Weak and numb feet in a man with knobby hands.
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Aged↗
Biomedical subjects
P W Kaplan
Publications and source records attributed to P W Kaplan.
No, some types of nonconvulsive status epilepticus cause little permanent neurologic sequelae (or: "the cure may be worse than the disease").
Nonconvulsive status epilepticus (NCSE) is characterized by a cognitive or behavioral change which lasts for at least 30 minutes, with EEG evidence of seizures. Although there is little argument that generalized nonconvulsive status epilepticus (GNSE) does not cause lasting deficits, there is still debate regarding the morbidity of complex partial status epilepticus (CPSE). Because the EEG is used for diagnosis, a strong argument can be made that NCSE is significantly under-recognized and diagnosed. Furthermore, since the documented cases of permanent neurologic sequelae are few, the potential permanent morbidity from CPSE may be significantly exaggerated. The literature indicates that comatose patients have a poor prognosis largely as a result of comorbid conditions and coma, whereas lightly obtunded or slightly confused patients with NCSE have little or no sequelae. Patients with NCSE may suffer (hypotension and respiratory suppression) from iatrogenic 'aggressive' treatment with intravenous anti-epileptic drugs (IV-AEDs), and the findings in the literature indicate that subjects treated with benzodiazepines may have a worse prognosis. The clinician must balance the potential but rare neurologic morbidity associated with NCSE against the not infrequent morbidity caused by IV-AEDs. Better stratification of the level of consciousness and comorbid conditions is needed when evaluating outcomes so as to clearly distinguish among the deficits due to: comorbid conditions; the effects of treatment and the effects of status epilepticus (SE) proper.
Anticonvulsants↗
Clinical correlates and prognosis in early spindle coma.
OBJECTIVE: To determine the prognostic significance of spindle coma (SC) according to etiology and EEG reactivity. METHODS: We reviewed 15 patients with SC due to various causes within 8 days of coma to determine the prognostic significance of this EEG pattern. RESULTS: The outcome among survivors was favorable: among 13 survivors, 9 were independent in all activities of daily living (ADLs) at 6 months; 3 were dependent in all ADLs; and one remained in coma. EEG reactivity to noxious stimuli best predicted outcome: All patients (whatever the coma etiology) with EEG reactivity survived; conversely, not all patients without EEG reactivity died. CONCLUSION: In our patients, EEG reactivity independent of etiology predicted survival, neurological examination did not predict outcome. Most SC survivors had a meaningful recovery achieving all ADLs. From the literature, the cause of SC was predictive of outcome: encephalopathy, seizures and trauma had the best prognosis while hypoxia, CRA and structural lesions carried the worst. Literature review revealed that 23% of patients [56/242] died or remained in a persistent vegetative state (PVS). Best outcomes occurred when SC was due to drugs, encephalopathy or seizures: (0/14 died or were in a PVS). With trauma 15% [25/169] died or were in a PVS). Intermediate outcomes occurred with hypoxia and cardio-respiratory arrest (CRA): 33% [7/21] died or were in a PVS. The gravest outcomes occurred with brain-stem and cerebral infarctions, and tumors: 73% [22/30] died or were in a PVS.
Activities of Daily Living↗
Prognosis in nonconvulsive status epilepticus.
Determining the prognosis in nonconvulsive status epilepticus (NCSE) is complicated by several factors: under-recognition of NCSE with its spontaneous resolution (thus decreasing the "denominator" of total cases that will have a poor outcome); incorrect diagnosis of NCSE based on misinterpretation of EEG "epileptiform" activity; mis-classification of certain EEG patterns as NCSE (e.g. PLEDs; triphasic waves); and grouping of different populations that have markedly different co-morbidities (ambulatory patients with NCSE together with comatose patients with electrographic seizure activity on EEG). There are almost no prospective studies with premorbid neuropsychometric studies, and retrospective studies typically include isolated cases, or case series that include conditions in which the cause of NCSE itself causes cognitive morbidity. To summarize available data, absence status (ambulatory generalized non-convulsive status epilepticus) would appear to carry no lasting morbidity. Complex partial status epilepticus in ambulatory patients rarely results in measurable permanent neurologic deficit, although rarely, short or long-standing deficits may clearly occur. Because intensive treatment with intravenous anticonvulsants (e.g. benzodiazepines or phenytoin) can confer morbidity, the equation has not yet been made as to whether the morbidity of such intensive treatment for all cases of NCSE exceeds the morbidity of the disease itself. Larger, prospective studies will be needed to truly determine the prognosis in the different types of NCSE, stratified according to associated degrees of impairment (minimally impaired, moderately obtunded, comatose).
