Search

Paroxysmal autonomic instability syndrome with dystonia (PAISD) is a possible complication that worsens the prognosis of hypoxic-ischemic encephalopathy related to non-fatal drowning. There are case reports of hyperbaric oxygen (HBO2) therapy enhancing recovery in such cases. We report a case of a 5-year-old boy admitted to the Pediatric Intensive Care Unit after a non-fatal drowning. He was transferred under mechanical ventilation and sedation, with hemodynamic instability and hypothermia. On admission he had a Glasgow Coma Score of 6. On the fifth day of admission he presented episodes of dystonia with decerebration posture, diaphoresis, tachycardia and hypertension, sometimes with identified triggers, suggesting PAISD. The episodes were difficult to control; multiple drugs were needed. Electroencephalography showed diffuse slow wave activity, and cranioencephalic magnetic resonance imaging showed hypoxia-related lesions, suggesting hypoxic-ischemic encephalopathy. Early after admission the patient started physiotherapy combined with normobaric oxygen therapy. Subsequently he started HBO2 therapy at 2 atmospheres, with a total of 66 sessions. Dystonia progressively subsided, with gradual discontinuation of therapy. He also showed improvement in spasticity, non-verbal communication and cephalic control. This case highlights the diagnostic and therapeutic challenges of PAISD and the potential benefit of HBO2 therapy, even in the subacute phase, in recovery of hypoxic-ischemic encephalopathy., Competing Interests: The authors of this paper declare no conflicts of interest exist with this submission., (Copyright© Undersea and Hyperbaric Medical Society.)

Cystic fibrosis liver disease (CFLD) affects a large proportion of cystic fibrosis (CF) patients; however encephalopathy is a rare complication. While classical hepatic encephalopathy can be a feature of end-stage liver disease, "hyperammonemic encephalopathy" can be precipitated in previously stable CFLD by various triggers including systemic corticosteroids. We describe one such case and review the relevant literature., (Copyright © 2020. Published by Elsevier Ltd.)

Opisthotonus as a presenting feature in neurosurgical patients is rare, with few reports describing such presentations. Only four reports of opisthotonos secondary to posterior fossa mass were identified. An unclear pathophysiology, and broad aetiology contribute to clinical misdirection. While posterior fossa lesions commonly present with signs of raised intracranial pressure, or cerebellar dysfunction, this case describes the presentation of an infant with opisthotonic posturing, ataxia and autonomic dysfunction secondary to a large pilocytic astrocytoma. Despite initial treatment of hydrocephalus, opisthotonus only resolved with complete surgical resection of the posterior fossa mass. At follow-up, the child remains well and without signs of hypertonicity or other signs or symptoms. Presentations involving opisthotonus are rare, and active exclusion of posterior fossa pathology is necessary. In this case, urgent surgical resection allowed for a positive patient outcome. Description of such a case may contribute to understanding of similar presentations in the neurosurgical context., (Crown Copyright © 2019. Published by Elsevier Ltd. All rights reserved.)

A 14-year-old previously healthy female was transferred from a local emergency department after being found unresponsive at home. Parental questioning revealed she had fever and pharyngitis 2 weeks before presentation. Past mental health history was negative, including concern for past or present suicidal ideation/attempts, suspected substance use, or toxic ingestion. In the emergency department, she was orotracheally intubated due to a Glasgow Coma Scale of 3. She was hemodynamically stable and euglycemic. Electrocardiogram showed sinus tachycardia. She underwent a noncontrast head computed tomography that was normal and subsequently underwent a lumbar puncture. She had a seizure and was given a loading dose of diazepam and fosphenytoin that led to cessation of extremity movements. She was subsequently transferred to the PICU for additional evaluation. Initial examination without sedation or analgesia demonstrated dilated and minimally responsive pupils, intermittent decorticate posturing, and bilateral lower extremity rigidity and clonus, consistent with a Glasgow Coma Scale of 5. Serum studies were unremarkable with the exception of mild leukocytosis. Chest radiograph only showed atelectasis. She was empirically started on antibiotics to cover for meningitis pending final cerebral spinal fluid test results. The pediatric neurology team was consulted for EEG monitoring, and the patient was eventually sent for computed tomography angiogram and magnetic resonance angiogram/venogram. We will review diagnostic evaluation and management of an adolescent patient with acute encephalopathy with decorticate posturing of unclear etiology., (Copyright © 2017 by the American Academy of Pediatrics.)

