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Cervical myelographic changes in hypertrophic interstitial polyneuropathy.

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Upper Airway
Obstruction in the
Shy-Drager Syndrome
Robert H. Israel, MD,
and Joseph M. Marino, M D
Patients with idiopathic orthostatic hypotension (ShyDrager syndrome) may present with respiratory complications [3]. W e recently had the opportunity to observe a
patient with the Shy-Drager syndrome who developed respiratory failure due to bilateral vocal cord paresis.
A 52-year-old woman was admitted for evaluation of progressive dyspnea. She noted a two-month history of increasing shortness of breath associated with wheezing but had n o
other pulmonary symptoms. Two years earlier she had been
diagnosed as having Shy-Drager syndrome. H e r recent
manifestations included idiopathic orthostatic hypotension,
fecal incontinence, urinary incontinence, dysarthria, cerebellar ataxia, and anhidrosis. H e r previous laboratory evaluation had revealed normal values for hemoglobin, white cell
count, serological tests, cholesterol, triglycerides, serum
thyroxine, electrolytes, serum cortisol, and urinalysis; chest
roentgenograms, electrocardiogram, pneumoencephalogram, and EM1 scan were also unremarkable. O n admission
she was in moderate respiratory distress and was utilizing
her accessory muscles of respiration. Inspiratory wheezes
were audible over the entire thorax. H e r diagnostic workup
included a flowholume loop, which showed an inspiratory
plateau characteristic of upper airway obstruction. Bronchoscopy revealed apposition of her vocal cords indicative
of bilateral vocal cord paresis. Acute respiratory failure
developed, necessitating an emergency tracheostomy. She
was subsequently discharged without respiratory symptoms.
The diagnosis of upper airway obstruction frequently is
not considered in the differential diagnosis of a dyspneic
patient. Many of these patients are misdiagnosed and
treated for asthma or bronchitis. Clinical clues to upper
airway obstruction include: inspiratory wheezing, stridor
over the trachea, and a prolonged inspiratory phase of
Physiologically the extrathoracic trachea has a tendency
to narrow during inspiration because intraluminal pressure
is lower than atmospheric pressure. Thus any narrowing of
the extrathoracic trachea will be accentuated during inspiration.
Normally the vocal cords are held apart by active contraction of the laryngeal muscles. Bilateral vocal cord paresis
causes the vocal cords to rest in the midline, obstructing the
From the Department of Medicine, The University of Rochester
School of Medicine and Dentistry, and S t Mary’s Hospital, Rochester, NY.
Accepted for publication Jan 28, 1977
Address reprint requests to Dr Israel, St Mary’s Hospital, 89
Genesee St, Rochester, NY 14611.
glottic opening. Apposition of the vocal cords as seen
through either direct or indirect laryngoscopy confirms the
Shim et a1 [4]concluded that inspiratory flow is decreased
in upper airway obstruction. They found that the ratio of
maximal midinspiratory flow to maximal midexpiratory flow
was less than 1 in 8 of 9 patients with upper airway obstruction. Other pulmonary function tests such as the maximal
breathing capacity [l] and the flowholume loop [21 are
helpful in diagnosing upper airway obstruction.
1. Jones JS, Renzetti
AD Jr, Mitchell MM: The maximal breathing
capacity in extrathoracic airway obstruction. Am Rev Resp Dis
106:925-927, 1972
2. Miller RD, Hyatt RE: Evaluation of obstructing lesions of the
trachea and larynx by flow-volume loops. Am Rev Resp Dis
1O8:475-48 1, 1973
3. Schwartz GA: The orthostatic hypotension syndrome of ShyDrager: a clinicopathologic report. Arch Neurol 16:123-139,
4. Shim C, Corro P, Park SS, et al: Pulmonary function studies in
patients with upper airway obstruction. Am Rev Resp Dis
106:233-238, 1972
Cervical Myelographic
Changes in Hypertrophic
Interstitial Polyneuropathy
Steven B. Hammerschlag, MD,
Lester S. Adelman, MD,
Elliott M. Marcus, MD,
and Samuel M. Wolpert, iMD
Hypertrophic interstitial neuropathy is usually suspected in
patients with peripheral neuropathy and palpably enlarged
peripheral nerves. We have recently studied an obese patient with this disease in whom the diagnosis was first
suggested by a cervical myelogram. W e can find only 3
previously reported cases in which changes on cervical
myelogram were described [ 1-31.