Brain↗
Etiology, neurologic correlations, and prognosis in alpha coma.
OBJECTIVE: To determine the factors affecting prognosis in alpha coma (AC). METHODS: Retrospective review of 36 study patients, 36 control coma patients matched for age and etiology, and meta-analysis of 335 cases in the world literature. RESULTS: Principal causes were cardiorespiratory arrest (CRA) (21 patients); infection, metabolic dysfunction, head trauma (3 each); and drugs, stroke and hypoxia (2 each). Outcome was predicated by EEG reactivity to noxious stimuli. Fourteen of the 15 patients with reactive EEGs, had measurable outcome, 8 awoke - all but two had etiologies other than CRA. Fourteen of 19 patients without EEG reactivity died; two had support discontinued and 3 awoke. Following CRA, 16/21 patients died and 3 had support discontinued. Only 3 patients made a good recovery - all with toxic or metabolic etiologies. Literature meta-analysis of 335 cases showed that overall, AC carried a poor prognosis (76% died). CRA (226 cases) had an 88% mortality; strokes (29 cases), a 90% mortality; hypoxia without cardiac arrest (28 cases), a 61% mortality; drug-induced AC (25 cases), an 8% mortality. CONCLUSIONS: Although the cause of AC largely predicts outcome, EEG reactivity in AC predicted survival: most patients with reactivity awoke; most of those without, died. Few survivors had meaningful recovery.
Adult↗
Ictus expectoratus: a sign of complex partial seizures usually of non-dominant temporal lobe origin.
Spitting (or expectoration) is rarely seen with seizures. In Western society, spitting is a striking behavioral aberration. A 13-year-old child had intermittent agitated behavior, episodes of rage, spitting and confusion lasting up to 2 minutes. He stood up in church and told the preacher to 'shut up and sit down'. Epilepsy monitoring revealed spitting with polysharp and spike seizures resolved over the right temporal lobe. Magnetic resonance imaging revealed a right temporal lobe ganglioglioma. Spitting seizures resolved after resection. Ictal expectoration is rare. It may occur with epigastric aura, nausea, chewing, swallowing and fumbling. Literature review disclosed 17 cases, 12 of which arose from the non-dominant hemisphere. Most regressed with surgery and anticonvulsants.
Adolescent↗
Assessing the outcomes in patients with nonconvulsive status epilepticus: nonconvulsive status epilepticus is underdiagnosed, potentially overtreated, and confounded by comorbidity.
Nonconvulsive status epilepticus (NCSE) is characterized by behavioral or cognitive change from baseline for at least 30 minutes with EEG evidence of seizures. Categorized into complex partial status epilepticus (with lateralized seizures), and generalized nonconvulsive status epilepticus (bilateral diffuse synchronous seizures), there is debate regarding the diagnosis and morbidity of NCSE. Because EEG is needed for diagnosis, only a high index of suspicion leads to a request for the study, whereas EEG is often unavailable after hours or on weekends. Furthermore, the cognitive changes during NCSE are often incorrectly ascribed to a postictal state, intoxication, psychogenic or psychotic states, and mental retardation. Regarding categorization, present classifications address EEG features but fail to distinguish among depths of coma. Deeply comatose patients (with coma etiologies that themselves carry poor prognoses) are mixed with lightly obtunded patients with no morbidity, confusing the prognosis. Thus, a classification that subsumes depth of coma, and possibly etiology, is sorely warranted. Regarding treatment, comatose NCSE patients treated with benzodiazepines may worsen, whereas generalized nonconvulsive status epilepticus patients may suffer iatrogenically from aggressive treatment (hypotension and respiratory depression) necessitating balancing the potential neurologic morbidity of NCSE against the possible morbidity of IV antiepileptic drugs. A high index of suspicion is needed to initiate EEG studies. Better stratification of level of consciousness will be needed to distinguish among morbidity due to underlying conditions, treatment, and the effects of status epilepticus, proper.
