Todd's Paralysis Research Articles

Child Neurology: Migraine with aura in children

The differential diagnosis for an acute hemiparesis in a child includes stroke, Todd paralysis, and hemiplegic migraine. In the context of an illustrative case, this review highlights the differences in clinical presentation among these entities and an approach to the diagnostic workup. Migraine with aura in children is reviewed, including migraine equivalents such as abdominal migraine and the particular presentation of hemiplegic migraine. An approach to the prophylactic and acute treatment for children with migraine with aura is offered.

Hyperfamiliarity for faces

To report 4 cases of hyperfamiliarity for faces (HFF) and review 5 previously reported cases. We identified cases of HFF from PubMed search and references in prior reports. Three of our 4 cases had pathologic findings that were most extensive in the left temporal lobe. HFF occurred after a tonic-clonic seizure (cases 1 and 3), during simple partial seizures (case 2), and in the setting of an increase in simple partial seizure frequency but not during seizures (case 4). All 9 cases were adults with 1 or more seizures; symptoms first occurred after seizures in 5 cases and during seizures in 1 case. Ictal symptoms lasted from seconds to minutes and from 2 days to more than 7 years in the other 6 cases. The duration of HFF was not associated with the presence or extent of a structural lesion. While in several cases HFF appears to result from a postictal Todd paralysis, the mechanism underlying persistent cases is uncertain. This modality (visual)-specific and stimulus (face)-specific syndrome is associated with diverse structural, functional imaging, and neurophysiologic findings. Lesions are more often left-sided and involve the temporal lobe. Epilepsy and seizures were present in all 9 cases, suggesting a pathophysiologic relationship, which likely varies among cases. Although only reported in 9 patients, HFF is probably much more common than it is diagnosed.

Moyamoya Induced Acute Paraplegia in A Child with Epilepsy

Objective disease (MMD) is a chronic, occlusive, cerebrovascular disorder of unknown pathogenesis, characterized by progressive stenosis of the bilateral supraclinoid internal carotid arteries, with concomitant formation of tortuous arterial collateral vessels at the base of the brain, which reconstitute distal branches of the cerebral circulation. In Japanese, Moyamoya means hazy puff of smoke and refers to the angiographic appearance of the abnormal network of vessels that develop at the base of the brain and basal ganglia to supply a collateral route of blood flow. We report here the case of disease in a 5 year-old girl with normal mentality with a one year history of epilepsy, with Todd's paralysis. This condition is rare and most patients are diagnosed in childhood. With this report we aim to underscore the possibility that a usual neurological sign could be associated with unusual neurological disorders.

Repetitive transcranial magnetic stimulation in a patient with atypical Todd's postepileptic paralysis

Repetitive transcranial magnetic stimulation in a patient with atypical Todd's postepileptic paralysis

Local cortical hypoperfusion imaged with CT perfusion during postictal Todd’s paresis

Postictal ("Todd's") paralysis, or "epileptic hemiplegia," is a well-known complication of focal or generalized epileptic seizures. However, it is unclear whether the pathophysiology of Todd's paralysis is related to alterations in cerebral perfusion. We report CT perfusion findings in a patient presenting with postictal aphasia and right hemiparesis. A 62-year-old woman with a history of alcohol abuse, closed head injury and posttraumatic epilepsy, presented with acute onset aphasia and right hemiparesis. A non-contrast head CT scan demonstrated no acute hemorrhage. Left hemispheric ischemia was suspected, and the patient was considered for acute thrombolytic therapy. MRI revealed a subtle increase in signal intensity involving the left medial temporal, hippocampal and parahippocampal regions on both T2-weighted FLAIR and diffusion-weighted sequences. CT angiography and CT perfusion study were performed. The CT perfusion study and CT angiography demonstrated a dramatic reduction in cerebral blood flow and blood volume involving the entire left hemisphere, but with relative symmetry of mean transit time, ruling out a large vessel occlusion. Clinical resolution of the aphasia and hemiparesis occurred within a few hours, and correlated with normalization of perfusion to the left hemisphere (detected by MR perfusion). This unique case is the first in which clinical evidence of Todd's paralysis has been correlated with reversible postictal hemispheric changes on CT and MR perfusion studies. This is important because CT perfusion study is being used more and more in the diagnosis of acute stroke, and one needs to be careful to not misinterpret the data.

