Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics.

BACKGROUND: Chiari malformation type 1 (CM1) is a common congenital anomaly of the craniocervical junction. CM1 is reported to run a usually benign course and patients typically experience no symptoms or chronic, slowly progressive symptoms. However, recent reports indicate that a subset of patients with CM1 may present with acute deterioration and sudden unexpected death (SUD). We report a case of SUD during sleep in a young man with CM1, which we believe was related to the administration of common and therapeutic doses of narcotic analgesics for the management of pain. We will clarify the pathophysiology of acute deterioration and SUD in CM1 and the possibility that the adverse effects of opiate analgesics likely were the leading cause of death in our patient. CASE DESCRIPTION: In this review, we present a 29-year-old male with worsening headache secondary to previously diagnosed CM1. The patient died suddenly and unexpectedly after administration of common and therapeutic doses of narcotic analgesics for the management of pain. CONCLUSION: The mechanism(s) of acute neurological deterioration and sudden death in patients with CM1 remains poorly understood. We believe the rapid fatal deterioration in our patient following administration of opioids suggests that this category of medication may cause sudden unexpected "neurogenic" cardiac death in CM1 patients by inducing sleep-related breathing difficulties and associated hypercapnia. Hypercapnia by further increasing intracranial pressure can result in a sudden pressure-induced decompensation of the cardiopulmonary control centers in the brain stem and cause instantaneous cardiorespiratory arrest.

A diagnostic and management dilemma: combined paraneoplastic myasthenia gravis and Lambert-Eaton myasthenic syndrome presenting as acute respiratory failure.

BACKGROUND: Neuromuscular junction disorders are usually categorized as either presynaptic or postsynaptic. The most frequently encountered disorder of the postsynaptic neuromuscular junction is acquired myasthenia gravis. Lambert-Eaton myasthenic syndrome is a well-known prototype of the presynaptic autoimmune disorders of neuromuscular transmission. These major disorders of neuromuscular transmission are relatively common and distinctly recognized, but co-occurrence of these disorders (overlap myasthenic syndrome) is rare and has so far attracted little attention. REVIEW SUMMARY: This report describes a patient with acquired myasthenia gravis and immunologic coexistence of Lambert-Eaton myasthenic syndrome (overlap myasthenic syndrome) in association with abdominal/uterine leiomyosarcoma. The patient presented with acute respiratory failure, making identification and management of her illness challenging. A general overview of the complexities associated with overlap between myasthenia gravis and Lambert-Eaton myasthenic syndrome is provided and this patient's complicated clinical course and response to therapy are discussed. CONCLUSION: To our knowledge, this is the first report of overlap myasthenic syndrome in conjunction with abdominal leiomyosarcoma. The immunologic coexistence of acquired myasthenia gravis and Lambert-Eaton myasthenic syndrome in a patient with a malignant smooth-muscle tumor is intriguing and suggests that a common paraneoplastic process targeting 2 different onconeural antigens was the underlying pathogenic mechanism in this patient.

Burst fracture of the first lumbar vertebra and conus-cauda syndrome complicating a single convulsive seizure: a challenge of diagnosis in the Emergency Department.

Fractures of the thoracic and lumbar vertebrae as a direct consequence of generalized epileptic convulsions are the most common non-traumatic type of fracture complicating epileptic seizures. The majority of these fractures are compression fractures that occur with minimal symptoms and virtually no permanent neurological sequela. Nevertheless, muscle contractions generated during generalized motor seizures can result in severe axial skeletal trauma and grave neurological complications. We describe the case of a 35-year-old man who suffered a burst fracture of the first lumbar vertebral body and acute conus medullaris-cauda equina syndrome as a direct consequence of a single grand mal seizure. The aim of this report is to draw attention to this serious complication of generalized convulsive seizures and alert readers to epilepsy-related vertebral fractures. Diagnosis and management of acute cauda equina-conus medullaris syndrome caused by lumbar fracture are reviewed.

Surgical management of hydrocephalic dementia in Paget's disease of bone: the 6-year outcome of ventriculo-peritoneal shunting.

Paget's disease of bone is a chronic progressive skeletal disorder usually occurring in the long bones and skull of older adults and elderly persons. In the skull, softening of the skull base may lead to basilar impression and consequently obstruction of the cerebrospinal fluid through the basilar cisterns, resulting ventricular enlargement in association with gait difficulties, incontinence and dementia: a syndrome resembling normal pressure hydrocephalus. The optimal management of hydrocephalus associated with Paget's disease of the skull is not well documented and is still debated. We report a patient with hydrocephalic dementia linked to Paget's disease of the skull who showed marked sustained improvement in her neurological condition after ventriculo-peritoneal shunt insertion. We have now followed this patient for 6 years. Our experience supports the view that ventricular shunting is the procedure of choice for treating hydrocephalus linked to the Paget's disease of bone and is best carried out in the early stages of the illness.