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Biomedical subjects

O F D'Cruz

Publications and source records attributed to O F D'Cruz.

14 recordsLinked to original sources

Parasomnias--an update.

Primary parasomnias are a group of sleep-related clinical disorders that occur in otherwise healthy children, which may be provoked by a variety of physical, psychosocial, and environmental stimuli. In typical cases, additional investigations are not necessary, and treatment should be instituted with the aim of reducing the frequency and adverse consequences of the parasomnia. In patients with co-existing clinical disorders, parasomnias need to be distinguished from symptoms of the underlying disorder. This article provides a brief review of the recent literature regarding parasomnias, and a clinically oriented approach to the management of parasomnias in patients with co-existing clinical disorders.

Child↗

Narcolepsy in a 12-month-old-boy.

Narcolepsy, although frequently beginning in childhood, is usually diagnosed in young adults. The diagnostic symptoms of narcolepsy are usually less typical in the young child, and sleep studies have not been standardized. We present a 12-month-old child with symptoms typical for narcolepsy who shows improvement with nonpharmacologic narcolepsy therapy.

Behavior Therapy↗

Intraoperative methods for confirmation of correct placement of the vagus nerve stimulator.

Vagus nerve stimulation is a progressive therapy for intractable epilepsy. Variations in cervical anatomy can complicate localization of the vagus nerve and may lead to inappropriate placement of the stimulator leads. We have developed two intraoperative techniques that improve correct identification of the vagus nerve. Both of these techniques utilize the co-localization of the recurrent laryngeal nerve with the vagus nerve. For patients undergoing stimulator placement with regional and local anesthesia, the stimulator current intensity is increased until alteration of voice can be confirmed with a voice test. Patients undergoing general anesthesia can also be tested by direct stimulation of the isolated vagus nerve. Utilizing visualization of the larynx and vocal cords via fiberoptic endoscopy, direct stimulation of the vagus nerve will produce a contraction of the left lateral wall of the larynx and tightening of the left vocal cord. Neither of these procedures produce any untoward effects for the patients. We have found these methods improve our ability to confirm correct placement of the stimulator with minimal increase in operative time (with Video).

Electric Stimulation Therapy↗

Casting stones.

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Child Abuse↗

Carbamazepine responsive epileptic oral motor and ocular motor apraxia.

We evaluated seven patients with oral motor apraxia and ocular motor apraxia. Apraxia in three patients (Group 1) with new-onset partial seizures and epileptiform discharges on EEG improved with carbamazepine. Four patients (Group 2) without seizures and nonepileptiform EEG findings had no change in apraxia after a trial of carbamazepine. Epileptic apraxia may precede clinical seizures and can respond to antiepileptic drugs.

Anticonvulsants↗

Improvement of epileptic seizure control with treatment of obstructive sleep apnoea.

Sleep deprivation increases the risk of recurrent seizures in epileptic patients. We identified 10 patients with recurrent seizures and sleep disruption related to obstructive sleep apnoea. Two patients were treated with positional therapy and the remaining eight patients were treated with continuous positive airway pressure. Three of the patients became seizure free and a fourth patient had a greater than 95% reduction in seizure frequency following only the initiation of therapy for the sleep apnoea. Three of these four patients responding to therapy, had a state-dependent seizure pattern. Two of the four responders did not exhibit the typical body habitus for obstructive sleep apnoea. Three additional patients improved in seizure frequency with change in anticonvulsant medication and treatment of the obstructive sleep apnoea. The remaining three patients had less than 50% reduction in seizure frequency with treatment of the obstructive sleep apnoea. These results indicate sleep disruption caused by sleep apnoea may increase the seizure frequency in some epileptic patients. Regardless of body habitus, epilepsy patients should be questioned carefully for a history of sleep disturbance and state dependence to their seizures. Treatment of sleep disorders in this population may lower the frequency of recurrent seizures.

Adult↗

Carbamazepine as a treatment for cataplexy.

Cataplexy is a common symptom associated with narcolepsy. We evaluated a 51-year-old female who developed symptoms of progressive daytime sleepiness with cataplexy. The diagnosis of narcolepsy was confirmed by overnight polysomnogram and was consistent with results of a multiple sleep latency study, and episodes of cataplexy were documented by video-EEG-EMG monitoring. Examination during a catapletic episode was significant for areflexia and paralysis. The frequency of cataplexy increased with fluoxetine, protriptyline and sertraline and the patient developed frank choreiform movements with protriptyline and sertraline. The cataplexy resolved following treatment with carbamazepine. We feel carbamazepine is a potential treatment for patients with refractory cataplexy.

Anticonvulsants↗

Sleeping on the job.

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Attitude of Health Personnel↗

Symptomatic cataplexy in pontomedullary lesions.

Cataplexy is a cardinal manifestation of the narcolepsy syndrome. Although symptomatic narcolepsy is well described, isolated cataplexy is extremely rare. We reviewed clinical and radiologic data in two patients with isolated symptomatic cataplexy and associated CNS disease. In an HLA-DR2-positive patient with chronic progressive MS, we confirmed cataplexy by observation of reported spells. MRI revealed diffuse white-matter lesions involving the medial medulla, pons, and subcortical white matter; protriptyline provided symptomatic relief. A second patient with a pontomedullary pilocytic astrocytoma developed infrequent but recurrent cataplectic attacks in association with sleep fragmentation due to nocturnal cough and nausea. MRI revealed an enhancing lesion involving the dorsal pons and medulla. Genetic predisposition and sleep fragmentation may precipitate symptomatic cataplexy in patients with structural pontomedullary lesions.

Adult↗

Möbius syndrome: evidence for a vascular etiology.

We report five infants with restricted lateral gaze, facial diplegia, feeding difficulty, and/or respiratory disorders without significant pulmonary disease. Viral studies were negative in all patients. Two children had radiologic findings that included brain-stem hypoplasia and symmetric calcification in the dorsal tectum at the junction of the midbrain and pons. Autopsy of one of these two children demonstrated capillary telangiectasia in the mesencephalon and pons. The other three children had normal computed tomographic (CT) scans. However, their autopsies revealed focal brain-stem necrosis with calcifications but without vascular malformation. We suggest that the capillary malformations in one of our patients directly resulted in a vascular-induced necrosis and the manifestation of Möbius sequence. The similarity of symmetric neuropathologic findings in the three other patients and the CT scan in the one surviving patient suggest focal hemodynamic changes restricted to the posterior circulation, indirectly supporting a vascular theory of embryopathogenesis.

Brain Diseases↗

Acute confusional migraine: case series and review of literature.

Acute confusional state (ACS) as a manifestation of migraine in the pediatric age group was initially described by Gascon and Barlow. The characteristics and natural history of acute confusional migraine (ACM) have been elaborated on by other authors. The diagnosis of ACM is, however, easily overlooked in clinical practice, due to its peculiar presentation. We describe three children who had acute onset of confusion. The differential diagnosis of acute confusional state and the clinical features and management of ACM are discussed.

Acute Disease↗