CCM1 gene deletion identified by MLPA in cerebral cavernous malformation

Familial cerebral cavernous malformations (CCMs) occur with a frequency of 1 in 2000 and may cause recurrent headaches, seizures, and hemorrhagic stroke. Exon-scanning-based methods have identified intragenic mutations in three genes, CCM1, CCM2, and CCM3, in about 70% of familial CCM. To date, only two large CCM2 and a single large CCM3 deletion have been published. In addition to direct sequencing of all three CCM genes, we applied a newly developed multiplex ligation-dependent probe amplification gene dosage assay (MLPA) designed to detect genomic CCM1-3 deletions/duplications. Direct sequencing did not reveal a mutation in the index case who presented with multiple CCMs that had caused a generalized tonic-clonic seizure with Todd's paralysis and headaches at the age of 5. In contrast, MLPA analyses detected a large deletion involving the entire CCM1 coding region in the proband and further affected members of this German CCM family. The MLPA results were corroborated by analyses of single nucleotide polymorphisms (SNPs) within the CCM1 gene. Thus, we here present the first report on a CCM1 gene deletion. Our results confirm a loss-of-function mutation mechanism for CCM1 and demonstrate that the use of MLPA enables a higher CCM mutation detection rate which is crucial for predictive testing of at-risk relatives.

0848 Todd's paralysis disclosing neurocysticercosis

0848 Todd's paralysis disclosing neurocysticercosis

A history of Todd and his paralysis.

To describe the history of Robert Bentley Todd (1809-1860) and certain of his contributions to medicine, including his original and subsequent descriptions of "epileptic hemiplegia," which came to be called "Todd's paralysis." The author conducted a comprehensive review of English-language literature, modern and historical, related to "Todd's paralysis" and examined Todd's original case histories and commentary by Todd, his contemporaries, and his successors. Todd held that some patients "who recover from a severe fit, or from frequently repeated fits of epilepsy, are often found to labor under hemiplegia, or other modifications of palsy." He believed that this resulted from "undue exaltation. [resulting in] a state of depression or exhaustion." Interestingly, Todd was the first to present an electrical theory of epilepsy, supported by his own animal experimentation, well before his better-known successor John Hughlings Jackson (1835-1911) (famous for his investigations of partial epilepsies and the eponymous "Jacksonian march"). Many neurologists and investigators followed Todd in acknowledging transient postictal paralysis as a distinct clinical entity. Yet whether the pathophysiology of "Todd's paralysis" is related to "neuronal exhaustion" or excessive inhibition is still controversial.

Misdiagnosis of stroke in tissue plasminogen activator–treated patients: Characteristics and outcomes

Study objective Misdiagnosis of acute ischemic stroke is a risk inherent in treating patients with acute deficits, yet few data exist on the problem. We report an evaluation of emergency department misdiagnoses in patients treated with tissue plasminogen activator for acute ischemic stroke. Methods We conducted an observational study of 151 consecutive patients treated without an acute stroke team. Initial diagnosis was compared with interim and hospital discharge diagnoses. Separate analyses were performed for patients without a final diagnosis of acute ischemic stroke and for those without a final diagnosis of either acute ischemic stroke or transient ischemic attack, combined. Results Ten of 151 patients (7%; 95% confidence interval [CI] 3% to 12%) had final diagnoses that did not include acute ischemic stroke. Six of 151 (4%; 95% CI 1% to 8%) had a final diagnosis other than acute ischemic stroke or transient ischemic attack (conversion disorder [4], complex migraine [1], and Todd's paralysis [1]). These “stroke mimics” had no intracranial hemorrhage (0%; 95% CI 0% to 31%), had less disability at discharge (modified Rankin Scale score mean±SD, 2.2±1.3 versus 3.2±1.8), and were younger (mean age±SD, 47±21 years versus 68±15 years) than patients with acute ischemic stroke or transient ischemic attack. An additional 4 (3%) patients had interim diagnoses other than acute ischemic stroke, all subsequently changed to acute ischemic stroke after magnetic resonance imaging or computed tomography. Conclusion These data show that in a 4-hospital system without an acute stroke team, thrombolytic treatment of patients with diagnoses mimicking stroke was infrequent, and hemorrhagic complications did not occur in any patients without an acute ischemic stroke. However, because the number of mimics was small, safety cannot be ensured with statistical confidence.

