Adam Hartman | Johns Hopkins University (original) (raw)

Papers by Adam Hartman

Current Neurology and Neuroscience Reports, 2007

The field of epilepsy has contributed significantly to localization of neurologic function, parti... more The field of epilepsy has contributed significantly to localization of neurologic function, particularly in the neocortex. Methodologies such as cortical stimulation, positron emission tomography, functional MRI, trans-cranial magnetic stimulation, surgical resection, and magnetoencephalography have been used successfully in patients with epilepsy to locate specific functions, primarily for the purpose of defining eloquent cortex before surgical resections. The left hemisphere serves language-related functions and verbal memory in most people, whereas the right hemisphere serves some language function in addition to perceiving most components of music and other forms of nonverbal material. Both hemispheres cooperate in understanding spatial relationships. Studies in patients with developmental abnormalities have enriched our understanding of localization of function within the cortex. Future studies may help us understand the sequence in which specific regions are activated during specific tasks and determine which regions are necessary for tasks and which are supplementary. The ability to predict preoperatively the effect of removal of specific tissues would benefit surgical planning for all patients who undergo cortical resections, including those with epilepsy.

Seminars in Pediatric Neurology, 2006

Patients with neurophakomatoses were not always considered to be candidates for surgical resectio... more Patients with neurophakomatoses were not always considered to be candidates for surgical resection of epileptogenic foci, given the multifocal nature of their disease. Advances in imaging, monitoring, and surgical technique have allowed the identification of particularly active areas of cortex that may provide these patients with better seizure control than medications alone. Options for surgical resection range from focal resections (in those with localized disease) to hemispherectomies (in those with hemispheric involvement).

Epilepsy Currents, 2014

Rasmussen syndrome affects previously normal people and forever changes their lives and the lives... more Rasmussen syndrome affects previously normal people and forever changes their lives and the lives of their families. Although understood as a probable autoimmune condition, medical treatment remains limited and surgery remains the only cure, although with inevitable functional consequences. Difficulties remain in deciding on the optimal timing of surgery. Here, we review data available to aid clinicians faced with making the decision of when to recommend hemispherectomy. Not all patients have rapidly progressive disease, however, and such patients may benefit from immunomodulatory treatment. Thus, a patient's clinical course requires careful evaluation in order to identify those who would benefit most from early surgery.

Hemidecortication and Intractable Epilepsy Adam L Hartman, James Frazier, and George I. Jallo Hem... more Hemidecortication and Intractable Epilepsy Adam L Hartman, James Frazier, and George I. Jallo Hemispherectomy in the treatment of intractable epilepsy was first reported by Krynauw in 1950 as an operative in-tervention for patients with infantile ... In Engel J, Pedley TA, eds. ...

Mesial temporal sclerosis is the most frequent cause of drug-resistant temporal lobe epilepsy but... more Mesial temporal sclerosis is the most frequent cause of drug-resistant temporal lobe epilepsy but has a satisfactory response to surgery, and is considered infrequent in children. To evaluate the clinical, electrographic and radiological spectrum of the disease in children. Retrospective study by review of charts of 44 children with a diagnosis of mesial temporal sclerosis on magnetic resonance imaging, attended at the "Hospital das Clínicas" of the University of São Paulo Faculty of Medicine. Febrile seizure was identified in the history of 54% of the patients. Injuries at the left side predominated in patients with schooling difficulties (p=0.049), in those with first seizures between six months and five years (p=0.021) and in those with complex febrile seizure (p=0.032). Thirteen patients were submitted to surgery and of these, eight remained without seizures. Febrile seizure may be related in a more direct way to the presence of left-side mesial temporal sclerosis.