Behavior↗
Vasomotor reactivity is exhuasted in transient ischaemic attacks with limb shaking.
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Aged↗
Intravenous valproate treatment of generalized nonconvulsive status epilepticus.
Status Epilepticus (SE) is a medical emergency warranting prompt treatment with rapidly-acting antiepileptic drugs. For immediate effect, benzodiazepines are the drugs of choice, but may result in morbidity due to respiratory suppression and hypotension. Nonconvulsive status epilepticus usually does not have significant neurological sequelae, and there is little evidence that generalized nonconvulsive status epilepticus (GNSE) causes lasting neurologic deficits. The risk versus benefit of potentially morbid medications must, therefore, be weighed against the "adverse effects" of GNSE. A patient with frequent episodes of GNSE lasting 4-48 hours had previously been treated with benzodiazepines, but required admission for consequent somnolence. Following intravenous valproate given during EEG monitoring, GNSE broke after 30 minutes. The confused patient returned to a normal cognition and returned home without sequelae. Intravenous valproate may provide an effective treatment alternative to benzodiazepines in GNSE without their associated morbidity.
Adult↗
Neurologic issues in eclampsia.
Preeclampsia/eclampsia is a complex multisystem disorder with potentially severe, irreversible sequelae. Much of the morbidity involves the central nervous system. Neurologists can make important contributions to the management of eclampsia. In consultation with their obstetric colleagues they can provide input to the investigation of intracerebral vascular events, the management of seizures and raised intracranial pressure. Particularly in the case of eclampsia at early gestational ages, input on the severity and nature of the neurologic problem will assist the obstetrician in determining whether to expedite or delay delivery. Recent multicenter studies show a benefit of magnesium sulfate over either phenytoin or diazepam in the prevention of seizures, but questions about the conclusions remain. More basic and clinical research is needed, particularly on the pathophysiology of seizures and eclamptic vasospastic components, in order to provide a better understanding and consequently to design optimal therapy. The challenge includes finding an appropriate animal model for this disease with which to test such therapies.
Brain↗
Global T-wave inversion associated with nonconvulsive status epilepticus.
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Arrhythmias, Cardiac↗
Postictal hemifacial purpura.
Non-traumatic stereotyped postictal purpura is rare. A 25-year-old woman presented with right facial, cheek and periorbital purpuric eruptions that occurred after secondarily generalized tonic-clonic seizures. The stereotyped, invariably right-sided facial skin eruption, which resolved in 48 hours, falsely raised concerns of spousal abuse. Possible pathophysiological mechanisms include: (a) valsalva-induced capillary hypertension with secondary purpura, (b) ictal corticolimbic stimulation of the autonomic nervous innervation of facial vasomotor structures, and (c) trigeminal-mediated local release of vasoactive substances. Although rare, such stereotyped patterns of purpura should be recognized to avoid incorrect attribution of spousal abuse.
Adult↗
Fixation-off and eyes closed catamenial generalized nonconvulsive status epilepticus with eyelid myoclonic jerks.
PURPOSE: Eyelid myoclonic jerks have been described in fixation-off-sensitive (FOS) epilepsy, but their relationship to nonconvulsive status epilepticus (NCSE) or to catamenial exacerbations is little reported. METHODS: We describe a woman of normal intelligence with catamenial periods of prolonged NCSE who exhibited various intra- and interseizure thresholds of polyspike suppression when her eyes were open, with particular visual inputs and with antiepileptic drug (AED) treatment. RESULTS: In one episode, on the first day of the woman's menstrual period, bursts of bilateral synchronous polyspike activity were briefly suppressed with visual fixation but were more lastingly suppressed after administration of lorazepam (LZP). During another period of NCSE, the SE was completely suppressed by visual fixation on objects and patterned checkerboard screens and by ocular convergence, was incompletely suppressed when her eyes were open in a dark room and when her eyes were open without visual fixation, but was not suppressed by mental activation alone. CONCLUSIONS: FOS polyspike bursts with eyelid myoclonic jerks may exhibit catamenial exacerbations, varying from completely suppressible with visual fixation to nonsuppressible during NCSE. These findings suggest an interplay between humoral factors, AEDs, and seizure threshold in this condition.
