Abstracts from Dr. Paulo Cesar Trevisol-Bittencourt’s Articles Available at MEDLINE

Abstracts from Dr. Paulo Cesar Trevisol-Bittencourt’s Articles Available at MEDLINE


1: Acta Neurol Scand. 2003 Dec;108(6):424-7.

Erratum in:
Acta Neurol Scand. 2004 Jan;109(1):84.

Interictal serum S100B levels in chronic neurocysticercosis and idiopathic
epilepsy.

Portela LV, Tort AB, Walz R, Bianchin M, Trevisol-Bittencourt PC, Wille PR,
Cardoso RC, Ishida MM, vonWangenheim A, Grisard EC, Steindel M, Goncalves CA,
Souza DO.

Departamento de Bioquimica, ICBS, Universidade Federal do Rio Grande do Sul,
Porto Alegre, RS, Brazil. roska@ufrgs.br

OBJECTIVE: To assess whether serum S100B levels could reflect a glial response
in patients with epilepsy secondary to neurocysticercosis (NCC) and with
idiopathic epilepsy. SUBJECTS AND METHODS: Serum S100B levels were measured
using an immunoluminometric assay in 20 patients with focal epilepsy related to
chronic NCC (NCC group), and 19 patients with focal epilepsy (EPI group),
matched by epidemiological and clinical data. Epileptic patients were compared
with 20 healthy controls (CON group) matched by age and sex. RESULTS: No
difference was observed in S100B levels among NCC, EPI and CON groups (P>0.39).
Serum S100B levels were not affected by antiepileptic drugs, frequency and type
of seizures. Preliminarily, significantly higher levels of S100B were observed
in patients with bilateral electroencephalographic (EEG) findings than in
patients with unilateral and normal EEG findings (P<0.05). CONCLUSION: Serum
S100B is normal in patients with focal epilepsy related or not to chronic NCC.

PMID: 14616295 [PubMed – indexed for MEDLINE]

 

2: Epilepsy Behav. 2001 Dec;2(6):558-562.

Cognitive Performance of Patients with Epilepsy and Calcified Neurocysticercotic
Lesions: A Case-Control Study.

Boppre MC, Wille PR, Guarnieri R, Rezek K, Carqueja C, Trevisol-Bittencourt PC,
Walz JC, Bianchin M, von Wangenheim A, Chaves ML, Walz R.

Servico de Neurologia, Hospital Regional de Sao Jose, Sao Jose, SC, Brazil

Neuropsychological tests were applied to 20 patients with focal epilepsy related
to calcified neurocysticercosis (NCC) (mean: three lesions/patient; NCC group),
22 patients with focal epilepsy without NCC (EPI group), and 29 healthy controls
matched for age, sex, and educational level. The EPI and NCC groups were matched
for age at onset of epilepsy, epilepsy duration, frequency of attacks, seizure
semiology, interictal EEG findings, and antiepileptic drugs used. There were no
differences in the digit span, word span, calculus, and Mini-Mental State
examination among the three groups studied. The NCC and EPI groups showed lower
scores than controls in immediate and delayed verbal memory, famous faces test,
spatial recognition span, abstractions and judgment, and visuoconstructional
abilities. The EPI group, but not the NCC group, also had lower scores in a
praxis tests. There were no differences between the NCC and EPI groups in any of
the tests applied (P > 15), except for the spatial recognition span, which was
lower in the former. Cognitive impairment is a prevalent neuropsychological
feature of patients with epilepsy and NCC.

PMID: 12609389 [PubMed – as supplied by publisher]

 

3: Eur J Hum Genet. 2002 Nov;10(11):773-81.

Mutational spectrum of the CHAC gene in patients with chorea-acanthocytosis.

Dobson-Stone C, Danek A, Rampoldi L, Hardie RJ, Chalmers RM, Wood NW, Bohlega S,
Dotti MT, Federico A, Shizuka M, Tanaka M, Watanabe M, Ikeda Y, Brin M, Goldfarb
LG, Karp BI, Mohiddin S, Fananapazir L, Storch A, Fryer AE, Maddison P, Sibon I,
Trevisol-Bittencourt PC, Singer C, Caballero IR, Aasly JO, Schmierer K, Dengler
R, Hiersemenzel LP, Zeviani M, Meiner V, Lossos A, Johnson S, Mercado FC,
Sorrentino G, Dupre N, Rouleau GA, Volkmann J, Arpa J, Lees A, Geraud G,
Chouinard S, Nemeth A, Monaco AP.

The Wellcome Trust Centre For Human Genetics, University of Oxford, UK.

Chorea-acanthocytosis (ChAc) is an autosomal recessive neurological disorder
whose characteristic features include hyperkinetic movements and abnormal red
blood cell morphology. Mutations in the CHAC gene on 9q21 were recently found to
cause chorea-acanthocytosis. CHAC encodes a large, novel protein with a yeast
homologue implicated in protein sorting. In this study, all 73 exons plus
flanking intronic sequence in CHAC were screened for mutations by denaturing
high-performance liquid chromatography in 43 probands with ChAc. We identified
57 different mutations, 54 of which have not previously been reported, in 39
probands. The novel mutations comprise 15 nonsense, 22 insertion/deletion, 15
splice-site and two missense mutations and are distributed throughout the CHAC
gene. Three mutations were found in multiple families within this or our
previous study. The preponderance of mutations that are predicted to cause
absence of gene product is consistent with the recessive inheritance of this
disease. The high proportion of splice-site mutations found is probably a
reflection of the large number of exons that comprise the CHAC gene. The CHAC
protein product, chorein, appears to have a certain tolerance to amino-acid
substitutions since only two out of nine substitutions described here appear to
be pathogenic.

PMID: 12404112 [PubMed – indexed for MEDLINE]

 

4: Arq Neuropsiquiatr. 2001 Jun;59(2-A):214-8.

[The most common conditions in a neurology specialty clinic]

[Article in Portuguese]

Trevisol-Bittencourt PC, Ferreira MA, Marasciulo AC, Collares CF.

Disciplina de Neurologia, Universidade Federal de Santa Catarina. pcb@hu.ufsc.br

OBJECTIVE: To present the most frequent diagnosis among patients referred for
neurological evaluation to estimate their labour capacities at the unit of
National Institute of Social Security (INSS), Florianopolis-SC, southern Brazil.
METHOD: Review of all medical records of 108 patients evaluated between October
97 and May 98. The sample was submitted to judicious medico-legal assessment to
define their final diagnosis. RESULTS: Neurological evaluation disclosed as the
commonest disorders, in decreasing order of frequency: epilepsy, rheumatic
diseases, psychiatric illnesses, neurological disorders related to chronic
alcoholism, head trauma syndrome and cerebrovascular diseases. CONCLUSION:
Neurological disorders may be responsible for important disability among workers
in our society. However, the potential for social rehabilitation, often
underestimated, must be considered. Moreover, diverse non-neurological
conditions used to be sent for neurological evaluation.

