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Clusters of tonic spasms as an initial manifestation of multiple sclerosis.

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3. Kennedy DH, Bone I, Weir AI: Early diagnosis of Legion4.
5.
6.
7.
8.
7.
naires' disease: distinctive neurological findings. Lancet
1:740-741, 1781
Lees AW, Tyrrell WF: Severe cerebral disturbance of Legionnaires' disease. Lancet 2:1336, 1778
Maskill MR, Jordan EC: Pronounced cerebellar features in
Legionnaires' disease. Br Med J 283:276, 1781
Morgan DJ, G a w k J: Severe peripheral neuropathy complicating Legionnaires' disease. Br Med J 283:1577-1578,
1781
Pearson SB, Dadds JH: Neurological complications of Legionnaires' disease. Postgrad Med J 57:107-110, 1781
Saleh F, Rodichok LD, Satya-Murtis S, Tillotson JR: Legionnaires' disease. Report of a case with unusual manifestations.
Arch Intern Med 140:1514-1516, 1780
Shetty KR, Cilyo CL, Starr BD, Harter DH: Legionnaires' disease with profound cerebellar involvement. Arch Neurol
37:379-380, 1780
Alzheimer Disease:
Plaques, Tangles, and
the Basal Forebrain
Peter J. Whitehouse, MD, P h D , A $
Robert G. Struble, P h D , " t
Arthur W. Clark, MD,"t$
and Donald L. Price, M D " t $
In reviewing our article entitled "Alzheimer Disease: Evidence for Selective Loss of Cholinergic Neurons in the
Nucleus Basalis" [7], we discovered an error which we
would like to bring to the attention of the readership.
In the Discussion of our article, but not in the Results,
we mentioned observing senile plaques in the nucleus
basalis of Meynert (nbM) in a familial case of Alzheimer
disease (AD). This is not accurate. W e did not find definite
plaques in the nbM in this patient, nor have we identified
senile plaques in the nbM in any case of nonfamilial A D
examined to date [8]. However, we confirm that
neurofibrillary tangles are present and often abundant in
the nbM in AD, a finding which is reported by others [2,31.
Neurons in the nbM may contain a great deal of lipofuscin
and may show vacuolar abnormalities; however, as reported in our original control case, these changes can also
be seen in age-matched controls, making the association of
these processes with A D less clear. In addition, we have
confirmed our finding in the original A D case [ I ] that the
loss of large neurons extends over the entire basal forebrain including the septum, the nucleus of the diagonal band
of Broca, as well as the midportion of the nbM shown in
Figure 1 of our earlier article [7].
We believe that understanding the distribution of
neurofibrillary tangles and senile plaques in A D may contribute to understanding of the pathophysiology of this
condition. We have recently discovered that immature
plaques, containing swollen neurites but no amyloid, are
rich in acetylcholinesterase (AChE); as plaques evolve, increasing amounts of amyloid appear to be deposited, while
the number of neurites and the AChE activity are markedly
reduced [4,
51. Since the main source of cholinergic inputs,
rich in AChE, is derived from the nbM, these observations
suggest that some individual plaques initially show
cholinergic innervation but that end-stage plaques are virtually devoid of cholinergic inputs. O n e can readily imagine
how this process, repeated over and over in the cortex,
could result in a cortex with markedly reduced cholinergic
presynaptic markem [6].