The control of body posture is a complex activity that needs a very close relationship between different structures, such as the vestibular system, and the muscle and joint receptors of the neck. Damage of even one of these structures can lead to abnormal postural reflexes. We describe a case of a woman with a left pontine ischaemia who developed a 'dystonic' extensor posture of the left limbs while turned on the right side. This clinical picture differs from previous reports on the subject, and may relate to ischaemic damage of a pontine structure involved in posture control, or of adjacent neural connections to be yet identified. To the best of our knowledge, this is the first case reported in the literature. Clinical examples of an altered interplay between vestibular and neck receptors are rare., (2015 BMJ Publishing Group Ltd.)

Aim: To determine the pathomorphological and clinical background to decerebrate posturing in humans following serious traumatic brain injury., Materials and Methods: One hundred and twenty patients who had been unconscious for more than 24 h underwent diagnostic MRI within 8 days after trauma. The presence of decerebrate rigidity as the clinical parameter was correlated to MRI findings, such as traumatic lesions in defined brain areas. Significance was presumed as p < 0.05., Results: On the day of MRI 43 (36%) patients exhibited decerebrate posturing: 19 (23%) cases were unilateral and 24 (77%) bilateral. There was a significant correlation between midbrain lesions and the presence of rigidity. If a midbrain lesion was found in the absence of pontine lesions, decerebrate rigidity could be concluded (p < 0.05). There was no significant correlation to the rigidity in the case of midbrain lesions accompanied by pontine lesions, and no correlation to the rigidity could be detected for other regions of the brain. Both the occurrence of decerebrate posturing and the detection of brainstem lesions at MRI correlated with the Glasgow Outcome Scale. The combination of both parameters improved the probability of predicting the outcome., Conclusion: The rate of decerebrate posturing increases significantly in the presence of midbrain lesions. The presence of pontine lesions appears to be of secondary importance. The chances of predicting the Glasgow Outcome Scale are improved by the combination of clinical information (decerebrate posturing) and radiological parameters (type of brainstem lesion)., (Copyright © 2014 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Recent studies of filmed hangings have documented the agonic sequences in these deaths. Considering this agonic sequence, one question comes to mind: which of these responses is an indicator of irreversible damage? Since decerebrate rigidity points toward lesions of the midbrain, it was initially thought that this posture could be an indicator of severe potentially irreversible damage. However, we here present a series of nonlethal filmed hangings by a 35-year-old male autoerotic practitioner, which will prove otherwise: in a film of an interrupted hanging, a decerebrate pattern of rigidity was observed at 20 seconds. However, the man later regained consciousness and seemed to present a full recovery without any noticeable symptoms. The scientific basis for the generalized assumption that death by hanging occur in 3 to 5 minutes will be reviewed. Though this estimation of the time is certainly precise and accurate enough for the needs of clinicians, it will be demonstrated that scientific evidence are not strong enough to be used in court. So how long does it take to suffer irreversible damage by hanging or by strangulation? The only honest and scientifically valid answer seems to be that we do not know.

A 25-years-old man experienced fever and diarrhea. Ten days later he noticed difficulty walking (day 1). On admission neurological examination revealed lethargy, dysarthria and weakness of limbs. Oculocephalic response was not be elicited and extensor toe signs were positive. In spite of treatment with aciclovir and methylprednisolone, he continued to show progressive deterioration developing to coma with decorticate posture. Autonomic symptoms (hyperhidrosis, hypersalivation and fever) and groaning were observed. Brain magnetic resonance image and brainstem evoked potential presented no abnormality, but electroencephalographic study showed a spindle pattern indicating spindle coma. Laboratory tests including cerebrospinal fluids showed no specific results. High-dose immunoglobulin was administered from day 6, and his consciousness level improved. External ophthalomoplegia and ataxic gait were observed after he became more alert. Because he had IgG type anti-GQ1b antibodies in the serum, a diagnosis was made of Bickerstaff's brainstem encephalitis (BBE). Six months after discharge he had complete resolution of his symptoms. This is the first report of spindle coma observed in a case of serologically confirmed BBE.

