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Central pontine myelinolysis diagnosed by magnetic resonance imaging.

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Central Pontine
Myelinolysis Diagnosed
by Magnetic
Kesonance Imaging
Katsuhiko Takeda, MD, Manabu Sakuta, MD,
and Fumihiko Saeki, MD
We have recently treated a 58-year-old nonalcoholic woman
admitted initially to another hospital because of a bone fracture. Bone fixation was performed under general anesthesia.
After recovery, the patient complained of headache and
nausea. Ten days later she became progressively obtunded
but her mental state improved markedly after she received
intravenous fluids and betamethazone. At that time laboratory data showed a serum sodium value of 110 mEq/liter,
potassium 4.5 mEq/liter, and chloride 75 mEq/liter. The
electrolyte abnormalities were rapidly corrected within two
days by an intravenous infusion of 35% sodium chloride,
whereupon her condition deteriorated again and she was
transferred to our hospital in coma.
O n admission, she had decorticate posturing. She did not
respond to verbal commands but withdrew her limbs in response to pinpricks. Pupils, doll’s eye reflex, and caloric
reflexes were normal. Her deep tendon reflexes were brisk
and plantar responses were extensor. An electroencephalogram showed moderate nonspecific generalized slowing.
Testing of brainstem auditory evoked potentials as well as
head computed tomographic (CT) scan and cerebral angiogram revealed no abnormalities.
Oral Audrocortisone acetate and L-thyroxine sodium therapy was started. Six weeks after admission she could follow
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the examiner’s finger with her eyes. At eight weeks she was
able to blink appropriately to “yes” and “no” questioning but
was tetraplegic and mute. CT scan demonstrated a hypodense nonenhancing lesion involving the central portion
of the pons and bilateral low-density lesions of the putamen.
Magnetic resonance imaging using inversion-recovery carried
out fifteen weeks after admission showed a low-density signal
area in the basis pontis that remained unchanged at thirty
weeks (Figure). The low-density signal area was anatomically
consistent with central pontine myelinolysis. Her condition
improved gradually, but eight months following admission
she remained dysarthric and quadriparetic.
There appear to be two clinical patterns in central pontine
myelinolysis. The first includes patients whose condition
worsens after rapid correction of the electrolyte imbalance
and who become immediately “locked-in” { 1, 31, while in the
second pattern the “locked-in’’ state is preceded by coma {Z,
41.The early clinical diagnosis in the latter group is more
difficult; CT scan often shows both pontine and extrapontine
lesions [ 5 ] and prognosis for recovery is poorer.
Department of Neurology
Japan Red Cross Medical Center
4-1-22 Hiroo Shibuya
Department of Internal Medicine
Toshiba Central Hospital
6-1-22 Higashiooi Shinagawa
Tokyo, Japan
Sagittal inversion-recovery magnetic resonance images at fifteen
and thirty wee& after onset of symptoms. showing a low-density
signal area in the pons (ldt).The lesion is visible afer seven
months (right).
