Non-convulsive status epilepticus as the initial manifestation in a family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant small-vessel disease caused by mutations of the NOTCH3 gene. It typically presents with migraine, recurrent brain ischaemia, and cognitive disorders. Seizures rarely present as the initial manifestation, with non-convulsive status epilepticus being even less frequent. We present a series of 3 related patients with this arteriopathy, 2 of whom presented status epilepticus as a manifestation of the disease.

Non-convulsive status epilepticus as the initial manifestation in a family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). / Status epilepticus no convulsivo como manifestación inicial en una familia con arteriopatía autosómica dominante cerebral con infartos subcorticales y leucoencefalopatía (CADASIL).

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant small-vessel disease caused by mutations of the NOTCH3 gene. It typically presents with migraine, recurrent brain ischaemia, and cognitive disorders. Seizures rarely present as the initial manifestation, with non-convulsive status epilepticus being even less frequent. We present a series of 3 related patients with this arteriopathy, 2 of whom presented status epilepticus as a manifestation of the disease.

Signo del tridente en la neurosarcoidosis medular / Trident sign in spinal cord neurosarcoidosis

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[Trident sign in spinal cord neurosarcoidosis]. / Signo del tridente en la neurosarcoidosis medular.

TITLE: Signo del tridente en la neurosarcoidosis medular.

Beneficial effects of lisuride in Meige disease.

We studied the therapeutic effects of apomorphine, bromocriptine, and lisuride on the dystonic spasms of two patients with Meige disease. Lisuride, a potent dopamine receptor agonist, and apomorphine had equal efficacy in lessening facial dyskinesia, while bromocriptine had no consistent effect. Lisuride can be an excellent drug in the treatment of this disabling disorder.

Treatment of Parkinson's disease with subcutaneous lisuride infusions.

Four patients with Parkinson's disease and severe fluctuating responses to levodopa and oral dopamine agonists were treated with continuous administration of lisuride infusions, administered by means of an externally worn pump. Levodopa dosage ranged from 300 to 687 mg/day and was kept stable throughout the study. In addition increasing doses of lisuride were injected subcutaneously in the abdomen. Lisuride doses ranged from 41 to 104 micrograms/h. A marked improvement in mobility was observed in every patient while severe biphasic dyskinesais almost remitted in one of them. The most common side-effect was the presence of subcutaneous nodules appearing at the injection site. Two cases had mild hemorrhagic complications and one initially had nausea. One patient developed acute psychiatric disturbances severe enough to be excluded from the study. Our findings suggest that lisuride subcutaneous infusions can be useful in severily handicapped parkinsonian patients, however local and psychiatric side-effects may be a serious threat in the long-term care.

Continuous dopaminergic stimulation in cranial dystonia.

Meige's disease is a distressing complaint, the treatment of which often poses a challenge to the neurologist. The patient described here had blepharospasm-oromandibular dystonia, which responded transiently to oral lisuride. On three occasions, drug holidays successfully restored efficacy but thereafter further trials proved fruitless. Continuous subcutaneous lisuride administration in 0.35 mg doses per day, by means of a portable infusion pump, led to sustained improvement for 7 months. No major side effects were observed. Our findings suggest that this treatment deserves further trials.

Bruxism secondary to chronic antidopaminergic drug exposure.

Eight cases of diurnal bruxism (DB) secondary to long-term antidopaminergic drug exposure are reported. Five exhibited a grinding pattern, one a clenching form, and two a mixed type. An odontological etiology was absent throughout. EMG recordings disclosed two distinct patterns of muscle activity, one with brief rhythmic, forceful contractions and the other featuring sustained prolonged contractions. Surface EMG and EEG monitoring during a 24-h period confirmed the absence of bruxism during sleep. Several drug trials failed to provide relief. Our findings support DB as a focal tardive dystonia syndrome.

L-dopa malabsorption in a parkinsonian patient with Strongyloides stercoralis duodenitis.

We report a parkinsonian patient initially responding to L-dopa who developed a severe loss of drug efficacy due to Strongyloides stercoralis duodenitis. The patient was put on mebendazole and metronidazole, and the parasitosis abated, allowing L-dopa reduction by 33%. Our patient illustrates the advisability of searching for Strongyloides stercoralis when L-dopa malabsorption is suspected in Parkinson's disease.

Paroxysmal dystonia responsive to anticholinergic drugs.

A case of a mentally retarded patient with sporadic paroxysmal dystonia, unresponsive to anticonvulsant therapy, is described. He had a long-standing history of neuroleptic drug intake. Trihexyphenidyl in a total daily dosage of 20 mg totally suppressed the crises.

Treatment of Tourette's syndrome with calcium antagonists.

Six males and one female with chronic tic disorders, whose ages ranged from 12 to 31 years, were evaluated before treatment, after 1 month on placebo, after a single 10 mg nifedipine dose (three patients), and monthly while on flunarizine 10-15 mg (mean dose of 13 mg). None of the patients receiving nifedipine improved, but treatment with flunarizine significantly decreased both motor and phonic tic severity and frequency in all but one patient. Side effects included mild transient headaches in one patient, depression in one, and bradykinesia in two. Although a double-blind study is essential to validate our findings, results suggest that flunarizine is a useful drug in the treatment of Gilles de la Tourette syndrome.

Gilles de la Tourette syndrome: clinical features of 75 cases from Argentina.

