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Occult hemispherectomy: an unusual finding at autopsy
... The specimen is linked to autopsy number 217/1970 and is part of the non-public section of the forensic specimen collection of the Institute of Legal Medicine and Forensic Sciences in Berlin. Previous papers have described other interesting specimens held in this collection [1,2]. ...
Over the past 2 decades, advances in understanding the pathophysiology of spinal cord injury (SCI) have stimulated the recent emergence of several therapeutic strategies that are being examined in Phase I/II clinical trials. Ten randomized controlled trials examining methylprednisolone sodium succinate, tirilizad mesylate, monosialotetrahexosylganglioside, thyrotropin releasing hormone, gacyclidine, naloxone, and nimodipine have been completed. Although the primary outcomes in these trials were laregely negative, a secondary analysis of the North American Spinal Cord Injury Study II demonstrated that when administered within 8 hours of injury, methylprednisolone sodium succinate was associated with modest clinical benefits, which need to be weighed against potential complications. Thyrotropin releasing hormone (Phase II trial) and monosialotetrahexosylganglioside (Phase II and III trials) also showed some promise, but we are unaware of plans for future trials with these agents. These studies have, however, yielded many insights into the conduct of clinical trials for SCI. Several current or planned clinical trials are exploring interventions such as early surgical decompression (Surgical Treatment of Acute Spinal Cord Injury Study) and electrical field stimulation, neuroprotective strategies such as riluzole and minocycline, the inactivation of myelin inhibition by blocking Nogo and Rho, and the transplantation of various cellular substrates into the injured cord. Unfortunately, some experimental and poorly characterized SCI therapies are being offered outside a formal investigational structure, which will yield findings of limited scientific value and risk harm to patients with SCI who are understandably desperate for any intervention that might improve their function. Taken together, recent advances suggest that optimism for patients and clinicians alike is justified, as there is real hope that several safe and effective therapies for SCI may become available over the next decade.
The surgical treatment of intractable epilepsy has evolved as new technical innovations have been made. Hemispherotomy techniques have been developed to replace hemispherectomy in order to reduce the complication rates while maintaining good seizure control.
Disconnective procedures are based on the interruption of the epileptic network rather than the removal of the epileptogenic zone. They can be applied to hemispheric pathologies, leading to hemispherotomy, but they can also be applied to posterior quadrant epilepsies, or hypothalamic hamartomas.
In this paper, the authors review the literature, present an overview of the historical background, and discuss the different techniques along with their outcomes and complications.
Today, hemispherectomy is a well-established procedure for the treatment of some sorts of catastrophic epilepsies. This, however, has not always been the case. The technique was developed to deal with brain tumors; however, the initial results were not remarkable. Moreover, when its morbidity became evident, it was almost abandoned. Had it not been for a shift in its use, with a huge increase in operations on patients with infantile hemiplegia, this surgery would certainly have disappeared. This paper focuses on the facts that surrounded these early years.
Anatomical hemispherectomy has been used for the treatment of seizures since 1938. However, it was almost abandoned in the 1960s after reports of postoperative fatalities caused by hydrocephalus, hemosiderosis, and trivial head traumas. Despite serious complications, the remarkable improvement of patients encouraged authors to carry out modifications on anatomical hemispherectomy in order to lessen its morbidity while preserving its efficacy. The effort to improve the technique generated several original procedures. This paper reviews current techniques of hemispherectomy and proposes a classification scheme based on their surgical characteristics. Techniques of hemispherectomy were sorted into two major groups: (1) those that remove completely the cortex from the hemisphere and (2) those that associate partial cortical removal and disconnection. Group 1 was subdivided into two subgroups based on the integrity of the ventricular cavity and group 2 was subdivided into three subgroups depending on the amount and location of the corticectomy. Grouping similar techniques may allow a better understanding of the distinctive features of each one and creates the possibility of comparing data from different authors.
In the gradual evolution of resections of frontal, temporal and occipital lobes for the cure or attempted cure of invasive and otherwise incurable cerebral tumors, it has been found that the right temporal lobe, either frontal lobe or either occipital lobe (the left posterior to the supramarginal gyrus) could be completely removed without apparent mental impairment. It therefore seemed a logical expectation that the right cerebral hemisphere might be removed with little, if any, change in the mentality of a right handed person. At times patients already paralyzed on the left side from the inroads of cerebral neoplasms and without any prospect of its correction are eager to live, even at the price of permanent loss of function on that side of the body, if only the mind remains undisturbed. Five times during the past five years patients in this condition have urged every effort to save their lives, regardless
The history of surgical treatment for hemispheric epilepsy is rich with colorful twists and turns. The authors trace the evolution of the surgical treatment of hemispheric epilepsy from radical anatomic resections to current less invasive disconnection procedures. Anatomic hemispherectomy (AH) was first described by Dandy in 1928 as a treatment for gliomas. The first report of this technique to control seizures was by McKenzie in 1938. AH gained wide popularity but began to fall out of favor after the description of superficial cerebral hemosiderosis in 1966. To reduce the morbidity and mortality associated with AH, Rasmussen introduced functional hemispherectomy in 1974. The technique of hemispherotomy was introduced in the 1990s to minimize the extent of brain removal while maximizing the white matter disconnections. Thus, surgery for hemispheric epilepsy has undergone dramatic transformation since the technique was first introduced. Less invasive techniques have been developed to reduce surgical morbidity. Although optimal seizure control is best achieved with radical AH, the newer less invasive disconnection techniques appear to achieve near-comparable postoperative seizure control with a significantly lower rate of complications.