PMID: 11400028 [PubMed – indexed for MEDLINE]

 

5: Arq Neuropsiquiatr. 1999 Sep;57(3B):787-92.

[Causes of late-onset epilepsy in an epilepsy clinic of Santa Catarina–Southern
Brazil]

[Article in Portuguese]

Rigatti M, Trevisol-Bittencourt PC.

Universidade Federal de Santa Catarina (UFSC).

OBJECTIVE: To identify the most common etiologies of late-onset epilepsy in our
society. METHOD: Retrospective and descriptive study in the CME/SUS of
Florianopolis from 1990 to 1998, evaluating 120 handbooks of patients with
late-onset epilepsy, that is, those that had initiated epileptic seizures of the
18 years in ahead. The collected variables were: age of the first seizure,
etiologies and morbid familial history. RESULTS: The prevalence rate found for
late-onset epilepsy was 29.48%. Most of the sample had partial symptomatic
epilepsy and the most frequent and important were: cysticercosis with 20%, head
trauma with 15%, febrile convulsions in infancy with 5% and 35% were classified
as idiopatic. CONCLUSION: Prevalence of late-onset epilepsy in our society is
29.48%, value slightly upper to the told one in developed countries (25%). Very
probably, this difference is consequence of the epidemic character of
cysticercosis among us. Moreover, head trauma and febrile convulsions are common
etiologies. Prophylaxis of epilepsy is viable and urgently necessary in our
society.

PMID: 10751913 [PubMed – indexed for MEDLINE]

 

6: Arq Neuropsiquiatr. 1999 Sep;57(3B):784-6.

Phenytoin as the first option in female epileptic patients?

Trevisol-Bittencourt PC, da Silva VR, Molinari MA, Troiano AR.

University Hospital, Santa Catarina Federal University (UFSC), Brasil.
pcb@hu.ufsc.br

OBJECTIVE: Phenytoin (PHT) is one of the first-choice drugs in several epileptic
syndromes, mostly in partial epilepsies, in which case it is effective as
carbamazepine and phenobarbital. However, like any other anti-epileptic drug
(AED), unpleasant side-effects are not rare. The aim of this study is the
evaluation of dermatological troubles related to chronic PHT usage in female
patients. METHOD: Between 1990-93, 731 new patients underwent investigation for
epilepsy at the Multidisciplinary Clinic for Epilepsy in our State. In this
sample 283 were AED users at the time of the first assessment. Sixty one female
patients taking PHT were identified. They were taking PHT in a dosage ranging
from 100 to 300 mg daily, in mono or polytherapy regimen, during 1-5 previous
years. RESULTS: More than 50% of the sample showed coarse facial features made
by the combination of several degrees of acne, hirsutism and gingival
hyperplasia. CONCLUSION: Except in emergency situations, PHT should not be
prescribed as the first option to the treatment of female epileptic patients,
because not uncommonly the cosmetic side-effects are more socially handicapping
than the epileptic syndrome by itself.

PMID: 10751912 [PubMed – indexed for MEDLINE]

 

7: Arq Neuropsiquiatr. 2000 Jun;58(2B):548-55.

[Interictal personality syndrome in non-dominant temporal lobe epilepsy: case
report]

[Article in Portuguese]

Trevisol-Bittencourt PC, Troiano AR.

Departamento de Clinica Medica, Hospital Universitario, Universidade Federal de
Santa Catarina. pcb@hu.ufsc.br

The syndrome of interictal personality in non-dominant temporal lobe epilepsy
consists of hyposexuality, hyperreligiosity, humorlessness and hypergraphia. Its
notification, in 1974, was followed by an extensive search for these traits in
broad epileptic populations. Nevertheless, these statistical studies failed to
match this syndrome in general temporal lobe epileptics, and its existence
became then target of doubt. We report the case of a 35 year-old man presenting
partial complex epilepsy, whose singularity lies in his sophisticated drawing
abilities. The large amount of buildings and houses he paints expresses his
hypergraphia. He also presents hyposexuality and hyperreligiosity. MRI shows
right mesial temporal sclerosis. Temporal hyperconnection, caused by a basal
temporal irritative focus, is the most probable pathophysiological mechanism.
Epileptic fits can be controlled in the majority of cases. However, behavioural
symptoms usually do not respond to pharmacological approach or psychotherapy.

Publication Types:
Case Reports

PMID: 10920421 [PubMed – indexed for MEDLINE]

 

8: Arq Neuropsiquiatr. 1999 Jun;57(2B):489-94.

[Neuroacanthocytosis. A case report]

[Article in Portuguese]

Troiano AR, Trevisol-Bittencourt PC.

Faculdade de Medicina da Universidade Federal de Santa Catarina (UFSC), Brasil.

We present a 45 years old man with neuroacanthocytosis. This gentleman has
complex partial seizures and generalized tonic-clonic seizures, as well as
movement disorders characterized by chorea and orofacial diskinesia.
Complementary examination shows acanthocytosis of 11% on peripheral blood,
irritative focus on right temporal lobe on EEG, serum creatinokinase of 101 U/l
and volume reduction and hypersignal on caudate nucleus and putamen bilaterally
on MRI.

Publication Types:
Case Reports
Review
Review of Reported Cases

PMID: 10450359 [PubMed – indexed for MEDLINE]

 

9: Arq Neuropsiquiatr. 1999 Jun;57(2B):401-4.

[Clinical-epidemiological study of patients with juvenile myoclonic epilepsy in
Santa Catarina State, Brazil]

[Article in Portuguese]

Figueredo R, Trevisol-Bittencourt PC, Ferro JB.

Clinica Multidisciplinar de Epilepsia da Policlinica de Referencia Regional
I/SUS, Florianopolis, SC, Brasil. delfigue@zaz.com.br

We aimed to characterize the clinical profile and to establish the prevalence of
juvenile myoclonic epilepsy (JME) among 939 consecutive patients referred to our
epilepsy clinic. Inclusion criteria in the study were: a) myoclonic jerks,
preferably on awakening; b) beginning of the symptoms between 8 and 26
year-of-age; c) typical pattern of electroencephalographic discharges; and d)
good response to sodium valproate (VPA). In a retrospective design, 26 cases of
JME were identified (prevalence 2.8%). Most of these patients were female
(73.1%). Mean age at onset of symptoms was 13 (range 7-18). Tonic-clonic
seizures were reported in 92.3% and absence seizures in 19.2%. Besides, 92.3% of
the patients had typical EEG epileptiform activity and 76.9% had good response
to VPA with daily dose ranging from 500 mg to 1500 mg.

PMID: 10450346 [PubMed – indexed for MEDLINE]

 

10: Arq Neuropsiquiatr. 1998 Mar;56(1):53-8.