A series of 75 cases of Gilles de la Tourette syndrome (GTS) from Argentina, whose ages ranged from 6 to 55 with a mean of 20.02, were evaluated to compare findings with those reported for other countries. Mean age at onset was 7.44 years and mean overall duration of symptoms was 12.58 years; 6.7% of cases were mild, 49% moderate and 44.3% severe. Most frequent presenting motor tics were excessive blinking in 41 followed by head jerking in 16 and eye winking in six, while phonic tics included coprolalia in 28.0%, echolalia in 17.5% and palilalia in 10.8%. Abnormal perinatal events were reported in 40.5%, while positive family history for tics was present in 26.66%. Obsessive-compulsive behaviour was evident in 66% and attention deficit disorder in 16% of cases. Self-injurious behaviour comprised onychophagia in 28 patients, lip-biting in seven and self-slapping in eight cases. Almost half of our patients were initially interpreted as having a psychogenic disorder indicating that GTS in Argentina is most likely underdiagnosed. It may be concluded that the overall pattern of GTS is not dissimilar to that described for European, Asian and American populations, thus highlighting the previously recognized cross-cultural uniformity.

[Multifocal demyelinating neuropathy after tetanus vaccine]. / Neuropatía desmielinizante multifocal siguiendo vacunación antitetánica.

A 39 year old man presenting multifocal demyelinating neuropathy (MFDN) is reported. Fifteen days before onset he had been vaccinated with 75 IU of tetanus toxoid. Although recent histories of vaccination or viral infection support the immunological hypothesis proposed for chronic acquired demyelinating neuropathies (CADN) it is reported for the first time in MFDN, suggesting that MFDN and CADN may share common pathophysiological mechanisms.

[A new levodopa benserazide preparation for Parkinson's disease with motor fluctuations refractory to standard L-dopa]. / Un nuevo preparado de levodopa benserazida en parkinsonianos con fluctuaciones motoras refractarias a tratamientos con L-dopa standard.

As Parkinson's disease worsens, many patients develop motor fluctuations which usually correlate directly or indirectly with L-dopa plasma levels. A new L-dopa-benserazide HBS preparation (Madopar) a control release pharmaceutical formulation which is activated when it contacts gastric fluid thus providing more stable L-dopa plasma levels, was assayed. Ten patients with a diagnosis of idiopathic Parkinson's disease and motor fluctuations otherwise unresponsive to conventional therapy were selected. The average age was 62 years and the duration of the disease 9 years. The motor status was evaluated on an hourly basis with the King's College Parkinson's disease rating scale; in addition, a nocturnal disability scale (Lees) was used. Out of the 10 patients, 2 dropped out within the first month due to worsening of parkinsonian signs, while 7 of the remainders preferred HBS preparation to the previous treatment. The number of off hours in this group was reduced by 58% and motor fluctuation became less severe. In only 3 cases was it possible to use HBS as monotherapy while in the rest standard L-dopa had to be added, specially as morning doses. The average L-dopa daily dose was increased by 36%. Unwanted effects included psychiatric disturbances in two (in one L-dopa dose had to be reduced) and epigastralgia in one. Our findings suggest that this L-dopa-benserazide control release may be considered an able therapeutic formulation in the control of motor fluctuations in Parkinson's disease.

[Neurologic complications by cocaine abuse]. / Complicaciones neurológicas por abuso de cocáina.

Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10% of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70% were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85%). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.

[Treatment of blepharospasm with botulinum toxin]. / Tratamiento del blefaroespasmo con toxina botulínica.

Blepharospasm is a relatively frequent cranial dystonia which may be seen either alone or related to orofacial-mandibular dystonia (Meige's syndrome). In its maximum degree it can cause functional blindness.Twelve patients with blepharospasm (4 essential and 8 Meige's syndrome) who had been previously treated unsuccessfully with drugs (trihexyphenidyl, biperiden, carbamazepine, lithium, baclofen, lisuride, imipramine, clonazepam and butyrophenones) were treated for 12 months with periocular injections of botulinum toxin (BOTOX). A "low" dose of 12,5 U per eye was employed. With this dose, eleven out of twelve patients experienced significant improvement which lasted from five to fifteen weeks. The only nonresponder obtained complete relief upon duplicating the dose. The only side effect was uni or bilateral ptosis in six patients which improved completely in seven to twenty one days. One patient developed a peripheral facial palsy with complete remission in nineteen days. No systemic side effects were noted. There was only one desertion from this study due to depression enhanced by prolonged (21 days) ptosis. All patients (including the deserter) agreed that treatment with BOTOX provided more relief than any other previous therapeutic method. Our results confirm those obtained by others but a more prolonged study is needed to better evaluate long term effects.

Development of parkinsonism after transient ischemic attacks.

In order to correlate the influence of cerebrovascular disorders with the appearance of parkinsonism, 115 patients aged between 32 and 84 years (mean = 65) with transient ischemic attacks were followed up for one year. They were treated with platelet antiaggregant drugs. None of them received neuroleptics, calcium antagonists or other drugs known to induce parkinsonism. During the study, 8 patients (mean = 75 years) developed parkinsonism, bilateral in all but one, who remarkably enough was the only case responding to L-dopa treatment. On comparing recorded with expected incidence, our series showed significantly greater values. Our findings suggest that cerebrovascular disorders are factors contributing to parkinsonism in the elderly.