvious LE during malnutrition, and diagnosable but milder disease when wellnourished (and treated). Patient 2 did not have antiDNA antibody while malnourished but did when wellnourished. Patient 3, with prior severe LE, had daily fever to 4OoC and had lymphopenia and new-onset paresthesias at the time of her death but had no other clinical
or serologic manifestations of LE. Her fever had been
attributed during life to continued, previously documented, atypical mycobacterial infection; no residual
infection was found at autopsy. The paresthesias had
Table 1. Relationship of clinical and serologic manifestations of SLE to weight in 3 patients with malnutrition
Patient
Reason for
malnutrition
1
Chronic vomiting and diarrhea
2
Food refusal (“anorexia nervosa”)
-
3
-
Anorexia due to disseminated atypical
mycobacterial infection and antituberculosis drug hepatitis
Date
Weight
(kg)
ANA
AntiDNA%
binding
(N
(20%)
9/72
42.7
4+P*
2/73
3/71
63.21.
41.74
1/73
1/78
2/79
(N=
150+)
Signs of lupus activity
97
149
3+D
69
5
207
105
71.8t
63.2t
I+
2+D
32
62
191
46.0
I+
9
184
Arthritis, pleurisy, abdominal pain,
proteinuria (biopsy: membranous),
possible bowel vasculitis
Proteinuria
Psychosis, rash, arthritis, fever, nephrosis
(biopsy: mesangial), seizures, chorea
None
Rash, alopecia, proteinuria (biopsy:
membranoproliferative)
Fever, lymphopenia, paresthesias;
autopsy: membranoproliferative
nephritis; porosis of spinal cord no
residual infection
* P = peripheral; D = diffuse.
*
CH50
p/ml
t Nearest documented approximation of premorbid weight.
With edema and ascites; “dry” weight not obtained until recovery from malnutrition.
156
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