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DysarthriaЧclumsy hand syndrome or homolateral ataxia and crural paresis.

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Results of Intelligence Tats in Patients with Wilson’s Disease
Test #1
Test #2
WAIS/WISC
WAIS-R
Pt
Age
Neuro
VIQ
PIQ
FIQ
Age
Neuro
VIQ
PIQ
FIQ
FIQX
1
2
19
35
12
17
20
15
2
1
2
1
2
1
0
97
109
94
102
92
116
110
109
104
89
79
81
108
122
102
107
91
92
86
114
117
43
58
33
49
45
1
0
91
107
88
96
93
122
100
107
85
83
84
109
96
95
107
85
90
88
119
97
102
114
92
97
95
126
104
3
4
5
6
7
Neuro
8
=
IQ, FIQ
37
32
0
0
1
0
0
99
neurological ranng of severity of symptoms 0 = none, 1 = mild, 2 = moderate, 3 = severe, VJQ
full scale IQ, FIQ* = WAIS-R FIQ adjusted by 7 points to be comparable with WAIS FIQ
=
verbal IQ, PIQ = performance
=
points were added to the WAIS-R score to achieve comparability with the WAIS and WISC scores.
None of the t-tests comparing the first and second results
of the Performance IQ, Verbal IQ, and Full Scale IQ was
significant ( p > .1 for all three tests), indicating no significant
change in scores over time. All patients scored at least in the
normal range at both assessments. The social and occupational histories of these patients also suggest that major
change in intellectual functioning did not occur. The severity
of neurological deficits improved in all 6 neurologically affected patients; 4 became asymptomatic.
All patients whose WD is not treated unavoidably suffer
progression both of their neurological and hepatic symptomatology, which is generally halted and often improved by
penicillamine treatment. The effect of long-term penicillamine treatment on intellectual functioning has never before
been studied. Our results provide no evidence that this drug
can improve IQ. However, since none of the patients studied
had an intellectual deficit at the time of the initial assessment,
this sample did not afford an opportunity to resolve this issue.
The results do suggest that intellectual functioning does not
worsen during penicillamine treatment. Only the patient who
had never been neurologically symptomatic showed a decline
in IQ scores, perhaps due to his periodic noncompliance.
Whether penicillamine can improve I Q in WD must await a
serial study of patients who clearly exhibit intellectual deficit
before treatment is started.
We are grateful to Joseph Richman, PhD, for permission to
use his clinical data.
Department of Psychiatry
Division (?f Genetic Medicine, Department of Medicine
Albert Einstein College of Medicine
Neu: York. N Y
References
1. Medalia A, Isaacs-Glaberman K, Scheinberg IH. Keuropsychological impairment in Wilson’s disease. Arch Neurol 1988;45:
502-504
2. Isaacs-Glaberman K, Medalia A, Scheinberg IH. Verbal recall
and recognition abilities in patients with Wilson’s disease. Cortex
1989;25:353-361
574 Annals of Neurology Vol 29 No 5
May 1991
3. Deiss A, Lynch RE, Lee GR, Cartwright GE. Long-term therapy
of Wilson’s disease. Ann Intern Med 1971;75:57-65
4. Goldstein NP, Ewert JC, Randall RV, et al. Psychiatric aspects
of Wilson’s disease (hepatolenticular degeneration): results of psychometric tests during long-term therapy. Am J Psychiatry 1968;
124~1555-1561
5. Wechsler D. Wechsler Adult Intelligence Scale-Revised Manual.
New York: Psychological Corporation, 1981
Dysarthria-Clumsy Hand
Syndrome or Homolateral
Ataxia and Crural Paresis?
Yves De Smet, MD
Glass and associates El] have suggested that dysarthriaclumsy hand syndrome (DCHS) and homolateral ataxia and
crural paresis (HACP) are two distinct clinical (and perhaps
pathological) syndromes. We recently reported the first case
(to our knowledge) of ataxic hemiparesis secondary to a cerebellar lesion {2]. The computed tomography scan showed a
left rostra1paravermian lacune in the vicinity of the peduncle.
The patient had all the clinical features of both DCHS and
HACP, according to Glass and colleagues { 11:moderate right
crural hemiparesis with Babinski sign; no aphasia, no face
weakness, and no tongue deviation but severe dysarthria and
severe dysphagia; right hemiataxia with clumsy hand and gait
ataxia; lack of sensory symptoms.
To us, ataxic hemiparesis results from a lesion involving
simultaneously both the cortico-spinal and cortico-pontocerebello-dentato-rubro-thalamo-cortical
(CPCDRTC)tracts,
anywhere between the subcortical and inferior pontine levels.
Only pure motor hemiparesis occurs beyond this parallel
common course (medullary pyramids). Above it, pure motor
hemiparesis occurs after a corticospinal lesion and “cerebellar” hemiataxia after a CPCDRTC one [3}. In the absence of
a corticospinal lesion, ischemia, edema, or mass effect explains the pyramidal signs [4]. In contrast to Landau [5}, we
{2) thus asserted the existence of ataxic hemiparesis. Moreover, and contrary to Glass and associates { l], we also regard
DCHS and HACP as two clinical variants of one and the
same syndrome: ataxic hemiparesis.