Neuropediatrics, 2014

Christianson syndrome (CS) is caused by mutations in SLC9A6 and is characterized by severe intell... more Christianson syndrome (CS) is caused by mutations in SLC9A6 and is characterized by severe intellectual disability, absent speech, microcephaly, ataxia, seizures, and behavioral abnormalities. The clinical phenotypes of CS and Angelman syndrome (AS) are similar. Differentiation between CS and AS is important in terms of genetic counseling. We report on two children with CS and confirmed mutations in SLC9A6 focusing on neuroimaging findings and review the available literature. Cerebellar atrophy (CA) occurs in approximately 60% of the patients with CS and develops after the age of 12 months. Hyperintense signal of the cerebellar cortex (CbC) is less common, and may be diffuse, patchy, or involve only the inferior part of the cerebellum and is best seen on coronal fluid attenuation inversion recovery images. CA and CbC-hyperintensity are not neuroimaging features of AS. In a child with the phenotype of AS, CA and/or CbC-hyperintensity are rather specific for CS and should prioritize s...

Seminars in Pediatric Neurology, 2011

When a child on anticonvulsant medications continues to have seizures, what other options should ... more When a child on anticonvulsant medications continues to have seizures, what other options should be considered? Over the past 100 years, dietary therapies for the treatment of intractable epilepsy have become more widely recognized, and their use has continued to expand throughout the world. An increasing number of studies has shown efficacy of these metabolic treatments in improving seizure control. Currently, 4 types of dietary therapy are available in the clinic: the classic long chain fatty acid "ketogenic" diet, the medium chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. These therapies should be considered earlier in the treatment of intractable epilepsy because they offer a different approach to treatment that has proven efficacious, tolerable, and cost-effective.

Pediatrics, 2007

The ketogenic diet, a high fat, adequate protein, low carbohydrate diet, has, during the past dec... more The ketogenic diet, a high fat, adequate protein, low carbohydrate diet, has, during the past decade, had a resurgence of interest for the treatment of difficult-to-control seizures in children. This review traces its history, reviews its uses and side effects, and discusses possible alternatives and the diet's possible mechanisms of action. Finally, this review looks toward possible future uses of the ketogenic diet for conditions other than epilepsy.

PLoS ONE, 2012

Objective: The mammalian target of rapamycin (mTOR) pathway integrates signals from different nut... more Objective: The mammalian target of rapamycin (mTOR) pathway integrates signals from different nutrient sources, including amino acids and glucose. Compounds that inhibit mTOR kinase activity such as rapamycin and everolimus can suppress seizures in some chronic animal models and in patients with tuberous sclerosis. However, it is not known whether mTOR inhibitors exert acute anticonvulsant effects in addition to their longer term antiepileptogenic effects. To gain insights into how rapamycin suppresses seizures, we investigated the anticonvulsant activity of rapamycin using acute seizure tests in mice.

Pediatric Neurology, 2014

Neurology, 2014

To describe a case series of adult patients in the intensive care unit in super-refractory status... more To describe a case series of adult patients in the intensive care unit in super-refractory status epilepticus (SRSE; refractory status lasting 24 hours or more despite appropriate anesthetic treatment) who received treatment with the ketogenic diet (KD). We performed a retrospective case review at 4 medical centers of adult patients with SRSE treated with the KD. Data collected included demographic features, clinical presentation, diagnosis, EEG data, anticonvulsant treatment, and timing and duration of the KD. Primary outcome measures were resolution of status epilepticus (SE) after initiation of KD and ability to wean from anesthetic agents. Ten adult patients at 4 medical centers were started on the KD for SRSE. The median age was 33 years (interquartile range [IQR] 21), 4 patients (40%) were male, and 7 (70%) had encephalitis. The median duration of SE before initiation of KD was 21.5 days (IQR 28) and the median number of antiepileptic medications used before initiation of KD was 7 (IQR 7). Ninety percent of patients achieved ketosis, and SE ceased in all patients achieving ketosis in a median of 3 days (IQR 8). Three patients had minor complications of the KD including transient acidosis and hypertriglyceridemia and 2 patients ultimately died of causes unrelated to the KD. We describe treatment of critically ill adult patients with SRSE with the KD, with 90% of patients achieving resolution of SE. Prospective trials are warranted to examine the efficacy of the KD in adults with refractory SE. This study provides Class IV evidence that for intensive care unit patients with refractory SE, a KD leads to resolution of the SE.