"Neuropathology Laboratory and Departments of
+Pathology and $Neurology
TheJohns Hopkins [Jniversity School of Medicine
Baltimore. M D 2 1205
References
1. Clark AW, Parhad IM, Struble RG, Whitehouse PJ, Price DL:
The nucleus basalis of Meynert (nbM) in Alzheimer's disease/
senile dementia of the Alzheimer's type (AD/SDAT). Presented at the IXth International Congress o n Neuropathology,
Vienna, Austria, Sept 5-10, 1982
2. Hirano A, Zimnierman HM: Alzheimer's neurofibrillary
changes: a topographic study. Arch Neurol 7:227-242, 1962
3. Ishii T Distribution of Alzheimer's neurofibrillary changes in
the brain stem and hypothalamus of senile dementia. Acta
Neuropathol (Berl) 6:181-187, 1766
4. Price DL, Whitehouse PJ, Struble RG, et al: Alzheimer's disease and Down's syndrome. Ann NY Acad Sci (in press)
5. Struble RG, Cork LC, Whitehouse PJ, Price DL: Cholinergic
innervation in neuritic plaques. Science 2 16:413-415, 1782
6. Terry RD, Davies P: Dementia of the Alzheimer type. Annu
Rev Neurosci 3:7/'-95, 1780
7. Whitehouse P.J, Price DL, Clark AW, Coyle JT, DeLong MR:
Alzheimer disease: evidence for selective loss of cholinergic
neurons in the nucleus basalis. Ann Neurol 10:122-126, 1781
8. Whitehouse PJ, Price DL, Struble RG, Clark AW, Coyle JT,
DeLong MR: Alzheimer's disease and senile dementia: loss of
neurons in the basal forebrain. Science 215:1237-1239, 1782
Clusters of Tonic Spasms
as an Initial Manifestation
of Multiple Sclerosis
Peter D. Heath, M:Sc, MRCP,
and Simon Nightingale, BSc, MRCP
Tonic spasms, the most commonly documented paroxysmal feature in multiple sclerosis, have been reported as the
initial manifestation in only seven patients, always as a
unilateral phenomenon [7]. W e cared for a 33-year-old man
who, at age 28 and against a background of good health,
developed intense unilateral body spasms and Lhermitte's
phenomenon. Spasms were induced by spinal flexion,
especially following sudden exertion, sexual intercourse,
ingestion of alcohol, o r moving into a cold environment.
Spinal flexion was followed, after a latency of about a minute, by a cramping sensation in the groin that intensified
and spread to involve the trunk, limbs, and face unilaterally. His arm became clumsy and assumed a "tetanic" posture [4],his leg became stiff, and his face felt cold but was
not twisted. Spasms lasted several minutes and were followed by a burning sensation in the contralateral leg.
494 Annals of Neurology Vol 12 No 5 November 1982
The initial cluster of 12 left-sided spasms in six months
was followed by 10 similar clusters, with variable remission intervals, over five years. Within a c l u t e r , spasms
were unilateral and stereotyped, though the side and extent
of the body involved varied from cluster to cluster. Clusters were terminated within 24 hours o n two separate occasions by therapy with carbamazepine, 100 mg every 8
hours.
At age 33 the patient developed a constant sensation of
tightness and tugging in his right groin, which persisted for
several months despite carbamazepine treatment. There
was no family history of a similar disorder. Neurological
examination was normal throughout the five years. Cerebrospinal fluid contained 40 lymphocytes per microliter
and a total protein of 0.47 mg per deciliter including 20%
gamma globulin fraction. Auditory, visual, and somatosensory evoked potentials, electroencephalogram, computed
tomographic scan, cervical spine roentgenograms, and
routine blood chemistry determinations were all normal.
Tonic spasms occur in multiple sclerosis, cerebral palsy,
and cervical cord trauma as well as in familial and idiopathic
cases [3, 61. With tonic spasms, Lhermitte's phenomenon,
nonparoxysmal sensory symptoms, and the cerebrospinal
fluid abnormalities, the most likely diagnosis in our patient
is multiple sclerosis. He is remarkable in having such
numerous clusters, which affected different sides of the
body and continued for five years before nonparoxysmal
features developed. These findings are reminiscent of the
familiar spatial and temporal pattern of nonparoxysmal
events in multiple sclerosis.
The mechanism for these attacks is postulated to result
from demyelination insufficient to produce a persistent
deficit but which renders axons hypersensitive to minor
stresses [2]. Precipitants include posture [4],movement
[ 5 ] , sensory stimulation of a trigger zone, or, as in our patient, flexion of the spine. A spinal cord origin for the tonic
spasms is suggested by the resemblance of the disorder to a
"Brown-Stquard lesion in reverse" [l].