[Prevalence of neurocysticercosis among epileptic in-patients in the west of
Santa Catarina–southern Brazil]

[Article in Portuguese]

Trevisol-Bittencourt PC, da Silva NC, Figueredo R.

Disciplina de Neurologia, Hospital Universitario UFSC, Brasil.
pcb.latino@intergate.com.br

Neurocysticercosis (NC) is an endemic condition in several areas of Brazil. It
is most likely the major responsible for the high prevalence of epilepsy in our
country, estimated in 1-2% of general population. We performed a study to
evaluate NC as aetiology of epilepsy in the west of Santa Catarina. This state
belong to southern Brazil and it has a very developed economy. However, due the
widespread swine farming in the west district, many of them without any sanitary
control, the national health authorities have considered all this area at risk
to NC. The study was carried out in Chapeco, the main town in that region, where
CT Scan service was started in 1995. All patients put on hospital care due
epileptic seizures in 1995-96 were considered. Febrile convulsions were excluded
of the sample. We found a very expressive prevalence rate of NC among patients
suffering from epilepsy. Roughly 24% of these patients, showed unequivocal
tomography evidences for the diagnosis of NC. Our data suggest cysticercosis as
a real endemic trouble in the area and, overdosis of information in proper
language, diffuse to the whole population, seems to be the only remedy to fight
against it. Moreover, we should pay special attention to everyone related to
swine farming, does not matter how this activity has been classified.

PMID: 9686120 [PubMed – indexed for MEDLINE]

 

11: Arq Neuropsiquiatr. 1997 Sep;55(3B):519-29.

Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2,
MJD/SCA3 and DRPLA) in a large group of Brazilian patients.

Lopes-Cendes I, Teive HG, Calcagnotto ME, Da Costa JC, Cardoso F, Viana E,
Maciel JA, Radvany J, Arruda WO, Trevisol-Bittencourt PC, Rosa Neto P, Silveira
I, Steiner CE, Pinto Junior W, Santos AS, Correa Neto Y, Werneck LC, Araujo AQ,
Carakushansky G, Mello LR, Jardim LB, Rouleau GA.

Centre for Research in Neuroscience, McGill University, Montreal, QC, Canada.
bke6@musicb.mcgill.ca

Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and
Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three
distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by
expansions of an unstable CAG repeat localized in the coding region of the
causative genes. Another related disease, dentatorubropallidoluysian atrophy
(DRPLA) is also caused by an unstable triplet repeat and can present as SCA in
late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3
and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90
unrelated families with various patterns of inheritance and originating in
different geographic regions of Brazil. We found mutations in 35 families (39%),
32 of them with a clear autosomal dominant inheritance. The frequency of the
SCA1 mutation was 3% of all patients; and 6% in the dominantly inherited SCAs.
We identified the SCA2 mutation in 6% of all families and in 9% of the families
with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30%
of all patients; and in the 44% of the dominantly inherited cases. We found no
DRPLA mutation. In addition, we observed variability in the frequency of the
different mutations according to geographic origin of the patients, which is
probably related to the distinct colonization of different parts of Brazil.
These results suggest that SCA may be occasionally caused by the SCA1 and SCA2
mutations in the Brazilian population, and that the MJD/SCA3 mutation is the
most common cause of dominantly inherited SCA in Brazil.

PMID: 9629399 [PubMed – indexed for MEDLINE]

 

12: Arq Neuropsiquiatr. 1992 Sep;50(3):369-74.

[Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report
of a case and review of the literature]

[Article in Portuguese]

Trevisol-Bittencourt PC.

Departamento de Clinica Medica, Universidade Federal de Santa Catarina (UFSC),
Brasil.

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome
by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent
clinical experience has corroborated and enlarged their original observations.
PSP has become familiar as a chronic progressive disorder with extrapyramidal
rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a
characteristic supranuclear ophthalmoplegia. It is a significant cause of
parkinsonism and its etiology remains obscure. The case of a patient from Santa
Catarina who presented definite clinical evidences of this syndrome is reported.
This is the first description in this southern Brazilian State, where at least
50 more patients should exist, if we may extrapolate the prevalence rate of this
condition in developed countries to this well developed area of Brazil. A review
of the literature was undertaken with emphasis on recent clinical and
therapeutic aspects of PSP.

Publication Types:
Case Reports

PMID: 1308417 [PubMed – indexed for MEDLINE]

 

13: Arq Neuropsiquiatr. 1991 Jun;49(2):208-10.

Ketoconazole in the treatment of cryptococcosis of the central nervous system.

Trevisol-Bittencourt PC, Sander JW, Silvado CS, Wittig EO.

Departamento de Clinica Medica, Hospital de Clinicas, Universidade Federal do
Parana, Curitiba, Brasil.

Two patients with cryptococcosis of the CNS were treated with ketoconazole
(KTZ), an imidazole derivative with fungistatic properties: they had either
failed standard therapy (Amphotericin-B + 5-Fluorocytosine) or suffered
intolerable side-effects to it. Both patients were administered KTZ 800 mg/day
as monotherapy for six months without interruption and both responded. One month
after KTZ therapy was withdrawn, however, a relapse of the infection was seen in
one case. Side-effects were minimal during the trial of treatment. KTZ could be
a useful drug in some cases of neurocryptococcosis.

Publication Types:
Case Reports

PMID: 1810241 [PubMed – indexed for MEDLINE]

 

14: Arq Neuropsiquiatr. 1991 Jun;49(2):172-9.

[Machado-Joseph disease: description of 5 members of a family]

[Article in Portuguese]

Teive HA, Arruda WO, Trevisol-Bittencourt PC.

Unidade de Ciencias Neurologicas, Curitiba, Brasil.

The authors report the clinical and laboratorial findings of 5 affected members
(all males) of a family with Machado-Joseph disease. The mode a inheritance was
autosomal dominant. The mean onset age was 38 years (range 30-50 years). The
clinical picture was pleomorphic and included cerebellar ataxia, external
ophthalmoplegia with bulging eyes, extrapyramidal/pyramidal syndromes,
amyotrophy with fasciculations and peripheral neuropathy, in variable degrees of
severity. In one patient parkinsonian rigidity was greatly improved with the use
of trihexaphenidyl and L-dopa. CT scan examinations disclosed a variable degree
of cerebellar atrophy, with mild cerebral atrophy in one patient. Brainstem
evoked potentials were normal in two patients. EMG showed denervation in three
patients. Muscle biopsy (gastrocnemium) with histochemical studies revealed
chronic muscle denervation in four cases. Sural nerve biopsy with conventional
pathological study was normal in four cases. This family was living in
Florianopolis, Santa Catarina, where there is a great number of Portuguese
descendants from the Azores Islands. The worldwide presence of the disease seems
to result from the genic diffusion of the disease with the Portuguese emigration
during the Great Navigations Era and with some later emigratory settlement.