Current Neurology and Neuroscience Reports, 2007

The field of epilepsy has contributed significantly to localization of neurologic function, parti... more The field of epilepsy has contributed significantly to localization of neurologic function, particularly in the neocortex. Methodologies such as cortical stimulation, positron emission tomography, functional MRI, trans-cranial magnetic stimulation, surgical resection, and magnetoencephalography have been used successfully in patients with epilepsy to locate specific functions, primarily for the purpose of defining eloquent cortex before surgical resections. The left hemisphere serves language-related functions and verbal memory in most people, whereas the right hemisphere serves some language function in addition to perceiving most components of music and other forms of nonverbal material. Both hemispheres cooperate in understanding spatial relationships. Studies in patients with developmental abnormalities have enriched our understanding of localization of function within the cortex. Future studies may help us understand the sequence in which specific regions are activated during specific tasks and determine which regions are necessary for tasks and which are supplementary. The ability to predict preoperatively the effect of removal of specific tissues would benefit surgical planning for all patients who undergo cortical resections, including those with epilepsy.

Seminars in Pediatric Neurology, 2006

Patients with neurophakomatoses were not always considered to be candidates for surgical resectio... more Patients with neurophakomatoses were not always considered to be candidates for surgical resection of epileptogenic foci, given the multifocal nature of their disease. Advances in imaging, monitoring, and surgical technique have allowed the identification of particularly active areas of cortex that may provide these patients with better seizure control than medications alone. Options for surgical resection range from focal resections (in those with localized disease) to hemispherectomies (in those with hemispheric involvement).

Epilepsy Currents, 2014

Rasmussen syndrome affects previously normal people and forever changes their lives and the lives... more Rasmussen syndrome affects previously normal people and forever changes their lives and the lives of their families. Although understood as a probable autoimmune condition, medical treatment remains limited and surgery remains the only cure, although with inevitable functional consequences. Difficulties remain in deciding on the optimal timing of surgery. Here, we review data available to aid clinicians faced with making the decision of when to recommend hemispherectomy. Not all patients have rapidly progressive disease, however, and such patients may benefit from immunomodulatory treatment. Thus, a patient's clinical course requires careful evaluation in order to identify those who would benefit most from early surgery.

Hemidecortication and Intractable Epilepsy Adam L Hartman, James Frazier, and George I. Jallo Hem... more Hemidecortication and Intractable Epilepsy Adam L Hartman, James Frazier, and George I. Jallo Hemispherectomy in the treatment of intractable epilepsy was first reported by Krynauw in 1950 as an operative in-tervention for patients with infantile ... In Engel J, Pedley TA, eds. ...

Mesial temporal sclerosis is the most frequent cause of drug-resistant temporal lobe epilepsy but... more Mesial temporal sclerosis is the most frequent cause of drug-resistant temporal lobe epilepsy but has a satisfactory response to surgery, and is considered infrequent in children. To evaluate the clinical, electrographic and radiological spectrum of the disease in children. Retrospective study by review of charts of 44 children with a diagnosis of mesial temporal sclerosis on magnetic resonance imaging, attended at the "Hospital das Clínicas" of the University of São Paulo Faculty of Medicine. Febrile seizure was identified in the history of 54% of the patients. Injuries at the left side predominated in patients with schooling difficulties (p=0.049), in those with first seizures between six months and five years (p=0.021) and in those with complex febrile seizure (p=0.032). Thirteen patients were submitted to surgery and of these, eight remained without seizures. Febrile seizure may be related in a more direct way to the presence of left-side mesial temporal sclerosis.