Prognosis for seizure recurrence in patients with newly diagnosed neurocysticercosis.

To determine the risk of seizure recurrence after a first seizure due to neurocysticercosis (NC) and to evaluate risk factors for seizure recurrence, including the influence of antihelminthic treatment. The authors prospectively followed 77 patients with a first seizure and active or transitional NC for >7 years (median 24 months). Thirty-one patients (40.3%) experienced seizure recurrence. Kaplan-Meier estimated recurrence was 22% at 6 months, 32% at 12 months, 39% at 24 months, and 49% at 48 and 84 months. Treatment with an antihelminthic (albendazole) did not influence recurrence. On multivariable analysis, none of the following predicted recurrence: sex, presenting seizure type, classification of NC, localization of cysts, Todd paralysis, neurologic deficits at presentation, EEG abnormalities. Only change in CT predicted recurrence: 22% in patients in whom cysts disappeared and 56% in patients with persistent cysts (p < 0.05). In this latter group, recurrence was associated with persistence of an active lesion. Of those with two seizures, estimated risk of a third seizure was 68% by 6 years after the second seizure. Seizure recurrence is high after a first acute symptomatic seizure due to NC, but this seems related to persistence of active brain lesions. Recurrence risk is low and in keeping with seizure risk following other brain insults leading to a static encephalopathy in those in whom the NC lesion clears. Patients with NC should receive antiseizure medications until the acute lesion clears on CT. There is no correlation between treatment with antihelminthic agents and seizure recurrence.

Lateral Temporal Hyperperfusion in Postictal Psychosis Assessed by 99mTc-HMPAO SPECT

Postictal psychosis is a rare complication of epileptic seizure characterized by reversible psychotic symptoms after flurries of seizure attack. It has been attributed to a phenomenon similar to Todd's paralysis without definitive proof. We studied regional cerebral blood flow (rCBF) of six patients with postictal psychosis by 99mTc-HMPAO SPECT scan. Baseline rCBF was compared with the rCBF during postictal psychosis. An asymmetry index (ASI) was calculated as 200 × [(ipsilateral ROI count density − contralateral ROI count density)/(ipsilateral ROI count density + contralateral ROI count density)] %. Significant differences could be found between ASIs during postictal psychosis and interictal state SPECT scan over the lateral temporal neocortex region ( P = 0.017). Although hyperperfusion abnormality in SPECT can be found in Todd's paralysis, such findings are more commonly found in cerebral hyperactivity conditions. Taking into account the clinical characteristics of postictal psychosis, namely a preceding lucid interval and crescendo–decrescendo clinical course, these may be an alternative psychopathogenic mechanism for the development of postictal psychosis.

Clinical and EEG analysis of initial status epilepticus during infancy in patients with mesial temporal lobe epilepsy

This study investigated the clinical and EEG characteristics of initial status epilepticus (SE) during infancy in patients with mesial temporal lobe epilepsy (MTLE). The subjects were six patients who had been brought to our emergency clinic and treated for their initial SE between 1977 and 1988, and later developed MTLE. We reviewed the medical records and laboratory findings at the time of the initial SE, and the clinical evolution up to the development of MTLE. The six patients included four females and two males. The initial SE developed at ages ranging from 7 months to 2 years and 9 months with a mean of 1 year and 2 months. These episodes were characterized by an elevated temperature of more than 38 °C (4/6 cases), clusters of prolonged seizures during one episode of SE (4/6 cases), long-lasting SE (120–380 min, mean 227 min, 6/6 cases), postictal prolonged loss of consciousness (median 5 h, 6/6 cases), and the presence of Todd's paralysis (3/6 cases). The lateralization of the ictal or postictal EEGs of the SE in five of the six cases was identical to that of the hippocampal atrophy later confirmed by MRI. Follow-up EEG examinations at a 6 month interval demonstrated temporal spike discharges appearing only after the onset of complex partial seizures. Two patients, who had no fever at the initial SE, were characterized by a very early appearance of epileptic EEG abnormality and a short interval between the initial SE and the development of complex partial seizures, suggesting that the SE was the first epileptic manifestation. The result of this study showed that SE progressing to MTLE tends to have complicated clinical manifestations characterized by clusters of unilateral or generalized SE followed by prolonged postictal unconsciousness, generalized clinical manifestations despite lateralized ictal EEG discharges, and the Todd's paresis in addition to the prolonged seizure duration.