H6pital Rigional du Nord
Clinique Saint-Louis
Ettelbruck, Luxembourg
References
1. Glass JD, Levey AI, Rothstein JD. The dysarthria-clumsy hand
syndrome: a distinct clinical entity related to pontine infarction.
Ann Neurol 1990;27:487-494
2. De Smet Y , Gengler L, Muller R. HCmiparCsie ataxique par lacune cerebelleuse. Acta Neurol Beig 1989;89:61-66
3. Montgomery EB. Signs and symptoms from a cerebral lesion that
suggest cerebellar dysfunction. Arch Neurol 1983;40:422-423
4. Bogousslavsky J, Regli F, Ghika J, Feldmeyer JJ. Painful ataxic
hemiparesis. Arch Neurol 1984;41:892-893
5. Landau WM. Clinical neuromythology 111. Ataxic hemiparesis:
special deluxe stroke or standard brand? Neurology 1988;38:
1799- 1801
References
1. Chin JH. Adenosine receptors in brain: neuromodulation and
role in epilepsy. Ann Neurol 1989;26:695-698
2. Dragunow M, Goddard GV, Laverty R. Is adenosine an endogenous anticonvulsant? Epilepsia 1985;26(5):480-487
3. Winn HR, Welsh JE, Bryncr C , et’al. Brain adenosine production
during the initial 60 seconds of bicuculline seizures in rats. Acta
Neurol Scand 1979;60:536-537
4. Dragunow M, Robertson HA. 8-Cyclopentyl 1,3-dimethyIxanthine prolongs epileptic seizures in rats. Brain Research
198 7 ;417:377-3 79
3. Peters SG, Wochos DN, Peterson GC. Status epilepticus as a
complication of concurrent electroconvulsive and theophylline
therapy. Mayo Clin Proc 1984;59:568-570
6. Shapira B, Lerer B, Gilboa D, et al. Facilitation of ECT by caffeine
pretreacment. Am J Psychiatry 1987;144:9, 1199-1202
7. Dragunow 1M. Purinergic mechanisms in epilepsy. Prog Neurob i d 1988;31 :85-108
8. Eldridge FL, Paydarfar D, Scott SC, Dowell RT. Role of endogenous adenosine in recurrent generalized seizures. Exp Neurol
1989;103:179-185
Reply
Jerome H . Chin, MD, PhZ)
Adenosine and
Seizure Termination
M. Dragunow
I read with interest D r Chin’s article “Adenosine Receptors
in Brain: Neuromodulation and Role in Epilepsy” {l). However, I was disappointed that D r Chin did not mention perhaps the most important role for adenosine in epilepsy: its
role as a natural anticonvulsant to block seizure spread and
to terminate seizures C2). A growing body of data shows
convincingly that adenosine is released during seizure activity
[3] and that by activating theophylline-sensitive adenosine
A1 receptors it terminates seizures in animals [2, 4).In the
presence of a subconvulsive dose of the adenosine antagonists caffeine, theophylline, or 8-cyclopentyl-theophylline,
partial seizures that would normally last only a few seconds
are converted into fully generalized seizures that can last
many minutes [2,4].Caffeine and theophylline also prolong
the duration of seizures in humans [ 5 , 61. Conversely, adenosine agonists convert generalized seizures into partial seizures
[2, 71. These results strongly support our hypothesis that
endogenous adenosine inhibits seizure spread and terminates
seizure discharges in the brain {7]. Loss of this adenosine
anticonvulsant system leads to starus epilepticus in animals
and humans [ 5 , 7, 8), although it is not known if status epilepticus occurring spontaneously in humans also involves
adenosine loss.
Thus, adenosine is a powerful endogenous anticonvulsant
substance in the nervous system that terminates seizures arid
will no doubt become an important target for anticonvulsant
drug development.
Department of Pharmacology and Clinical Pharmacoloa
University of Auckland School of Medicine
Auckland, New Zealand
I appreciate Dr Dragunow’s interest in my article and in the
important role of adenosine as an endogenous neuromodulacor. D r Dragunow provides additional citations, including
some of his own, to support one of the central themes of my
review, namely, that adenosine may function as an endogenous anticonvulsant to inhibit seizure initiation and propagation. It is hoped that future research will substantiate this
proposal and result in pharmacological manipulation of adenosine systems for the treatment of epilepsy.
Department of Neurology
University of California
San Francisco. C A
A
1
Lar bamazepine
and the Heart
iMario Puletti, MD,” Cesare Iani, MD,t
Mario Curione, MD,” Massimo Trappolini, MD,*
and Mario Manfredi, MDP
Reports of sinoatrial or atrioventricular conduction disorders
in patients treated with carbamazepine have recently appeared {l-41. Given the widespread use of this drug, we
undertook systematic study of its cardiac effects. Two protocols were planned: the present one, which uses routine cardiological examination, and a second protocol now in development that requires Holter monitoring before and during
carbamazepine treatment. We report here on the results of
the first protocol.
Ninety-two patients with epilepsy (36 males and 56 females) were studied. Average age was 36.5 years (range 7 to
77 yr; 33 patients were over 40 yr). None had signs of cardiac
Annals of Neurology
Vol 29 No 5
May 1991 575
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ataxia, hands, syndrome, crural, paresis, dysarthriaчclumsy, homolateral
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