Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproa... more Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproate was given for 3 days before surgery. At craniotomy, sections of brain and samples of cerebrospinal fluid (CSF) and plasma were obtained and assayed for sodium valproate concentrations. Brain levels were 6.8 to 27.9% of plasma concentrations, and CSF levels were 7.6 to 25.0% of plasma levels. There was a significant correlation between brain and CSF levels and plasma on Spearman ranking. These results confirm that valproate is not selectively concentrated in brain fractions, and the concentration in brain is related to the free anticonvulsant level in plasma.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure ("hereditary neuropathy with liability to pressure palsies" (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakness. With this case we alert surgeons and physical therapists that patients with EDS can have not only a complicated course after TSA, but rare concurrent conditions that can further increase the propensity of neurological injuries that result in compromised shoulder function.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure ("hereditary neuropathy with liability to pressure palsies" (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoul...
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure (“hereditary neuropathy with liability to pressure palsies” (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakne...
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure (“hereditary neuropathy with liability to pressure palsies” (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakne...
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproa... more Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproate was given for 3 days before surgery. At craniotomy, sections of brain and samples of cerebrospinal fluid (CSF) and plasma were obtained and assayed for sodium valproate concentrations. Brain levels were 6.8 to 27.9% of plasma concentrations, and CSF levels were 7.6 to 25.0% of plasma levels. There was a significant correlation between brain and CSF levels and plasma on Spearman ranking. These results confirm that valproate is not selectively concentrated in brain fractions, and the concentration in brain is related to the free anticonvulsant level in plasma.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure ("hereditary neuropathy with liability to pressure palsies" (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakness. With this case we alert surgeons and physical therapists that patients with EDS can have not only a complicated course after TSA, but rare concurrent conditions that can further increase the propensity of neurological injuries that result in compromised shoulder function.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure ("hereditary neuropathy with liability to pressure palsies" (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoul...
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure (“hereditary neuropathy with liability to pressure palsies” (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakne...
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
This is a unique case of a female patient with features of classical and hypermobile types of Ehl... more This is a unique case of a female patient with features of classical and hypermobile types of Ehlers-Danlos syndrome (EDS) who developed complex scapular winging from spinal accessory and long thoracic neuropathies. These neurological problems became manifest after an uncomplicated total shoulder arthroplasty (TSA). The patient had a complex postoperative course with extensive work-up in addition to revision shoulder surgery and manipulations to treat shoulder stiffness. It was eventually suspected that the periscapular nerve impairments occurred during physical therapy sessions after her TSA. This interpretation was further supported by genetic evidence that, in addition to EDS, the patient had an unrecognized genetic propensity for nerve palsies from stretch or pressure (“hereditary neuropathy with liability to pressure palsies” (HNPP)). By two years after the TSA the neuropathies had only partially improved, leaving the patient with persistent scapular winging and shoulder weakne...
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma ... more Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for screening. Using drug incubation in chick-embryo hepatocyte culture, we found that porphyrin was increased by carbamazepine, clonazepam, and valproate. These agents should probably be avoided or used with caution in porphyric patients. Alternative approaches to acute porphyric attacks with seizures are discussed.
The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to b... more The spinal form of MS is a clinical conundrum, the solution of which may yield many answers; to be certain that it is MS and not another disease causing the myelopathy is often difficult. We evaluated 20 patients with myelopathy of undetermined etiology (clinical findings limited to the spinal cord) using T2-weighted cranial magnetic resonance imaging (T2 MRI) and cranial computerized tomography (CT). Some patients were also studied with flash visual evoked responses (FVER) and spinal fluid analysis for myelin basic protein (MBP) and oligoclonal banding (OCB). Thirteen patients had T2 MRIs consistent with demyelinating disease (two or more areas of increased signal intensity, of appropriate size, in periventricular/subcortical white matter), while only one CT showed focal lesions. FVER were abnormal in 8 of 15 patients tested; spinal fluid OCB was present in 12 of 16 patients tested, only 1 of whom had elevated MBP. T2 MRI showed lesions typical of demyelination in the majority of study patients, was much more sensitive than CT, and was well correlated with evidence of demyelination by other tests. Although the specificity of T2 MRI in MS is unknown, it may be very high in this clinical setting.
Uploads
Papers by Robert Miska