Semiology of typical and atypical Rolandic Epilepsy: a video‐EEG analysis

ABSTRACT Since the first descriptions of Rolandic Epilepsy or benign epilepsy with centrotemporal spikes (BECTS), typical and atypical forms have been reported. Indeed, classical focal seizures are sometimes associated with various atypical ictal symptoms and cognitive or behavioural disorders. In an effort to define early clinical and EEG criteria allowing early distinction between typical and atypical forms, we recently conducted a prospective study in a cohort of children with Rolandic Epilepsy. The results of this study have been reported elsewhere. We now discuss the semiological characteristics, and comment on the video‐EEG data collected during this study. Symptoms were classified into three major categories: “classical focal seizures”; “spike and wave related symptoms”; and “paraictal symptoms”. Classical focal seizures constitute the electroclinical expression of the development and the propagation of a focal cortical neuronal discharge. “Spike and wave related symptoms” are brief neurological or neuropsychological phenomena having a relatively strict temporal relation with individual components of isolated focal or generalized spikes and waves. “Paraictal symptoms” consist of acquired progressive and fluctuating motor or cognitive deficits and are not directly correlated with Todd paralysis. We present detailed video‐EEG material of selected cases and discuss the usefulness of such distinctions in terminology. We suggest that variability in clinical expression probably reflects the implication of different pathophysiological mechanisms, which in turn could explain differences in sensitivity to treatment. (Published with videosequences.)

Should physicians give tPA to patients with acute ischemic stroke? Against: and just what is the emperor of stroke wearing?

Should physicians give tPA to patients with acute ischemic stroke? Against: and just what is the emperor of stroke wearing?

Technetium-99m-ethyl cysteinate dimer brain perfusion loss in pediatric postictal psychosis

A 91/i-YEAR-OLD BOY was admitted to the hospital with status epilepticus and was treated with intravenous diazepam, phenytoin, and midazolam. After the convulsions stopped, he remained agitated and confused. Twenty-four hours later, psychotic ideation was noticed. He developed visual hallucinations, confused speech, auditory illusions, psychomotor restlessness, and disorganized behavior. His medical history was remarkable for a right hemiplegia diagnosed in the first year of life, caused by a polycythemia-related neonatal stroke. A brain computed tomography (CT) scan that was performed a year later showed an enlarged left ventricle and left hemispheric atrophy. At age 9, the patient was hospitalized for prolonged left-sided complex partial seizures, followed by Todd's paralysis on the right side. The neurological examination on the current admission revealed mild right hemiparesis, unchanged from before. Routine serum biochemistry and a complete blood count were normal. A lumbar puncture was normal. Repeated electroencephalograms (EEGs) showed left-sided slowing but no epileptic activity. A brain magnetic resonance imaging (MRI) scan showed severe atrophy of the left hemisphere with a thalamic hypodensity (Fig 1), similar to the previous imaging. The patient was diagnosed with postictal schizophrenia-like psychosis. A perfusion brain single photon emission computed tomography (SPECT) was performed 30 minutes after injecting 12 mCi (444 MBq) of Technetium (Tc)-99m-ethyl cysteinate dimer (ECD) intravenously by using a dualhead gamma camera. The images showed markedly decreased perfusion to the left hemisphere, including the basal ganglia and the thalamus (Fig 2A). The hypoperfusion was most prominent in the left medial temporal lobe. Moderately diminished perfusion was also observed in the frontal lobes. The psychosis lasted for 7 days and resolved gradually without treatment. A repeat SPECT study 3 months later (Fig 2B) showed improved perfusion to the left temporal lobe and to the fight frontal lobe. The patient remained asymptomatic after a 6-month follow-up. Brief psychotic episodes may follow a bout of seizure clusters or a recent exacerbation in seizure frequency. 17 Postictal psychosis may develop in nearly 10% of adult patients, 6 but it is rare in children. The psychosis may be caused by repeated electrical discharges that change local neurotransmitter activity. It is also conceivable that structural brain abnormalities, eg, cortical dysgenesis or diffuse brain lesions, may lead to both the epilepsy and the psychosis. Postictal psychosis usually resolves within a few days. 5 Recurrent seizures may exacerbate the psychosis, and careful monitoring of anticonvulsant therapy is mandatory. EEG abnormalities persist in the majority of patients during the psychosis. 1 Some suggested risk factors are early onset and severe epilepsy, secondary generalization of seizures, certain anticonvulsive drugs, and temporal lobectomy. Most investigators suggest that psychosis in epilepsy is preferentially associated with temporal lobe seizures. 8-a2 MRI studies show that postictal psychosis is most likely to occur in patients with resistant temporal lobe epilepsy stemming from mesial temporal sclerosis, especially on the left side. 6 Changes in postsynaptic dopamine receptor sensitivity have been suggested as the mediating mechanism. SPECT studies have shown low levels of striatal dopamine D2 receptors in patients with peri-ictal psychosis. 13 Possible differential diagnoses for our patient are listed below.