Publication Types:
Case Reports
Review
Review, Tutorial

PMID: 1810235 [PubMed – indexed for MEDLINE]

 

15: Epilepsy Res. 1990 Dec;7(3):226-9.

The efficacy and long-term tolerability of lamotrigine in the treatment of
severe epilepsy.

Sander JW, Trevisol-Bittencourt PC, Hart YM, Patsalos PN, Shorvon SD.

INSEG-Epilepsy Research Group, Institute of Neurology, London, U.K.

We report the effects of the addition of lamotrigine, a novel antiepileptic
drug, to the therapy of 125 patients with severe refractory epilepsy. Forty-five
patients (36%) reported adverse experiences and in 19 (15%), the drug was
withdrawn. The commonest adverse experiences were diplopia, headache, ataxia,
drowsiness, skin rash and deterioration in seizure control. Two patients were
withdrawn for other reasons. The remaining 104 patients were followed for a mean
of 11 months (range 3-27): 26 (25%) of these showed a marked improvement in
seizure frequency (a 50% or more reduction when compared with the pre-trial
period), but no patient was rendered seizure-free. Tolerance to the effects of
the drug was not seen.

PMID: 2289481 [PubMed – indexed for MEDLINE]

 

16: Arq Neuropsiquiatr. 1990 Dec;48(4):520-4.

[Lennox-Gastaut syndrome of late onset? Report of a case]

[Article in Portuguese]

Trevisol-Bittencourt PC, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina, UK.

The Lennox-Gastaut syndrome (LGS), although described earlier, was first
accepted in 1966 as a form of severe childhood epileptic encephalopathy,
characterized by refractory epileptic seizures of diverse types, typical EEG
abnormalities and slow mental development. This condition, however, is
surrounded by controversy as the clinical criteria used by various authors to
define it are quite different. It is very likely that this eponym has been used
to harbour distinct epileptic conditions, all of which have in common a slow
spike and wave complex in their EEG recording. Despite the fact that one of the
hallmarks of LGS is an onset in early childhood, to add to the confusion, some
cases of a LGS-like condition with onset in adulthood have been described. We
report here one such case: a 28 year old man whose LGS-like condition started
after severe head trauma at the age of 20. In addition we briefly review the
difficulties in making this diagnosis, using this case to illustrate these
aspects.

Publication Types:
Case Reports

PMID: 2128796 [PubMed – indexed for MEDLINE]

 

17: J Neurol Neurosurg Psychiatry. 1990 Nov;53(11):1008-10.

Comment in:
J Neurol Neurosurg Psychiatry. 1991 Sep;54(9):849.

Evaluation of vigabatrin as an add-on drug in the management of severe epilepsy.

Sander JW, Trevisol-Bittencourt PC, Hart YM, Shorvon SD.

Institute of Neurology, National Hospital for Nervous Diseases, London, United
Kingdom.

The effects of the addition of Vigabatrin, a new anti-epileptic drug, to the
therapy of 128 patients with severe medically refractory epilepsy is reported.
Forty two (33%) of patients experienced side effects, which were predominantly
neurotropic. In 28 (22%), the drug was withdrawn because of these side effects.
The commonest side effects were drowsiness and behavioural change. The remaining
100 patients were followed for a mean of 30 weeks (range 12-75). Forty one of
these patients showed a marked improvement in seizure frequency (a 50% or more
reduction when compared with the pre-trial period), and nine (7%) were rendered
seizure free. Apparent tolerance to the effects of the drug were noted in five
patients. An exacerbation of seizures may occur if the drug is withdrawn too
quickly. Vigabatrin appears to be a promising new anti-epileptic drug.

Publication Types:
Clinical Trial

PMID: 2283513 [PubMed – indexed for MEDLINE]

 

18: Arq Neuropsiquiatr. 1990 Sep;48(3):261-9.

[Epilepsy at a psychiatric hospital]

[Article in Portuguese]

Trevisol-Bittencourt PC, Becker N, Pozzi CM, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina, Brasil.

Epilepsy was surveyed in the largest psychiatric hospital in the Santa Catarina
State, southern Brazil. This establishment was designed for one thousand
long-term beds but at the time of the survey there were 1126 inpatients.
Diagnosis ranged from anxiety neurosis to schizophrenia although patients with
epilepsy, with or without psychiatric symptoms were also admitted. The following
aspects were analyzed: prevalence of epilepsy, seizure types, antiepileptic drug
treatment and psychiatric diagnosis. 171 patients with epilepsy were identified
(prevalence 152/1000), generalized tonic clonic attacks were the commonest
seizure type and polytherapy was the standard treatment. In at least 85 of the
epileptic patients there was no reason for prolonged institutionalization in a
psychiatric environment. Moreover, most of the sample were prescribed large
amounts of sedative drugs. A multidisciplinary approach and outpatients services
are urgently required to improve the prognosis and well-being of patients with
epilepsy who are referred to psychiatric care.

PMID: 2264780 [PubMed – indexed for MEDLINE]

 

19: Arq Neuropsiquiatr. 1990 Sep;48(3):360-5.

[Epilepsy and Turner’s syndrome: report of a case and review of the literature]

[Article in Portuguese]

Trevisol-Bittencourt PC, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina.

Fifty years ago Henry Turner identified the association of sexual infantilism,
webbed neck and cubitus valgus to be a separate entity and subsequently gonadal
dysgenesis was appended to the definition. Twenty years after the original
report it was demonstrated that in typical patients with Turner’s syndrome the
chromosomal composition was 45.XO and in addition cases of mosaicism were
described. Although much general information is now available about Turner’s
syndrome the neurological complications are largely neglected. In this paper we
review the case of a patient with Turner’s syndrome who at age of 16 years
developed severe epilepsy. In addition, a survey of the literature concerning
neurological abnormalities associated with this syndrome is presented. It seems
clear that neurological symptoms in patients with Turner’s syndrome deserves
consideration for many reasons; may be the most significant is the evidence that
patients with this syndrome have a high incidence of vascular abnormalities,
which may also affect the CNS. Empirically patients with Turner’s syndrome are
at risk of developing cerebral vascular diseases. Also the relatively high
frequency of cognitive abnormalities in these patients seems to justify the
hypothesis that in a certain percentage of cases a CNS dysfunction or
malformation, not necessarily of a vascular nature, may be a component of the
syndrome still nor described.

Publication Types:
Case Reports
Review
Review, Tutorial

PMID: 2124799 [PubMed – indexed for MEDLINE]

 

20: Neurology. 1990 Jun;40(6):1010-1.

Comment on:
Neurology. 1989 Oct;39(10):1294-7.

Absence status.

Sander JW, Hart YM, Trevisol-Bittencourt PC.