The Working Group on Human Asphyxia has analyzed 14 filmed hangings: 9 autoerotic accidents, 4 suicides, and 1 homicide. The following sequence of agonal responses was observed: rapid loss of consciousness in 10 ± 3 seconds, mild generalized convulsions in 14 ± 3 seconds, decerebrate rigidity in 19 ± 5 seconds, beginning of deep rhythmic abdominal respiratory movements in 19 ± 5 seconds, decorticate rigidity in 38 ± 15 seconds, loss of muscle tone in 1 minute 17 seconds ± 25 seconds, end of deep abdominal respiratory movements in 1 minute 51 seconds ± 30 seconds, and last muscle movement in 4 minutes 12 seconds ± 2 minutes 29 seconds. The type of suspension and ethanol intoxication does not seem to influence the timing of the agonal responses, whereas ischemic habituation in autoerotic practitioner might decelerate the late responses to hanging.

The corticospinal tract is not incriminated in decerebrate rigidity (DR). However, this has not yet been proven in humans. We applied transcranial magnetic stimulation (TMS) in a decerebrate patient to support the hypothesis. A patient suffering from pontine hemorrhage with the fourth ventricular extension was admitted unconscious and in a decerebrate posture. Five days later, she regained consciousness but remained in a decerebrate posture. Motor-evoked potentials (MEPs) to TMS were measured 1 week after she had regained consciousness, and this provoked muscle responses in her hands and feet bilaterally. During the follow-up, the patient's muscle tone became persistently flaccid, although her strength increased to varying degrees in different body and limb muscles. She remained bedridden for 3 years after the stroke and could neither turn on the bed by herself nor perform skilled movements using her hands. The findings of TMS confirmed the animal studies in that the mechanism of decerebrate rigidity did not come through a damage of the corticospinal pathway. This also implies that a preserved corticospinal tract function cannot guarantee a good motor recovery in a stroke patient., (Copyright © 2011. Published by Elsevier B.V.)

It has been proposed that filmed hangings may hold the key to a better understanding of human asphyxia, and The Working Group on Human Asphyxia was formed to systematically review and compare these video recordings. This study analyzed eight filmed hangings. Considering time 0 to represent the onset of the final hanging, rapid loss of consciousness was observed (at 8-18 sec), closely followed by convulsions (at 10-19 sec). A complex pattern of decerebrate rigidity and decorticate rigidity then followed. Between 1 min 38 sec and 2 min 15 sec, muscle tone seemed to be lost, the body becoming progressively flaccid. From then on, isolated body movements were observed from time to time, the last one occurring between 1 min 2 sec and 7 min 31 sec. As for the respiratory responses, all cases presented deep rhythmic abdominal respiratory movements (last one between 1 min 2 sec and 2 min 5 sec)., (© 2010 American Academy of Forensic Sciences.)

Hepatic encephalopathy is a syndrome of neuropsychiatric dysfunction caused by portosystemic venous shunting with or without the presence of intrinsic liver disease. Clinical presentations are variable ranging from an abnormal sleep pattern to somnolence and deep coma. Decerebrate and decorticate posturing, have been rarely reported with hepatic encephalopathy. We report a case of a 59-year-old-man with a history of Child-Pugh B liver disease secondary to chronic alcoholism who was admitted because of coma. He had a transjugular intrahepatic portosystemic shunt 3 months prior to his presentation. He was found to have decerebrating posture. He was treated for hepatic encephalopathy with complete recovery and resolution of the neurologic findings. The physician should be aware that decerebration and decortication posture can occur with hepatic encephalopathy and can be reversible.