Hemispherotomy generally is performed in hemiparetic patients with severe, intractable epilepsy arising from one cerebral hemisphere. In this study, the authors evaluate the efficacy of hemispherotomy and present an analysis of the factors influencing seizure recurrence following the operation.
The authors performed a retrospective review of 49 patients (ages 0.2-20.5 years) who underwent functional hemispherotomy at their institution. The first 14 cases were traditional functional hemispherotomies, and included temporal lobectomy, while the latter 35 were performed using a modified periinsular technique that the authors adopted in 2003.
Thirty-eight of the 49 patients (77.6%) were seizure free at the termination of the study (mean follow-up 28.6 months). Of the 11 patients who were not seizure free, all had significant improvement in seizure frequency, with 6 patients (12.2%) achieving Engel Class II outcome and 5 patients (10.2%) achieving Engel Class III. There were no cases of Engel Class IV outcome. The effect of hemispherotomy was durable over time with no significant change in Engel class over the postoperative follow-up period. There was no statistical difference in outcome between surgery types. Analysis of factors contributing to seizure recurrence after hemispherotomy revealed no statistically significant predictors of treatment failure, although bilateral electrographic abnormalities on the preoperative electroencephalogram demonstrated a trend toward a worse outcome.
In the present study, hemispherotomy resulted in freedom from seizures in nearly 78% of patients; worthwhile improvement was demonstrated in all patients. The seizure reduction observed after hemispherotomy was durable over time, with only rare late failure. Bilateral electrographic abnormalities may be predictive of posthemispherotomy recurrent seizures.
To study the outcome after hemispherectomy (HP) in a homogeneous adult patient population with refractory hemispheric epilepsy.
Fourteen adult patients submitted to HP were studied. Patients had to be at least 18 years old, and have refractory epilepsy, clearly focal lateralized seizures and unilateral porencephalus consistent with early middle cerebral artery infarct on magnetic resonance imaging (MRI). All patients were submitted to functional hemispherectomy. We analyzed age of seizure onset, age by the time of surgery, gender, seizure type and frequency, interictal and ictal electroencephalography (EEG) findings, MRI and IQ scores preoperatively; seizure frequency, drug regimen, and IQ outcome were studied postoperatively.
Mean follow-up was 64 months. All patients had frequent daily seizures preoperatively. All patients had unilateral simple partial motor seizures (SPS); 11 patients had secondarily generalized tonic-clonic (GTC) seizures and five patients had complex partial seizures (CPS), preoperatively. All patients had hemiplegia and hemianopsia. Twelve patients had unilateral EEG findings, and in two epileptic discharges were seen exclusively over the apparently normal hemisphere. Twelve patients were seizure-free after surgery and two patients had at least 90% improvement in seizure frequency. Pre- and postoperative mean general IQ was 84 and 88, respectively. Five of the twelve Engel I patients were receiving no drugs at last follow-up. There was no mortality or major morbidity.
Our results suggest that well-selected adult patients might also get good results after HP. Although good results were obtained in our adult series, the same procedure yielded a much more striking result if performed earlier in life.
Anatomical hemispherectomy is frequently employed in the surgical management of pediatric patients with medically refractory epilepsy.
In this chapter, we review the historical evolution of this surgical procedure, outline the indications and the criteria for selecting surgical candidates and describe the important pre-operative evaluation of the surgical candidates.
We provide a detailed description of our surgical technique, anesthesiological considerations, and post-operative care plan. Ultimately we analyze the most common complications associated with this procedure.
Anatomical hemispherectomy performed in carefully selected pediatric patients with medically intractable epilepsy can be a safe and efficacious surgical procedure.
Hemispherectomy for intractable unihemispheric epilepsy (IUE) has long been established in pediatric patients. This study reports the first series examining hemispherectomy exclusively in adult patients (>18 years old). Nine adults with IUE underwent hemispherectomy at the University of Minnesota. All patients had unilateral hemiplegia and visual field loss. Seven patients (77.8%) were Engel class I/II at last follow-up. Five (83.3%) of the six patients with >30 years of follow-up were seizure free. No surgery-related mortality, hydrocephalus, or superficial cerebral hemosiderosis occurred. Hemispherectomy is an effective procedure in appropriately selected adult patients, resulting in excellent long-term seizure control and no mortality.
Fig. 3 Oblique intracranial view of specimen 172 CBF demonstrating the location were surgery took place. The falx cerebri, and the areas were the right cerebral hemisphere was disconnected during surgery, are visible 124
- Je Leestma
- Forensic
Leestma JE. Forensic neuropathology. New York: Raven Press; 1988. Fig. 3 Oblique intracranial view of specimen 172 CBF demonstrating the location were surgery took place. The falx cerebri, and the areas were the right cerebral hemisphere was disconnected during surgery, are visible 124 Forensic Sci Med Pathol (2013) 9:122–124 123
Germany e-mail: rene.gapert@ucd.ie R
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