Publication Types:
Comment
Letter

PMID: 2111897 [PubMed – indexed for MEDLINE]

 

21: Acta Neurol Scand. 2003 Dec;108(6):424-7.

Erratum in:
Acta Neurol Scand. 2004 Jan;109(1):84.

Interictal serum S100B levels in chronic neurocysticercosis and idiopathic
epilepsy.

Portela LV, Tort AB, Walz R, Bianchin M, Trevisol-Bittencourt PC, Wille PR,
Cardoso RC, Ishida MM, vonWangenheim A, Grisard EC, Steindel M, Goncalves CA,
Souza DO.

Departamento de Bioquimica, ICBS, Universidade Federal do Rio Grande do Sul,
Porto Alegre, RS, Brazil. roska@ufrgs.br

OBJECTIVE: To assess whether serum S100B levels could reflect a glial response
in patients with epilepsy secondary to neurocysticercosis (NCC) and with
idiopathic epilepsy. SUBJECTS AND METHODS: Serum S100B levels were measured
using an immunoluminometric assay in 20 patients with focal epilepsy related to
chronic NCC (NCC group), and 19 patients with focal epilepsy (EPI group),
matched by epidemiological and clinical data. Epileptic patients were compared
with 20 healthy controls (CON group) matched by age and sex. RESULTS: No
difference was observed in S100B levels among NCC, EPI and CON groups (P>0.39).
Serum S100B levels were not affected by antiepileptic drugs, frequency and type
of seizures. Preliminarily, significantly higher levels of S100B were observed
in patients with bilateral electroencephalographic (EEG) findings than in
patients with unilateral and normal EEG findings (P<0.05). CONCLUSION: Serum
S100B is normal in patients with focal epilepsy related or not to chronic NCC.

PMID: 14616295 [PubMed – indexed for MEDLINE]

 

22: Epilepsy Behav. 2001 Dec;2(6):558-562.

Cognitive Performance of Patients with Epilepsy and Calcified Neurocysticercotic
Lesions: A Case-Control Study.

Boppre MC, Wille PR, Guarnieri R, Rezek K, Carqueja C, Trevisol-Bittencourt PC,
Walz JC, Bianchin M, von Wangenheim A, Chaves ML, Walz R.

Servico de Neurologia, Hospital Regional de Sao Jose, Sao Jose, SC, Brazil

Neuropsychological tests were applied to 20 patients with focal epilepsy related
to calcified neurocysticercosis (NCC) (mean: three lesions/patient; NCC group),
22 patients with focal epilepsy without NCC (EPI group), and 29 healthy controls
matched for age, sex, and educational level. The EPI and NCC groups were matched
for age at onset of epilepsy, epilepsy duration, frequency of attacks, seizure
semiology, interictal EEG findings, and antiepileptic drugs used. There were no
differences in the digit span, word span, calculus, and Mini-Mental State
examination among the three groups studied. The NCC and EPI groups showed lower
scores than controls in immediate and delayed verbal memory, famous faces test,
spatial recognition span, abstractions and judgment, and visuoconstructional
abilities. The EPI group, but not the NCC group, also had lower scores in a
praxis tests. There were no differences between the NCC and EPI groups in any of
the tests applied (P > 15), except for the spatial recognition span, which was
lower in the former. Cognitive impairment is a prevalent neuropsychological
feature of patients with epilepsy and NCC.

PMID: 12609389 [PubMed – as supplied by publisher]

 

23: Eur J Hum Genet. 2002 Nov;10(11):773-81.

Mutational spectrum of the CHAC gene in patients with chorea-acanthocytosis.

Dobson-Stone C, Danek A, Rampoldi L, Hardie RJ, Chalmers RM, Wood NW, Bohlega S,
Dotti MT, Federico A, Shizuka M, Tanaka M, Watanabe M, Ikeda Y, Brin M, Goldfarb
LG, Karp BI, Mohiddin S, Fananapazir L, Storch A, Fryer AE, Maddison P, Sibon I,
Trevisol-Bittencourt PC, Singer C, Caballero IR, Aasly JO, Schmierer K, Dengler
R, Hiersemenzel LP, Zeviani M, Meiner V, Lossos A, Johnson S, Mercado FC,
Sorrentino G, Dupre N, Rouleau GA, Volkmann J, Arpa J, Lees A, Geraud G,
Chouinard S, Nemeth A, Monaco AP.

The Wellcome Trust Centre For Human Genetics, University of Oxford, UK.

Chorea-acanthocytosis (ChAc) is an autosomal recessive neurological disorder
whose characteristic features include hyperkinetic movements and abnormal red
blood cell morphology. Mutations in the CHAC gene on 9q21 were recently found to
cause chorea-acanthocytosis. CHAC encodes a large, novel protein with a yeast
homologue implicated in protein sorting. In this study, all 73 exons plus
flanking intronic sequence in CHAC were screened for mutations by denaturing
high-performance liquid chromatography in 43 probands with ChAc. We identified
57 different mutations, 54 of which have not previously been reported, in 39
probands. The novel mutations comprise 15 nonsense, 22 insertion/deletion, 15
splice-site and two missense mutations and are distributed throughout the CHAC
gene. Three mutations were found in multiple families within this or our
previous study. The preponderance of mutations that are predicted to cause
absence of gene product is consistent with the recessive inheritance of this
disease. The high proportion of splice-site mutations found is probably a
reflection of the large number of exons that comprise the CHAC gene. The CHAC
protein product, chorein, appears to have a certain tolerance to amino-acid
substitutions since only two out of nine substitutions described here appear to
be pathogenic.

PMID: 12404112 [PubMed – indexed for MEDLINE]

 

24: Arq Neuropsiquiatr. 2001 Jun;59(2-A):214-8.

[The most common conditions in a neurology specialty clinic]

[Article in Portuguese]

Trevisol-Bittencourt PC, Ferreira MA, Marasciulo AC, Collares CF.

Disciplina de Neurologia, Universidade Federal de Santa Catarina. pcb@hu.ufsc.br

OBJECTIVE: To present the most frequent diagnosis among patients referred for
neurological evaluation to estimate their labour capacities at the unit of
National Institute of Social Security (INSS), Florianopolis-SC, southern Brazil.
METHOD: Review of all medical records of 108 patients evaluated between October
97 and May 98. The sample was submitted to judicious medico-legal assessment to
define their final diagnosis. RESULTS: Neurological evaluation disclosed as the
commonest disorders, in decreasing order of frequency: epilepsy, rheumatic
diseases, psychiatric illnesses, neurological disorders related to chronic
alcoholism, head trauma syndrome and cerebrovascular diseases. CONCLUSION:
Neurological disorders may be responsible for important disability among workers
in our society. However, the potential for social rehabilitation, often
underestimated, must be considered. Moreover, diverse non-neurological
conditions used to be sent for neurological evaluation.