Neuropediatrics, 2014

Christianson syndrome (CS) is caused by mutations in SLC9A6 and is characterized by severe intell... more Christianson syndrome (CS) is caused by mutations in SLC9A6 and is characterized by severe intellectual disability, absent speech, microcephaly, ataxia, seizures, and behavioral abnormalities. The clinical phenotypes of CS and Angelman syndrome (AS) are similar. Differentiation between CS and AS is important in terms of genetic counseling. We report on two children with CS and confirmed mutations in SLC9A6 focusing on neuroimaging findings and review the available literature. Cerebellar atrophy (CA) occurs in approximately 60% of the patients with CS and develops after the age of 12 months. Hyperintense signal of the cerebellar cortex (CbC) is less common, and may be diffuse, patchy, or involve only the inferior part of the cerebellum and is best seen on coronal fluid attenuation inversion recovery images. CA and CbC-hyperintensity are not neuroimaging features of AS. In a child with the phenotype of AS, CA and/or CbC-hyperintensity are rather specific for CS and should prioritize s...

Seminars in Pediatric Neurology, 2011

When a child on anticonvulsant medications continues to have seizures, what other options should ... more When a child on anticonvulsant medications continues to have seizures, what other options should be considered? Over the past 100 years, dietary therapies for the treatment of intractable epilepsy have become more widely recognized, and their use has continued to expand throughout the world. An increasing number of studies has shown efficacy of these metabolic treatments in improving seizure control. Currently, 4 types of dietary therapy are available in the clinic: the classic long chain fatty acid "ketogenic" diet, the medium chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. These therapies should be considered earlier in the treatment of intractable epilepsy because they offer a different approach to treatment that has proven efficacious, tolerable, and cost-effective.

Pediatrics, 2007

The ketogenic diet, a high fat, adequate protein, low carbohydrate diet, has, during the past dec... more The ketogenic diet, a high fat, adequate protein, low carbohydrate diet, has, during the past decade, had a resurgence of interest for the treatment of difficult-to-control seizures in children. This review traces its history, reviews its uses and side effects, and discusses possible alternatives and the diet's possible mechanisms of action. Finally, this review looks toward possible future uses of the ketogenic diet for conditions other than epilepsy.

PLoS ONE, 2012

Objective: The mammalian target of rapamycin (mTOR) pathway integrates signals from different nut... more Objective: The mammalian target of rapamycin (mTOR) pathway integrates signals from different nutrient sources, including amino acids and glucose. Compounds that inhibit mTOR kinase activity such as rapamycin and everolimus can suppress seizures in some chronic animal models and in patients with tuberous sclerosis. However, it is not known whether mTOR inhibitors exert acute anticonvulsant effects in addition to their longer term antiepileptogenic effects. To gain insights into how rapamycin suppresses seizures, we investigated the anticonvulsant activity of rapamycin using acute seizure tests in mice.

Pediatric Neurology, 2014

Neurology, 2014

To describe a case series of adult patients in the intensive care unit in super-refractory status... more To describe a case series of adult patients in the intensive care unit in super-refractory status epilepticus (SRSE; refractory status lasting 24 hours or more despite appropriate anesthetic treatment) who received treatment with the ketogenic diet (KD). We performed a retrospective case review at 4 medical centers of adult patients with SRSE treated with the KD. Data collected included demographic features, clinical presentation, diagnosis, EEG data, anticonvulsant treatment, and timing and duration of the KD. Primary outcome measures were resolution of status epilepticus (SE) after initiation of KD and ability to wean from anesthetic agents. Ten adult patients at 4 medical centers were started on the KD for SRSE. The median age was 33 years (interquartile range [IQR] 21), 4 patients (40%) were male, and 7 (70%) had encephalitis. The median duration of SE before initiation of KD was 21.5 days (IQR 28) and the median number of antiepileptic medications used before initiation of KD was 7 (IQR 7). Ninety percent of patients achieved ketosis, and SE ceased in all patients achieving ketosis in a median of 3 days (IQR 8). Three patients had minor complications of the KD including transient acidosis and hypertriglyceridemia and 2 patients ultimately died of causes unrelated to the KD. We describe treatment of critically ill adult patients with SRSE with the KD, with 90% of patients achieving resolution of SE. Prospective trials are warranted to examine the efficacy of the KD in adults with refractory SE. This study provides Class IV evidence that for intensive care unit patients with refractory SE, a KD leads to resolution of the SE.