Department of Neurology
Royal Victoria Infirmary
Newcastle- Upon-Tyne, England
References
1. Ekbom KA, Westerberg CE, Osterman PO: Focal sensorymotor seizures of spinal origin. Lancet 1:67, 1968
2. Espir MLE, Millac P: Treatment of paroxysmal disorders in
multiple sclerosis with carbamazepine. J Neurol Neurosurg
Psychiatry 33:528-53 1, 1970
3. Lance JW: Familial paroxysmal dystonic choreoathetosis and its
differentiation from related syndromes. Ann Neurol 2:285293, 1977
4. Matthews WB: Tonic seizures in disseminated sclerosis. Brain
81:193-206, 1958
5 . Matthews WB: Paroxysmal symptoms in multiple sclerosis. J
Neurol Neurosurg Psychiatry 38:617-623, 1975
6. Nathanson M: Paroxysmal phenomena resembling seizures,
related to spinal cord and root pathology. J Mt Sinai Hosp N Y
29:145-151, 1962
7. Twomey JA, Espir MLE: Paroxysmal symptoms as the first
manifestation of multiple sclerosis. J Neurol Neurosurg
Psychiatry 43:296-304, 1980
Bechterew's Phenomenon
in a Human Patient
David S. Zee, MD,"? Thomas J. Preziosi, MD,"
and Leonard R. Proctor, MD$
Bechterew [I] reported experiments in which the
labyrinths of an animal were successively destroyed at
intervals u p to several days. After the second lesion, he
observed a reversal of the direction of the compulsive
movements (eye and head nystagmus, body turning) if the
interval between the two operations was sufficiently long.
This phenomenon, called Bechterew compensation or
Bechterew nystagmus, reflects the action of the central
mechanism by which the brain normally compensates for
vestibular imbalance. So, for exampie, when labyrinthine
function is acutely lost on one side, the asymmetry between
the peripheral vestibular inputs is compensated centrally,
probably by restoration of the level of spontaneous activity
in the vestibular nuclei on the side of the hypofunctioning
labyrinth [2]. Then, when the remaining labyrinth is destroyed, the vestibular nuclei again become imbalanced,
creating spontaneous nystagmus even though now there is
no input to the vestibular nuclei from either labyrinth.
Though well documented in experimental animals, this sequence of events rarely occurs in human beings. W e have
examined a patient, however, who lost vestibular function
first on one side and then, separated by an interval of several years, the other. He showed the classic Bechterew
phenomenon.
The patient was in good health until early 1977, when, at
the age of 54 years, he began having episodes of tinnitus in
the left ear with disequilibrium, each lasting only a few seconds. O n the day of admission in July, 1777, he had a transient episode of tinnitus and decreased hearing in the left
ear with disequilibrium, nausea, and vomiting lasting several minutes. Later in the day he developed the same
symptoms, but this time they did not subside spontaneously and he was hospitalized. Four days later he had formal vestibular testing, which showed a spontaneous nystagmus, slow phases directed to the left, that increased with
eye closure. Caloric testing showed no response in the left
ear but normal responses in the right ear. Audiometric
testing showed no hearing in the left ear.
Six weeks later, in August, 1777, he was admitted to T h e
Johns Hopkins Hospital for further evaluation. At that
time he showed no spontaneous nystagmus during fixation,
even behind Frenzel glasses. Neurological examination
showed a slight right facial paresis and subjective impairment of pinprick and temperature sensation on the left side
of the face. Gait was mildly impaired, with difficulty on
making turns. There was questionable heel to shin dysmetria in the left lower extremity. Computerized tomography was normal and cerebral angiography showed no
abnormalities in the posterior (or anterior) circulation.
Roentgenograms of the spine showed cervical spondylosis.
The patient was discharged feeling well.
In October, 1977, he had repeat vestibular function tests
that again showed no response to Hallpike or ice-water
caloric stimulation on the left side. With eye closure only,
Notes and Letters
495
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