PMID: 11400028 [PubMed – indexed for MEDLINE]

 

25: Arq Neuropsiquiatr. 1999 Sep;57(3B):787-92.

[Causes of late-onset epilepsy in an epilepsy clinic of Santa Catarina–Southern
Brazil]

[Article in Portuguese]

Rigatti M, Trevisol-Bittencourt PC.

Universidade Federal de Santa Catarina (UFSC).

OBJECTIVE: To identify the most common etiologies of late-onset epilepsy in our
society. METHOD: Retrospective and descriptive study in the CME/SUS of
Florianopolis from 1990 to 1998, evaluating 120 handbooks of patients with
late-onset epilepsy, that is, those that had initiated epileptic seizures of the
18 years in ahead. The collected variables were: age of the first seizure,
etiologies and morbid familial history. RESULTS: The prevalence rate found for
late-onset epilepsy was 29.48%. Most of the sample had partial symptomatic
epilepsy and the most frequent and important were: cysticercosis with 20%, head
trauma with 15%, febrile convulsions in infancy with 5% and 35% were classified
as idiopatic. CONCLUSION: Prevalence of late-onset epilepsy in our society is
29.48%, value slightly upper to the told one in developed countries (25%). Very
probably, this difference is consequence of the epidemic character of
cysticercosis among us. Moreover, head trauma and febrile convulsions are common
etiologies. Prophylaxis of epilepsy is viable and urgently necessary in our
society.

PMID: 10751913 [PubMed – indexed for MEDLINE]

 

26: Arq Neuropsiquiatr. 1999 Sep;57(3B):784-6.

Phenytoin as the first option in female epileptic patients?

Trevisol-Bittencourt PC, da Silva VR, Molinari MA, Troiano AR.

University Hospital, Santa Catarina Federal University (UFSC), Brasil.
pcb@hu.ufsc.br

OBJECTIVE: Phenytoin (PHT) is one of the first-choice drugs in several epileptic
syndromes, mostly in partial epilepsies, in which case it is effective as
carbamazepine and phenobarbital. However, like any other anti-epileptic drug
(AED), unpleasant side-effects are not rare. The aim of this study is the
evaluation of dermatological troubles related to chronic PHT usage in female
patients. METHOD: Between 1990-93, 731 new patients underwent investigation for
epilepsy at the Multidisciplinary Clinic for Epilepsy in our State. In this
sample 283 were AED users at the time of the first assessment. Sixty one female
patients taking PHT were identified. They were taking PHT in a dosage ranging
from 100 to 300 mg daily, in mono or polytherapy regimen, during 1-5 previous
years. RESULTS: More than 50% of the sample showed coarse facial features made
by the combination of several degrees of acne, hirsutism and gingival
hyperplasia. CONCLUSION: Except in emergency situations, PHT should not be
prescribed as the first option to the treatment of female epileptic patients,
because not uncommonly the cosmetic side-effects are more socially handicapping
than the epileptic syndrome by itself.

PMID: 10751912 [PubMed – indexed for MEDLINE]

 

27: Arq Neuropsiquiatr. 2000 Jun;58(2B):548-55.

[Interictal personality syndrome in non-dominant temporal lobe epilepsy: case
report]

[Article in Portuguese]

Trevisol-Bittencourt PC, Troiano AR.

Departamento de Clinica Medica, Hospital Universitario, Universidade Federal de
Santa Catarina. pcb@hu.ufsc.br

The syndrome of interictal personality in non-dominant temporal lobe epilepsy
consists of hyposexuality, hyperreligiosity, humorlessness and hypergraphia. Its
notification, in 1974, was followed by an extensive search for these traits in
broad epileptic populations. Nevertheless, these statistical studies failed to
match this syndrome in general temporal lobe epileptics, and its existence
became then target of doubt. We report the case of a 35 year-old man presenting
partial complex epilepsy, whose singularity lies in his sophisticated drawing
abilities. The large amount of buildings and houses he paints expresses his
hypergraphia. He also presents hyposexuality and hyperreligiosity. MRI shows
right mesial temporal sclerosis. Temporal hyperconnection, caused by a basal
temporal irritative focus, is the most probable pathophysiological mechanism.
Epileptic fits can be controlled in the majority of cases. However, behavioural
symptoms usually do not respond to pharmacological approach or psychotherapy.

Publication Types:
Case Reports

PMID: 10920421 [PubMed – indexed for MEDLINE]

 

28: Arq Neuropsiquiatr. 1999 Jun;57(2B):489-94.

[Neuroacanthocytosis. A case report]

[Article in Portuguese]

Troiano AR, Trevisol-Bittencourt PC.

Faculdade de Medicina da Universidade Federal de Santa Catarina (UFSC), Brasil.

We present a 45 years old man with neuroacanthocytosis. This gentleman has
complex partial seizures and generalized tonic-clonic seizures, as well as
movement disorders characterized by chorea and orofacial diskinesia.
Complementary examination shows acanthocytosis of 11% on peripheral blood,
irritative focus on right temporal lobe on EEG, serum creatinokinase of 101 U/l
and volume reduction and hypersignal on caudate nucleus and putamen bilaterally
on MRI.

Publication Types:
Case Reports
Review
Review of Reported Cases

PMID: 10450359 [PubMed – indexed for MEDLINE]

 

29: Arq Neuropsiquiatr. 1999 Jun;57(2B):401-4.

[Clinical-epidemiological study of patients with juvenile myoclonic epilepsy in
Santa Catarina State, Brazil]

[Article in Portuguese]

Figueredo R, Trevisol-Bittencourt PC, Ferro JB.

Clinica Multidisciplinar de Epilepsia da Policlinica de Referencia Regional
I/SUS, Florianopolis, SC, Brasil. delfigue@zaz.com.br

We aimed to characterize the clinical profile and to establish the prevalence of
juvenile myoclonic epilepsy (JME) among 939 consecutive patients referred to our
epilepsy clinic. Inclusion criteria in the study were: a) myoclonic jerks,
preferably on awakening; b) beginning of the symptoms between 8 and 26
year-of-age; c) typical pattern of electroencephalographic discharges; and d)
good response to sodium valproate (VPA). In a retrospective design, 26 cases of
JME were identified (prevalence 2.8%). Most of these patients were female
(73.1%). Mean age at onset of symptoms was 13 (range 7-18). Tonic-clonic
seizures were reported in 92.3% and absence seizures in 19.2%. Besides, 92.3% of
the patients had typical EEG epileptiform activity and 76.9% had good response
to VPA with daily dose ranging from 500 mg to 1500 mg.

PMID: 10450346 [PubMed – indexed for MEDLINE]

 

30: Arq Neuropsiquiatr. 1998 Mar;56(1):53-8.

[Prevalence of neurocysticercosis among epileptic in-patients in the west of
Santa Catarina–southern Brazil]

[Article in Portuguese]

Trevisol-Bittencourt PC, da Silva NC, Figueredo R.

