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Volume 3, No 4 - Fall 2006

Volume 3, No 4 - Fall 2006

Table of Contents

Malformations of Cortical Development and Epilepsy, Part 1: Diagnosis and Classification Scheme Diagnosis Update
Malformations of cortical development (MCDs) are a common cause of epilepsy, although seizures are not always the most prominent neurologic manifestation of these disorders. In localization-related epilepsy, certain features should create a strong suspicion that an MCD is the underlying cause; these include developmental delay and static focal neurologic deficits, a family history of developmental delay or epilepsy, frequent seizures from onset, and episodes of focal status epilepticus. MCDs can be classified according to a number of different criteria emphasizing clinical phenotype, imaging findings, pathology, or genetic defects. The overall classification of MCDs is based on the 3 fundamental events of cortical formation: 1) proliferation of neurons and glia in the ventricular and subventricular zones; 2) multidirectional migration of immature but postmitotic neurons to the developing cerebral cortex; and 3) cortical organization. Among the most common and distinct syndromes and entities affecting patients with MCDs and epilepsy are focal cortical dysplasia, hemimegalencephaly, tuberous sclerosis, classical lissencephaly, periventricular nodular heterotopia, focal subcortical heterotopia, polymicrogyria, and schizencephaly, all of which are discussed herein. [Rev Neurol Dis. 2006;3(4):151-162]
Surgery for Intracerebral Hematoma: The Search for the Elusive Right Candidate Management Update
The value of surgery for patients with intracerebral hemorrhage (ICH) remains a topic of debate. Although several studies have been unable to prove the benefit of surgical intervention of ICH, there is available evidence to suggest that some patients may experience favorable outcome with surgery. Identifying optimal candidates and the timing of surgery for the treatment of ICH are crucial issues. Studies have explored the value of early and ultra-early surgical intervention, as well as the role of stereotactic hematoma evacuation. The International Surgical Trial in Intracerebral Hemorrhage suggested that favorable surgical outcome was more likely for patients with superficial hematomas, and patients who underwent craniotomy in the trial seemed to fare better than those treated with other surgical techniques. A retrospective study of patients with brain tissue shift has provided evidence that surgery may benefit some deteriorating patients; treatment decisions in this patient population should be based on case-bycase assessments of the likelihood of recovery after evacuation. It is critical that therapeutic alternatives for patients with ICH continue to be explored. [Rev Neurol Dis. 2006;3(4):163-172]
Neurologic Complications of Primary Systemic Amyloidosis Diagnosis and Management Update
Amyloid is a botanical term that denotes a waxy, amorphous, eosinophilic material and is used to describe similar pathological findings in humans. The deposits in primary systemic amyloidosis are derived from monoclonal serum proteins in plasma cell dyscrasia, which are degraded locally in tissues and deposited in insoluble sheets that damage organs. The cause of organ damage in amyloidosis is unclear but most likely due to the direct toxic effects of amyloid. Symptoms of the disorder include neuropathy, myopathy, and cardiac or renal insufficiency; there is often multiple-organ involvement. Congestive heart failure and nephrotic syndrome are predominant causes of death. Differential difficulties exist in diagnosing the disorder, and familial amyloid polyneuropathy directly mimics the disease. Diagnostic tools include electromyography, laboratory testing for abnormalities in serum and urine, and histological investigation of appropriate tissue. The median survival of patients is 2 years. However, melphalan and prednisone treatment for at least 1 year has resulted in increased survival rates. There have also been reports of benefit from high-dose chemotherapy followed by peripheral blood stem cell transplantation. Without early therapy, however, the disease has a dismal prognosis, and peripheral neuropathy usually persists or worsens despite therapy and improvement in other organs. [Rev Neurol Dis. 2006;3(4):173-181]
Botulism: The Challenge of Diagnosis and Treatment Diagnosis and Management Update
Botulism is a rare paralytic disease caused by a neurotoxin produced from the spore-forming bacterium Clostridium botulinum and in rare cases Clostridium butyricum and Clostridium baratii. Botulism has 4 naturally occurring syndromes: foodborne, wound, infant botulism, and adult intestinal toxemia. Inhalational botulism could result from aerosolization of botulinum toxin, and iatrogenic botulism can result from injection of toxin. All of these produce the same clinical syndrome of symmetrical cranial nerve palsies followed by descending, symmetric flaccid paralysis of voluntary muscles, which may progress to respiratory compromise and death. Treatment includes meticulous intensive care that includes mechanical ventilation, when necessary, and administration of antitoxin. [Rev Neurol Dis. 2006;3(4):182-189]
Advances in the Diagnosis, Treatment, and Understanding of Parkinson’s Disease and Parkinsonism Meeting Review
Highlights from the Twentieth Annual Symposium on the Etiology, Pathogenesis, and Treatment of Parkinson’s Disease and Other Movement Disorders October 8, 2006 Chicago, IL [Rev Neurol Dis. 2006;3(4):191-194]