Lance-Adams Syndrome

Lance-Adams syndrome (LAS) is a rare complication of successful cardiopulmonary resuscitation and is often accompanied by action myoclonus. LAS is seen in patients who have undergone a cardiorespiratory arrest, later regained consciousness, and then developed myoclonus days or weeks after the event. Less than 150 cases of LAS have been reported in the worldwide medical literature. Here, we present a 32-year-old man who suffered from myoclonus after hypoxic brain damage due to hanging himself. This case was diagnosed as Lance-Adams syndrome according to a history of hypoxic brain damage, the clinical features, and the neuroimages such as brain SPECT. Making an early diagnosis and properly managing LAS is positively related to improving the patient's functional outcome. If patients have posthypoxic myoclonus after successful cardiopulmonary resuscitation, we should consider the diagnosis of LAS and initiate a proper rehabilitation program.

Síndrome de Lance-Adams: presentación de dos casos / Lance-Adams syndrome: report of two cases

Lance-Adams syndrome was described in 1963 is a rare complication due to recovered hypoxic episodes or prolonged hypotension events. Is characterized by action myoclonus and cerebellar ataxia. We report two patients studied with this syndrome. A 51 year-old men and a 72 years-old men fully recovered after a brief cardiorespiratory arrest they developed intention myoclonus, triggered by voluntary movements, posture, also by sounds, touches and emotional stimuli. It also was accompanied by cerebellar syndrome, ataxia and posture control alterations. They had a Magnetic Resonance (MR), EEG and normal metabolic parameters. Myoclonus was treated with sodium valproate and clonazepam. The neurophysiologic interpretation of this motor imbalance is an abnormal functioning of the Central Pattern Generator Netwoks (CPGN) located in the mesencephalic region. Hypoxic lesions in vermian purkinje and paravermal cerebellum neurons have an inhibitory effect in this system, producing motor control attenuation, generating an imbalance in the motoneurons of the spinal cord contraction sequence, which starts shooting in an uncoordinated way. As in almost all cerebellar lesions with time they tend to compensate and to diminish myoclonus.

El Síndrome de Lance-Adams descrito en 1963, es una rara complicación que sigue tardíamente a episodios hipóxicos o de hipotensión prolongada, ya recuperados. Se caracteriza por mioclonías de acción y ataxia cerebelosa. Se describen dos pacientes estudiados con este síndrome. Son dos hombres de 51 y 72 años que después de un paro cardiorrespiratorio breve, de recuperación completa, iniciaron mioclonías de intención, activadas por movimientos voluntarios, posturas, estímulos sonoros, táctiles y afectivos. Acompañado además de un síndrome cerebeloso, ataxia de la marcha y alteraciones del control postural. Cursaron con RM (Resonancia Magnética), EEG (Electroencefalograma) y parámetros metabólicos sin relevancia patológica. Las mioclonías fueron controladas con ácido valproico y clonazepam. La interpretación neurofisiológica de este desajuste motor es la alteración en el funcionamiento del patrón central de circuitos generadores (PCCG) ubicado en la región mesencefálica. Las lesiones hipóxicas de las neuronas de Purkinje del vermis y paravermianas del cerebelo, que tienen un efecto inhibitorio para este sistema, producen una atenuación del control motor del PCCG, generando desajuste en la secuencia de la contracción de las motoneuronas de la médula espinal, que comienzan a dispararse de manera independientemente. Como ocurre con la mayoría de las lesiones cerebelosas, con el tiempo tienden a compensarse y por consiguiente a disminuir las mioclonías.

Acute Posthypoxic Myoclonus in Recovery Room: A case report / 대한마취과학회지

Acute posthypoxic myoclonus is not an uncommon complication of cardiopulmonary arrest. The presence of myoclonus early after hypoxia or anoxia has been reported as a poor prognostic factor. Especially posthypoxic myoclonus status epilepticus was defined as continuous myoclonic seizure activity lasting 30 minutes or more which is thought to reflect irreversible neocortical damage, but there have been very rarely reported complete recovery. We report a case of the patient who developed posthypoxic myoclonus status epilepticus and completely recovered without any complications.

A Case of Posthypoxic Myoclonus with Lamotrigine Add-On Therapy / 대한간질학회지

Posthypoxic myoclonus is poorly controlled with current treatments. Based on clinical experience, valproate and benzodiazepines have been used to treat myoclonic seizures. Rarely, some antiepileptic drugs may exacerbate myoclonic seizures. Although lamotrigine is controversial for treatment in myoclonic seizures, we experience a case of posthypoxic myoclonus improved with lamotrigine add-on therapy.

Is the Intensive Anticonvulsant Treatment for Control of Acute Posthypoxic Myoclonic Status Epilepticus Necessary?

BACKGROUND: Acute posthypoxic myoclonic seizure is not an uncommon presentation in clinical practices. However, there have been only a few reports which include detailed description on the treatment of acute posthypoxic myoclonic status. METHODS: We retrospectively analyzed the etiology, clinical course, nature and duration of myoclonus after hypoxic brain damage during a ten year period from 1992 to 2002. Patients who had possible explanations for myoclonic seizure other than hypoxic-ischemic events were excluded. RESULTS: Eighty-two patients aged 24 to 81 years old were included in this study. All but 3 patients with pulmonary edema had a history of cardiorespiratory arrest caused by probable cardiogenic origin, acute asthma attack and upper airway obstruction. Ictal EEGs demonstrated intermittent generalized spike and polyspike activities or biPLEDs. Most patients died within a week and 6 patients remained in persistent vegetative states. The clinical outcome was not affected by the drug response of myoclonic status epilepticus or etiology of hypoxia. CONCLUSIONS: Posthypoxic myoclonic status reflects severe and diffuse cortical damage, and implies a very poor prognosis. It may be a transient manifestation of severe cortical damage before massive and irreversible neocortical neuronal death, and aggressive anticonvulsant drug treatment would not be necessary in most patients.

A Case of Hypoxic Encephalopathy Following Anesthesia for a Patient with Ludwig`s Angina / 대한마취과학회지

Ludwig's Angina is the term given to the symptoms resulting from infection of the submandibular space and is characterized especially by extreme edema of the floor of the mouth. Airway obstruction and respiratory difficulty is common due to edema of the mouth, tongue, and the glottis, from mediastinitis due to spread, or from septicemia or pneumonia. Thus it is necessary for the anesthetist to attend to specific management of airway maintenance. A 49 year old male had an operation for incision and drainage under balanced anesthesia. After the operation, posthypoxic myoclonus due to anoxia for three minutes appeared while in the recovery room. The patient, controlled by oxygen supply and diazepam and antiepileptics, has recovered from the seizures, but has not recovered from intention myoclonus and speech disturbance, which are slowly improving. This report describes this case of posthypoxic myoclonus following anesthesia and reviews the literatures.