Disciplina de Neurologia, Hospital Universitario UFSC, Brasil.
pcb.latino@intergate.com.br

Neurocysticercosis (NC) is an endemic condition in several areas of Brazil. It
is most likely the major responsible for the high prevalence of epilepsy in our
country, estimated in 1-2% of general population. We performed a study to
evaluate NC as aetiology of epilepsy in the west of Santa Catarina. This state
belong to southern Brazil and it has a very developed economy. However, due the
widespread swine farming in the west district, many of them without any sanitary
control, the national health authorities have considered all this area at risk
to NC. The study was carried out in Chapeco, the main town in that region, where
CT Scan service was started in 1995. All patients put on hospital care due
epileptic seizures in 1995-96 were considered. Febrile convulsions were excluded
of the sample. We found a very expressive prevalence rate of NC among patients
suffering from epilepsy. Roughly 24% of these patients, showed unequivocal
tomography evidences for the diagnosis of NC. Our data suggest cysticercosis as
a real endemic trouble in the area and, overdosis of information in proper
language, diffuse to the whole population, seems to be the only remedy to fight
against it. Moreover, we should pay special attention to everyone related to
swine farming, does not matter how this activity has been classified.

PMID: 9686120 [PubMed – indexed for MEDLINE]

 

31: Arq Neuropsiquiatr. 1997 Sep;55(3B):519-29.

Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2,
MJD/SCA3 and DRPLA) in a large group of Brazilian patients.

Lopes-Cendes I, Teive HG, Calcagnotto ME, Da Costa JC, Cardoso F, Viana E,
Maciel JA, Radvany J, Arruda WO, Trevisol-Bittencourt PC, Rosa Neto P, Silveira
I, Steiner CE, Pinto Junior W, Santos AS, Correa Neto Y, Werneck LC, Araujo AQ,
Carakushansky G, Mello LR, Jardim LB, Rouleau GA.

Centre for Research in Neuroscience, McGill University, Montreal, QC, Canada.
bke6@musicb.mcgill.ca

Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and
Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three
distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by
expansions of an unstable CAG repeat localized in the coding region of the
causative genes. Another related disease, dentatorubropallidoluysian atrophy
(DRPLA) is also caused by an unstable triplet repeat and can present as SCA in
late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3
and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90
unrelated families with various patterns of inheritance and originating in
different geographic regions of Brazil. We found mutations in 35 families (39%),
32 of them with a clear autosomal dominant inheritance. The frequency of the
SCA1 mutation was 3% of all patients; and 6% in the dominantly inherited SCAs.
We identified the SCA2 mutation in 6% of all families and in 9% of the families
with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30%
of all patients; and in the 44% of the dominantly inherited cases. We found no
DRPLA mutation. In addition, we observed variability in the frequency of the
different mutations according to geographic origin of the patients, which is
probably related to the distinct colonization of different parts of Brazil.
These results suggest that SCA may be occasionally caused by the SCA1 and SCA2
mutations in the Brazilian population, and that the MJD/SCA3 mutation is the
most common cause of dominantly inherited SCA in Brazil.

PMID: 9629399 [PubMed – indexed for MEDLINE]

 

32: Arq Neuropsiquiatr. 1992 Sep;50(3):369-74.

[Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report
of a case and review of the literature]

[Article in Portuguese]

Trevisol-Bittencourt PC.

Departamento de Clinica Medica, Universidade Federal de Santa Catarina (UFSC),
Brasil.

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome
by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent
clinical experience has corroborated and enlarged their original observations.
PSP has become familiar as a chronic progressive disorder with extrapyramidal
rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a
characteristic supranuclear ophthalmoplegia. It is a significant cause of
parkinsonism and its etiology remains obscure. The case of a patient from Santa
Catarina who presented definite clinical evidences of this syndrome is reported.
This is the first description in this southern Brazilian State, where at least
50 more patients should exist, if we may extrapolate the prevalence rate of this
condition in developed countries to this well developed area of Brazil. A review
of the literature was undertaken with emphasis on recent clinical and
therapeutic aspects of PSP.

Publication Types:
Case Reports

PMID: 1308417 [PubMed – indexed for MEDLINE]

 

33: Arq Neuropsiquiatr. 1991 Jun;49(2):208-10.

Ketoconazole in the treatment of cryptococcosis of the central nervous system.

Trevisol-Bittencourt PC, Sander JW, Silvado CS, Wittig EO.

Departamento de Clinica Medica, Hospital de Clinicas, Universidade Federal do
Parana, Curitiba, Brasil.

Two patients with cryptococcosis of the CNS were treated with ketoconazole
(KTZ), an imidazole derivative with fungistatic properties: they had either
failed standard therapy (Amphotericin-B + 5-Fluorocytosine) or suffered
intolerable side-effects to it. Both patients were administered KTZ 800 mg/day
as monotherapy for six months without interruption and both responded. One month
after KTZ therapy was withdrawn, however, a relapse of the infection was seen in
one case. Side-effects were minimal during the trial of treatment. KTZ could be
a useful drug in some cases of neurocryptococcosis.

Publication Types:
Case Reports

PMID: 1810241 [PubMed – indexed for MEDLINE]

 

34: Arq Neuropsiquiatr. 1991 Jun;49(2):172-9.

[Machado-Joseph disease: description of 5 members of a family]

[Article in Portuguese]

Teive HA, Arruda WO, Trevisol-Bittencourt PC.

Unidade de Ciencias Neurologicas, Curitiba, Brasil.

The authors report the clinical and laboratorial findings of 5 affected members
(all males) of a family with Machado-Joseph disease. The mode a inheritance was
autosomal dominant. The mean onset age was 38 years (range 30-50 years). The
clinical picture was pleomorphic and included cerebellar ataxia, external
ophthalmoplegia with bulging eyes, extrapyramidal/pyramidal syndromes,
amyotrophy with fasciculations and peripheral neuropathy, in variable degrees of
severity. In one patient parkinsonian rigidity was greatly improved with the use
of trihexaphenidyl and L-dopa. CT scan examinations disclosed a variable degree
of cerebellar atrophy, with mild cerebral atrophy in one patient. Brainstem
evoked potentials were normal in two patients. EMG showed denervation in three
patients. Muscle biopsy (gastrocnemium) with histochemical studies revealed
chronic muscle denervation in four cases. Sural nerve biopsy with conventional
pathological study was normal in four cases. This family was living in
Florianopolis, Santa Catarina, where there is a great number of Portuguese
descendants from the Azores Islands. The worldwide presence of the disease seems
to result from the genic diffusion of the disease with the Portuguese emigration
during the Great Navigations Era and with some later emigratory settlement.

