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C Bentes

Publications and source records attributed to C Bentes.

10 recordsLinked to original sources
PubMed2001

Allgrove syndrome in adulthood.

A 35-year-old man with a past history of achalasia developed progressive spastic tetraparesis, distal limb atrophy, dysarthria, and dysphagia. A clinical diagnosis of amyotrophic lateral sclerosis (ALS) was considered before neurophysiological investigation, which disclosed a polyneuropathy and a prolonged central conduction time. One year later, the patient developed dysautonomic symptoms. Following confirmation of adrenal insufficiency, a diagnosis of Allgrove syndrome was made. This is a rare case, and we emphasize its clinical similarity with ALS.

Adult↗
PubMed2001

Epileptic seizures following subcortical infarcts.

Stroke is one of the most frequent causes of seizures in adulthood. Subcortical infarcts have been associated with post-stroke epileptic seizures, although less frequently than cortical ischaemia. We studied 113 patients from a hospital stroke registry. The patients had subcortical non-lacunar infarcts (64 striatocapsular infarcts, 13 thalamic, 8 choroidal artery territory, 28 internal border zone and white matter medullary infarcts) and at least 1 year of follow-up. Only 4 patients (3.5%) with striatocapsular infarcts suffered an epileptic seizure. Two seizures occurred within the first 24 h, 1 within the first month and 1 within the first year of stroke onset. Emboligenic cardiac conditions were significantly more common in patients with seizures (difference 37%; 95% confidence intervals 2-72%). Subsequently to subcortical infarct, epileptic seizures are infrequent, tend to occur early after stroke and have a very low 1-year recurrence risk.

Cerebral Infarction↗
PubMed1999

Multifocal motor neuropathy mimicking motor neuron disease: nine cases.

Multifocal motor neuropathy with persistent conduction block (MMN) is a rare clinical entity, mimicking motor neuron disease (MND). In order to research which are the most frequent nerves and segments where conduction block (CB) can be identified, we reviewed the clinical and neurophysiological data of nine patients with MMN who were studied and followed by the authors. Weakness and muscle atrophy of the dominant hand was the most frequent presentation. Lower limbs were involved later in the disease evolution. The ulnar and median nerves were the most affected nerves. They had conduction blocks mostly at the forearm and at Erb's point-elbow (or above elbow) segments. Both common peroneal and tibial nerves were frequently affected at their distal segments, but proximal segments were also probably involved. The presence of anti-GM1 antibodies was variable, and their determination was not essential for the diagnosis of MMN. Eight patients given IV immunoglobulin therapy had no disease progression. One patient was responsive to corticosteroids. The CB identification in our patients allowed us to clearly distinguish MMN from MND. The good prognosis and need for management with IV immunoglobulin, support the crucial role of a careful neurophysiological study to diagnose this clinical entity.

Adult↗
PubMed1999

Fibrillation and sharp-waves: do we need them to diagnose ALS?

BACKGROUND: Fibrillation/sharp-waves (fibs-sw) are considered an essential neurophysiological marker in Amyotrophic Lateral Sclerosis (ALS). We investigated how frequently an initial electrophysiological (EMG) evaluation, performed in ALS patients, revealed no fibs-sw. METHODS: The initial EMG studies from 68 ALS patients were analysed. The presence of fibs-sw in at least two muscles of each limb and two cranial-innervated muscles was assessed. RESULTS: We observed no fibs-sw in cranial-innervated muscles, and 7/15 bulbar-onset patients had no fibs-sw in limb muscles. Two of 28 upper-limb-onset patients, who had a short history before EMG, showed no fibs-sw. All lower-limb-onset patients had fibs-sw. Generally patients with no fibs-sw had diffuse abundant fasciculations. CONCLUSIONS: The absence of fibs-sw should not necessarily preclude the neurophysiological diagnosis of ALS in bulbar-onset patients. Early affected upper-limb-onset patients might show no fibs-sw, but profuse fasciculations associated with mild to moderate denervation-reinnervation in weak muscles are found.

Adult↗
PubMedDate not supplied

Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in Guillain-Barré syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.

Adult↗
PubMedDate not supplied

[Two myopathy cases].

INTRODUCTION: Early diagnosis and therapeutic measures of frequent pathologies like hyperthyroidism made their neurological complications less frequent. In spite of well known, these neurological complications are some times forgotten. CLINICAL CASES: We describe two myopathy cases, which presentation was namely fatigue. The inespecificity of this complaint create aetiology and syndromatic diagnosis difficulties, and therapeutic decision. In first case, the symptoms were very gradual, what is usual in older patients, and became extremely incapacitate, depending on others for every daily activity. In the second case, a young patient with diplopia suggested myasthenia. Both cases were diagnosed with hyperthyroidism and a quick improvement occurred with proper medication. Based in these cases, a thyrotoxic myopathy brief comment is made, as in spite of being frequent, these cases presented diagnosis difficulties.

Adolescent↗
PubMedDate not supplied

Sensory potential can be preserved in severe common peroneal neuropathy.

Neuropathy of Common Peroneal Nerve (CPN) is a frequent clinical condition, generally caused by compression at the fibula head. Three neurophysiological patterns were described: 1) segmental demyelination with conduction block; 2) axonal damage with loss of motor units and sensory potential; 3) a mixed pattern. We report 5 patients with foot drop in whom CPN neuropathy was identified. In 3 in spite of impressive abnormalities in various motor branches and fascicles of the nerve, the peroneus nerve sensory potential remained well preserved. Focal neuropathies can be remarkably selective in terms of motor and sensory deficits, the reason can rely on a different location of the fibres or be related to a distinct histological-biochemical constitution. A preserved SPSP should not exclude a CPN neuropathy.

Action Potentials↗
PubMedDate not supplied

Supratentorial giant cell ependymoma.

Ependymomas account for 3 to 9% of all neuroepithelial tumors and, although occurring most often within the ventricular system, they may arise from the extraventricular parenchyma as well. Several histologic patterns of these neoplasm are well known, but little attention has been devoted to a variant composed of giant elements. We describe the case of a 13-year-old girl experiencing a 3-month history of partial seizures in whom cranial magnetic resonance imaging showed an extraventricular, right parietotemporal neoplasm, adherent to the overlying dura mater. Histologic, immunohistochemical and ultrastructural findings were consistent with those of a high-grade ependymoma. The tumor was characterized by the presence of a major component of pleomorphic giant cells, as also seen in pleomorphic xantoastrocytoma, subependymal giant cell astrocytoma and glioblastoma multiforme. Similar elements have been described in two filum terminale and one supratentorial, intraventricular ependymoma, respectively. Histologic and evolutional data of those and of our own case suggest that isolated giant cells are not necessarily linked to a bad prognosis in ependymomas.

Adolescent↗