A 33-year-old woman had been weak since infancy and had
poorly developed musculature. Muscle biopsy at age 14
showed focal atrophy. Nerve conduction studies at age 28
demonstrated severe slowing in motor conduction of the
median and ulnar nerves. Sensory examination was normal.
At age 32 she developed increasing weakness of her extremities and numbness of the left leg.
Examination at age 33 revealed weakness of all extremities and absent deep tendon reflexes. Plantar responses were extensor bilaterally. Pain sensation was de-
From Tufts-New England Medical Center, Boston, MA.
Accepted for publication Feb 1, 1977
Address reprint requests to Dr Adelman, Neuropathology Laboratory,Tufts-new England Medical Center, 17 1 Harrison Ave, Boston, MA 02111.
Notes and Letters
Refire nces
1. Anderman F, Lloyd-Smith DL, Mavor H, et al: Observations on
hypertrophic neuropathy of DGjkrine and Sottas. Neurology
(Minneap) 12:712-724, 1962
2. Bellon EM, Kaufman B, Tucker ME: Neuropathy: plain film and
myelagraph ic changes. Radiology 103:3 19-3 2 2, 1972
3. SyrnondsCP, Blackwood W: Spinal cord compression in hypertrophic neuritis. Brain 85:251-260, 1962
The Inhibitory Effect of
Multiple Scleiosis and
Control Sera on Certain
- elin- Catabolizing
Iris Fletchcr Norstrand, MD, PhD,
and Paul Druck, BS
Cervical myelograrn (anteroposterior view) showing
multiple bilateral intradural extramedullary Jillirrg dejicts
dae to the thickened n e r z e roots (arron7s).
creased distal to the midcalves and midpalms. Vibratory
sensation was diminished distally. Motor and sensory nerve
conduction velocities were slow.
A cervical myelogram showed bilateral intradural, extramedullary filling defects at multiple levels (Figure) and
minimal enlargement of the intervertebral foramens. A
biopsy of the left sural nerve showed a decreased axonal
population with “onionskin” proliferation of the endoneurial fibrous tissue. Both the light and electron microscopical
features were consistent with hypertrophic interstitial
The patient was
nerves could not be palpated because of her obesity.
84 Annals of Neurology Vol 2
No 1 July 1977
Many investigators believe that the initial event in demyelination in multiple sclerosis is digestion of basic protein by
acid proteinase prior to any breakdown of lipids, or before
any focal immunological reaction occurs [ 1-41. Accordingly, we postulated that multiple sclerosis might be due to
lack of an inhibitory substance to this o r some other
myelin-catabolizing enzymes. To examine this hypothesis it
was necessary to show that body fluids (serum or spinal fluid)
from control patients have an inhibitory effect, whereas
body fluids from patients with multiple sclerosis either have
no inhibitory effect or cause activation or induction of the
respective enzymes.
Sera from 26 patients with multiple sclerosis (21 faradvanced and 5 early cases) and from 26 controls ( 10 normal
adults and 16 patients with various other neurological conditions) were tested for the presence or absence of an inhibitor
or activator of the neural enzymes acid proteinase, arylsulfatase, and cerebroside galactosidase, obtained from
homogenates of normal human brain. Similarly, spinal fluid
(both unconcentrated and concentrated ten times by
dialysis) was examined in 8 patients with multiple sclerosis
and 10 controls solely for its effect o n acid proteinase. For
each enzyme studied, the enzyme reaction was run alone
using 0.002 M phosphate buffer, p H 7.4, instead of the
appropriate body fluid. Four separate determinations were
made concomitantly, substituting serum and spinal fluid
from controls and from patients with multiple sclerosis for
From the Neurology Service, Brooklyn Veterans Administration
Hospital, Brooklyn, N Y .
Accepted for publication Dec 21, 1976.
Address reprint requests to Dr Norstrand, Neurology Service,
Brooklyn Veterans Administration Hospital, 800 Poly P1, Brooklyn, NY 11209.
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myelographic, change, interstitial, hypertrophic, cervical, polyneuropathy
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