Publication Types:
Case Reports
Review
Review, Tutorial

PMID: 1810235 [PubMed – indexed for MEDLINE]

 

35: Epilepsy Res. 1990 Dec;7(3):226-9.

The efficacy and long-term tolerability of lamotrigine in the treatment of
severe epilepsy.

Sander JW, Trevisol-Bittencourt PC, Hart YM, Patsalos PN, Shorvon SD.

INSEG-Epilepsy Research Group, Institute of Neurology, London, U.K.

We report the effects of the addition of lamotrigine, a novel antiepileptic
drug, to the therapy of 125 patients with severe refractory epilepsy. Forty-five
patients (36%) reported adverse experiences and in 19 (15%), the drug was
withdrawn. The commonest adverse experiences were diplopia, headache, ataxia,
drowsiness, skin rash and deterioration in seizure control. Two patients were
withdrawn for other reasons. The remaining 104 patients were followed for a mean
of 11 months (range 3-27): 26 (25%) of these showed a marked improvement in
seizure frequency (a 50% or more reduction when compared with the pre-trial
period), but no patient was rendered seizure-free. Tolerance to the effects of
the drug was not seen.

PMID: 2289481 [PubMed – indexed for MEDLINE]

 

36: Arq Neuropsiquiatr. 1990 Dec;48(4):520-4.

[Lennox-Gastaut syndrome of late onset? Report of a case]

[Article in Portuguese]

Trevisol-Bittencourt PC, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina, UK.

The Lennox-Gastaut syndrome (LGS), although described earlier, was first
accepted in 1966 as a form of severe childhood epileptic encephalopathy,
characterized by refractory epileptic seizures of diverse types, typical EEG
abnormalities and slow mental development. This condition, however, is
surrounded by controversy as the clinical criteria used by various authors to
define it are quite different. It is very likely that this eponym has been used
to harbour distinct epileptic conditions, all of which have in common a slow
spike and wave complex in their EEG recording. Despite the fact that one of the
hallmarks of LGS is an onset in early childhood, to add to the confusion, some
cases of a LGS-like condition with onset in adulthood have been described. We
report here one such case: a 28 year old man whose LGS-like condition started
after severe head trauma at the age of 20. In addition we briefly review the
difficulties in making this diagnosis, using this case to illustrate these
aspects.

Publication Types:
Case Reports

PMID: 2128796 [PubMed – indexed for MEDLINE]

 

37: J Neurol Neurosurg Psychiatry. 1990 Nov;53(11):1008-10.

Comment in:
J Neurol Neurosurg Psychiatry. 1991 Sep;54(9):849.

Evaluation of vigabatrin as an add-on drug in the management of severe epilepsy.

Sander JW, Trevisol-Bittencourt PC, Hart YM, Shorvon SD.

Institute of Neurology, National Hospital for Nervous Diseases, London, United
Kingdom.

The effects of the addition of Vigabatrin, a new anti-epileptic drug, to the
therapy of 128 patients with severe medically refractory epilepsy is reported.
Forty two (33%) of patients experienced side effects, which were predominantly
neurotropic. In 28 (22%), the drug was withdrawn because of these side effects.
The commonest side effects were drowsiness and behavioural change. The remaining
100 patients were followed for a mean of 30 weeks (range 12-75). Forty one of
these patients showed a marked improvement in seizure frequency (a 50% or more
reduction when compared with the pre-trial period), and nine (7%) were rendered
seizure free. Apparent tolerance to the effects of the drug were noted in five
patients. An exacerbation of seizures may occur if the drug is withdrawn too
quickly. Vigabatrin appears to be a promising new anti-epileptic drug.

Publication Types:
Clinical Trial

PMID: 2283513 [PubMed – indexed for MEDLINE]

 

38: Arq Neuropsiquiatr. 1990 Sep;48(3):261-9.

[Epilepsy at a psychiatric hospital]

[Article in Portuguese]

Trevisol-Bittencourt PC, Becker N, Pozzi CM, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina, Brasil.

Epilepsy was surveyed in the largest psychiatric hospital in the Santa Catarina
State, southern Brazil. This establishment was designed for one thousand
long-term beds but at the time of the survey there were 1126 inpatients.
Diagnosis ranged from anxiety neurosis to schizophrenia although patients with
epilepsy, with or without psychiatric symptoms were also admitted. The following
aspects were analyzed: prevalence of epilepsy, seizure types, antiepileptic drug
treatment and psychiatric diagnosis. 171 patients with epilepsy were identified
(prevalence 152/1000), generalized tonic clonic attacks were the commonest
seizure type and polytherapy was the standard treatment. In at least 85 of the
epileptic patients there was no reason for prolonged institutionalization in a
psychiatric environment. Moreover, most of the sample were prescribed large
amounts of sedative drugs. A multidisciplinary approach and outpatients services
are urgently required to improve the prognosis and well-being of patients with
epilepsy who are referred to psychiatric care.

PMID: 2264780 [PubMed – indexed for MEDLINE]

 

39: Arq Neuropsiquiatr. 1990 Sep;48(3):360-5.

[Epilepsy and Turner’s syndrome: report of a case and review of the literature]

[Article in Portuguese]

Trevisol-Bittencourt PC, Sander JW.

Departamento de Neurologia, Hospital Universitario, Universidade Federal de
Santa Catarina.

Fifty years ago Henry Turner identified the association of sexual infantilism,
webbed neck and cubitus valgus to be a separate entity and subsequently gonadal
dysgenesis was appended to the definition. Twenty years after the original
report it was demonstrated that in typical patients with Turner’s syndrome the
chromosomal composition was 45.XO and in addition cases of mosaicism were
described. Although much general information is now available about Turner’s
syndrome the neurological complications are largely neglected. In this paper we
review the case of a patient with Turner’s syndrome who at age of 16 years
developed severe epilepsy. In addition, a survey of the literature concerning
neurological abnormalities associated with this syndrome is presented. It seems
clear that neurological symptoms in patients with Turner’s syndrome deserves
consideration for many reasons; may be the most significant is the evidence that
patients with this syndrome have a high incidence of vascular abnormalities,
which may also affect the CNS. Empirically patients with Turner’s syndrome are
at risk of developing cerebral vascular diseases. Also the relatively high
frequency of cognitive abnormalities in these patients seems to justify the
hypothesis that in a certain percentage of cases a CNS dysfunction or
malformation, not necessarily of a vascular nature, may be a component of the
syndrome still nor described.

Publication Types:
Case Reports
Review
Review, Tutorial

PMID: 2124799 [PubMed – indexed for MEDLINE]

 

40: Neurology. 1990 Jun;40(6):1010-1.

Comment on:
Neurology. 1989 Oct;39(10):1294-7.

Absence status.

Sander JW, Hart YM, Trevisol-Bittencourt PC.

Publication Types:
Comment
Letter

PMID: 2111897 [PubMed – indexed for MEDLINE]