Resolution of Decerebrate Posturing from Paroxysmal Autonomic Instability with Dystonia Syndrome

Dear Editor, Decorticate and decerebrate postures are abnormal stereotypical trunk and extremity motions which may present in response to an unpleasant stimulus from an external or an internal source that are usually suggestive of serious brain or spinal damage.[1] Charles Sherrington, a Nobel Laureate, in 1898 initially documented decerebrate posturing after transecting live cat and monkey brainstems.[2] Both postures can be caused by brain lesions in a variety of anatomical areas, however, they often include some degree of brainstem injury.[1] Episodes of decerebrate posturing with preexisting changes in the central nervous system were noted in response to a variety of physiological conditions. They are not limited to hyperpyrexia, reduced tissue oxygenation, metabolic disturbances, sensory disturbances, hypoglycemia, and meningeal irritability and well-being. A 64-year-old male patient to the emergency department as unresponsive and irritable. The patient had been mute, unresponsive, and had a decreased appetite (only liquid intake) for 3 days, which was accompanied by fever. The patient had comparable hypoglycemia symptoms a week ago with CBG 50 mg/dl and was treated with 25% dextrose IV stat. The patient had a history of agitated behavior, accompanied by vigorous upper and lower limb movement, up rolling of eyes, clenching of teeth, and generalized clonic seizures secondary to alcohol withdrawal. There was no history of palpitations, vomiting, or loose stools. The patient had no history of comorbidities at the time of eliciting history. The patient has been a chronic alcoholic for 12 years along with a history of betel nut chewing for 6 years. On examination, the patient was unconscious, unresponsive, and febrile with a Glasgow Coma Scale (GCS) of 8/15 (E2-V2-M4). Vitals on presentation were SPO2 100% in room air, PR – 72 bpm, and blood pressure – 160/100 mmHg. Clubbing was present; there was no pallor, icterus, cyanosis, or edema. Systemic examination of the cardiovascular, respiratory, and gastrointestinal systems was normal. Central nervous system examination for motor, sensory, memory, and speech functions could not be elicited. His GCS score on arrival was 8 (E2-V2-M4). First computed tomography (CT) showed right parenchymal hematoma with subdural extension also noted subarachnoid hemorrhage on the same side, mild intraventricular hemorrhage and a midline shift, as well as minimal herniation impacting the brainstem. An open craniotomy under general anesthesia was done to evacuate the hematoma. Follow-up CT showed a significant reduction in the hematoma and midline displacement. Sedation was gradually withdrawn, and the patient remained unresponsive for 48 h. Consent was taken from attendees to present this case for academic purposes. The patient scored 24 on the Disability Rating Scale[3] and Level of Cognitive Functioning Scale score of 2 (generalized response). Later over months, the neurological symptoms improved, during discharge, he had improved response to commands, was able to recognize objects showed better visual acuity. In 4 days, he entered a “classic” locked-in state with total quadriplegia. He underwent a tracheostomy to protect his airway. He had intermittent prolonged tonic decerebrate posturing with trismus, which made oral suctioning challenging. Each episode lasted for 1–3 h with spontaneous recovery. These episodes were associated with tachycardia, hypertension, and diaphoresis (mimicking paroxysmal autonomic instability with dystonia). During these episodes, he communicated by eye movements. Muscle tone returned to normal between episodes, and he experienced modest spasticity in all four limbs, as well as hyperreflexia. He received anti-edema medications. Over the course of a month, his motor function gradually improved until he fully recovered. As previously highlighted, compressive lesions benefit from surgical intervention, if carried out in a timely manner; thus, early CT or magnetic resonance imaging is advised. Other variables, such as infection and metabolic disturbance, should be investigated using serum and cerebrospinal fluid samples. Given the poor prognosis for patients with abnormal posture, especially decerebration, aggressive treatment is ethically questionable mandating a case-by-case examination. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.