1. Gerber 0,Geller M, Stiller T, et al: Central pontine myelinolysis:
resolution shown by computed tomography. Arch Neurol
40:116-117, 1983
2. Hazratji SMA, Kim RC, Marasigan AV: Evolution of pontine
and extrapontine myelinolysis. J Comput Assist Tomogr 7:356361, 1983
3. Telfer RD, Miller EM: Central pontine myelinolysis following
hyponatremia, demonstated by computerized tomography. Ann
Neurol 6455-456, 1979
4. Thompson DS, Hutton JT, Stears JC, et al: Computerized tomography in the diagnosis of central and extrapontine myelinolysis. Arch Neurol 38:243-246, 1981
5. Wright DG, Lauren0 R, Victor M: Pontine and extrapontine
myelinolysis. Brain 102:361-387, 1979
Adrenoleukomyeloneuropathy with Onset
in Early Childhood
Neal L. Rosen, MD, Richard Lechtenberg, MD,
Krystyna Wisniewski, MD, Raju Pullarkat, MD,
and Harvey S. Bennett, M D
Adrenoleukodystrophy (ALD) and adrenomyeloneuropathy
(AMN) are degenerative diseases of central nervous system
white matter and adrenal tissue [S, 91. ALD is transmitted as
a sex-linked recessive trait and usually causes progressive
dementia, quadriparesis, and blindness starting in childhood
and leading to death by the second decade of life [lo]. AMN
is characterized by adult-onset hypogonadism, spastic quadriparesis, dementia, and polyneuropathy, with adrenal failure
developing in affected men [2}. Both ALD and AMN are
believed to be expressions of a metabolic defect that allows
esters of very-long-chain saturated fatty acids to accumulate
[ 4 , 101. Characteristic trilaminar cytoplasmic inclusions have
been found in neuronal, adrenal, and other tissues in both
disorders [lo]. Cerebellar signs and pathology have been
described in a variant of these two diseases called adrenoleukomyeloneuropathy (ALMN) [ 3 , 4 , 61. W e encountered
an unusual case of ALMN with onset in early childhood,
histochemical evidence of lipodystrophy, and computed
tornographic (CT) signs of cerebellar disease.
The patient first exhibited developmental delays at 3 years
of age. At 3.5 years, minor motor seizures appeared and
were treated ineffectively with phenobarbital. A year later he
had poor finger coordination and mild dysarthria. At 5 years
he exhibited poor acquisition of language. Despite trials of
phenytoin and carbamazepine, he continued to have seizures
at least monthly. His electroencephalogram (EEG) showed
diffuse slowing and multifocal spikes. He had poor handwriting, ataxic gait, poor finger coordination, and impulsive behavior, all of which worsened as he approached his ninth
birthday. At 9 years of age the patient’s reading and spelling
were at a second grade level. His speech was slow and scanning. Pes cavus was prominent bilaterally. Ankle flexors
had 415 strength. His gait was broad-based and unsteady
Computed tomographic scan of the brain at the level of the fourth
ventricle. Subcortical structures in the cerebellar hemispheres K X hibit markedly decreased density.
with bilateral circumduction. Marked dysmetria and dysdiadochokinesia were apparent in his arm and leg movements. Tendon reflexes were 4 + , with ankle clonus and
bilateral extensor plantar responses.
His maternal half brother had progressive gait ataxia, dysarthria, and dementia and died of unknown cause at age 23.
His maternal half sister was alive and well at age 13. Two
maternal uncles had slow speech, and one exhibited seizures
and gait difficulty. His maternal grandfather had epilepsy.
Findings on routine blood count, differential, electrolytes,
liver function tests, and urinalysis were normal. Cerebrospinal fluid protein, glucose, and IgG levels and cell count were
all normal. Thyroid hormone, prolactin, follicle-stimulating
hormone, and luteinizing hormone levels were all normal.
The cortisol level at 10 AM was 9.8, but the adrenocorticotropic hormone level was elevated to 290 pg per milliliter.
Serum cortisol was 10.8 pg per deciliter before stimulation
with cortrogen (0.25 mg) and at 60 minutes after stimulation.
His electrocardiogram was normal. The EEG was diffusely
slow with focal paroxysmal slow-wave activity. Nerve conduction velocities were slowed, and muscle action potentials
were of low amplitude. A monopolar needle electromyogram revealed no acute denervation. C T scanning of the
brain revealed extensive areas of decreased density that were
largely limited to the subcortical areas of the paravermal
cerebellum (Figure).
Leukocyte lysosomal enzyme studies showed normal
levels of arylsulfatase, hexosaminidase, and plasma phytanic
acid. Results of urine and plasma aminoacid chromatography
were normal. However, plasma sphingomyelin analyzed by
Notes and Letters
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central, diagnosed, pontine, myelinolysis, magnetic, imagine, resonance
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