Tc-99m HMPAO brain perfusion SPECT images in a patient with Todd's paralysis.

Tc-99m HMPAO brain perfusion SPECT was performed in a 10-month-old patient with postepileptic transient left hemiplegia using a dual-headed gamma camera. Decreased perfusion to the right basal ganglia and thalamus was shown. Diagnostic information could be obtained on such a small patient with Todd's paralysis by conventional brain perfusion SPECT.

Acute medical therapy

The management of the acute stroke patient requires a coordinated, multidisciplinar y effort. In most emergency departments, the ratio of stroke to stroke mimics is 1 to 4 or i to 5. Therefore, there must be an efficient, skilled procedure for eliminating those diseases that mimic stroke such as Todd's paralysis, transient ischemic attacks (TIA), hysteria, migraine, and carpal ~nnel syndrome. Stroke patients must be stabilized according to basic protocols (airway, breathing, circulation) before neurologic evaluation begins; this process should require no more than a minute or two. Stroke management then includes diagnosis and possibly treatment. All of this is best accomplished with an institutionalized Stroke Team, comparable with a Code Blue team that rehearses, monitors performance, and uses feedback to continually improve care. Most of the time, acute stroke care will be merely supportive. In a small number of cases, however, thrombolysis will be indicated. Because the use of thrombolytic therapy is complicated, we devote the majority of our remarks to its use including rationale, patient selection, and drug administration. Brief remarks will be included for nonspecific management.

Index of Suspicion

Index of Suspicion

Some Contributions of Robert Bentley Todd

A Dubliner by birth and education, Robert Bentley Todd (1809–1860) settled in London, achieving success as physician and educator. He was professor of anatomy and physiology at King's College, and a founder of King's College Hospital. His publications were numerous; he edited a Cyclopaedia of Anatomy and Physiology in which he introduced the terms afferent and efferent and pointed to the location of the major lesion of tabes dorsalis. He described postictal paralysis in his Lumleian Lectures (1849); the features of “Todd's paralysis” are discussed. He appeared for the prosecution at the Smethurst murder trial (1859). He prescribed wine and brandy copiously for fevers.

Technetium-99m-HMPAO SPECT in patients with hemiconvulsions followed by Todd's paralysis

We performed technetium-99m-hexamethylpropylene- amineoxime (Tc-HMPAO) single photon emission computed tomography in two patients with prolonged hemiconvulsions followed by transient hemiparesis (Todd's paralysis). In both cases, a prolonged post-ictal cerebral hyperperfusion state of approximately 24 h was observed, even after the neurological deficits had resolved. The cerebral hyperperfusion in both cases was of much longer duration than that in previously reported cases of single and uncomplicated focal seizures. The prolonged cerebral hyperperfusion might have been due to impairment of the cerebrovascular autoregulation in seizures followed by Todd's paralysis.