Adult↗
Reversible hypercalcemic cerebral vasoconstriction with seizures and blindness: a paradigm for eclampsia?
Although hypercalcemia may cause drowsiness, lethargy, weakness, confusion and coma it rarely causes seizures or cerebral infarction. The patient presented had a clinical evolution from hallucinosis to a generalized tonic-clonic seizure, and subsequent cortical blindness with occipital cerebral ischemia as evidenced by SPECT and MRI scans. EEG revealed occipital PLEDs. With reversal of hypercalcemia, there was a return of vision, resolution of EEG epileptiform activity, although with some residual occipital infarction. This case, in concert with a literature review of hypercalcemia, reveals examples of occipital and watershed ischemia, blindness, seizures and hypertension, a pattern markedly similar to that of eclampsia. Furthermore, medications such as magnesium sulfate, believed to reverse cerebrovasospasm responsible for the eclamptic neurologic findings, may counter the effects of hypercalcemia at a cellular level, lending support to a calcium-mediated injury in eclampsia.
Aged↗
Somatosensory evoked potentials in adrenomyeloneuropathy.
Adrenomyeloneuropathy (AMN) is an X-linked metabolic disorder causing accumulation of very-long-chain fatty acids with multifocal nervous system demyelination of the peripheral nerves, spinal cord, and cerebrum. The extent to which the disorder affects upper versus lower limbs or peripheral versus CNS has not been electrophysiologically defined in a large population nor differentiated in men and women. To determine patterns of nervous system demyelination and define gender differences, we studied 83 AMN patients with short latency median and posterior tibial nerve somatosensory evoked potentials (SSEPs). Most women (10/16) had abnormal median SSEPs all involving central pathways, whereas most men (59/67) had abnormal median SSEPs involving both peripheral and central pathways. Tibial SSEPs were abnormal in both sexes (14/15 women, 67/67 men), with either peripheral or central pathway involvement. This study demonstrates the frequent widespread involvement of both peripheral nerve and central somatosensory pathways in men with AMN and the predominantly central involvement in women.
Adolescent↗
Nonconvulsive status epilepticus.
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Electroencephalography↗
Nonconvulsive status epilepticus in the emergency room.
PURPOSE: The study reviewed emergent cases of nonconvulsive status epilepticus (NCSE) to evaluate causes of diagnostic and management delay and examined frequent diagnostic features suggestive of NCSE. METHODS: In a retrospective study, we assessed the clinical presentation of 23 patients with one or more NCSE episodes, their medical history, EEG, and antiepileptic drug (AED) treatment. We also evaluated causes of diagnostic delay in patients referred to the emergency room (ER) in confusional states. RESULTS: There was considerable overlap in clinical features of patients with complex partial SE (CPSE) and generalized nonconvulsive SE (GNSE). Delays in seeking medical attention were common. Diagnosis was significantly delayed in 10 patients. Three cases illustrate the possible markedly different presentations of NCSE. CONCLUSIONS: NCSE often goes unrecognized or is mistaken for behavioral or psychiatric disturbance. The pleomorphic clinical presentation of NCSE indicates that EEG and a therapeutic trial of AEDs afford the best diagnostic measures in acute waxing and waning confusional states associated with agitation, bizarre behavior, staring, increased tone, mutism, or subtle myoclonus.
Adult↗
Visual system abnormalities in adrenomyeloneuropathy.
We studied the visual system in 59 men with adrenomyeloneuropathy. Pattern-reversal visual evoked potentials, magnetic resonance imaging, and clinical examination revealed that visual pathways are affected in 63% of patients, involving the optic discs, optic nerves, lateral geniculate bodies, optic radiations, and parietooccipital cortex. This indicates both primary demyelination of the optic nerves and discs, and more diffuse involvement of postchiasmal structures.
Adrenoleukodystrophy↗