Central nervous system symptoms occur in a substantial portion of patients with systemic lupus erythematosus. However, coma is a rare presentation and is usually secondary to complications such as subarachnoid hemorrhage, seizure, or ischemia. Here, we present a 49-year-old woman with lupus erythematosus and a history of recurrent aseptic meningitis and mild subarachnoid hemorrhage who presented with altered mental status and lethargy that progressed rapidly over hours to the herniation syndrome of coma, extensor posturing, and unilateral pupillary dilation. Spinal fluid showed massive protein elevation (>1600), and head computed tomography revealed global cerebral edema. The clinical and radiologic findings rapidly reversed with intravenous corticosteroids and mannitol within 24 hours, and her mental status improved to baseline. Her course was complicated by 2 episodes of recurrent encephalopathy when corticosteroids were tapered; these resolved after resuming high dosing. Because of ongoing pancytopenia, chemotherapy immunosuppression was delayed, and instead she received intravenous immunoglobulin with improvement in the pancytopenia. She remained cognitively intact during subsequent corticosteroid tapering. Rapid development of coma in lupus patients may be due to a primary process of the disease impacting blood brain barrier integrity. Although rare, this potentially fatal complication may be reversible with acute corticosteroid administration.

Vertex epidural haematomas (VEDH) are rare and difficulties are encountered in diagnosis and management. This is a case report of a patient with a vertex epidural haematoma who presented with signs of severe head injury with upper limb decerebrate posture. We discuss the challenges of radiological investigation and neurosurgical management of VEDH.

We present the case of a 13-year-old boy with a ruptured cerebral arteriovenous malformation who had rapidly progressive cardiac failure leading to death. Serial electrocardiograms, cardiac enzymes, echocardiograms, and pulmonary artery catheter data confirmed severe ventricular dysfunction related to myocardial ischemia and infarction. Cardiac dysfunction after cerebral insult is commonly described in adults with aneurysmal subarachnoid hemorrhage and has been termed "neurogenic stunned myocardium" because of its transient nature in most of patients. In children, cardiovascular dysfunction has been described in a few reports and only after traumatic brain injury. No deaths have been reported. This case report illustrates the potentially lethal consequences of cardiovascular dysfunction in children after ruptured cerebral arteriovenous malformation with subarachnoid hemorrhage. Compromised cardiac function should be considered during the early evaluation and management of these patients and supportive care instituted to limit secondary brain injury from poor perfusion.

The human pathophysiology of asphyxia by hanging is still poorly understood, despite great advances in forensic science. In that context, filmed hangings may hold the key to answer questions regarding the sequence of events leading to death in human asphyxia. Four filmed hangings were analyzed. Rapid loss of consciousness was observed between 13 sec and 18 sec after onset of hanging, closely followed by convulsions (at 14-19 sec). A complex pattern of decerebration rigidity (19-21 sec in most cases), followed by a quick phase of decortication rigidity (1 min 00 sec-1 min 08 sec in most cases), an extended phase of decortication rigidity (1 min 04 sec-1 min 32 sec) and loss of muscle tone (1 min 38 sec-2 min 47 sec) was revealed. Very deep respiratory attempts started between 20 and 22 sec, the last respiratory attempt being detected between 2 min 00 sec and 2 min 04 sec. Despite differences in the types of hanging, this unique study reveals similarities that are further discussed.

Objectives: Animal and human studies support mild therapeutic hypothermia as an effective means of preventing brain injury in comatose patients resuscitated from cardiac arrest. However, there is little clinical experience with predicting neurologic outcome in this patient population. We present 4 comatose patients resuscitated from cardiac arrest treated with mild hypothermia whose in-hospital neurologic prognosis was determined by board-certified neurologists to be grave, yet were ultimately discharged from the hospital with no or minimal neurologic sequelae., Results: We report 4 comatose patients resuscitated from cardiac arrest treated with mild hypothermia. On hospital admission, all patients had a Glasgow Coma Score between 3 and 6 and a FOUR Score between 1 and 5. Mild hypothermia (32 degrees C - 33 degrees C) was implemented for 24 to 40 hours. Examination by board-certified neurologists before and during hypothermia or the rewarming phase suggested a grave prognosis. All 4 patients had sudden and dramatic neurologic recovery 9 to 24 hours after rewarming to normothermia and were ultimately discharged with no or minimal neurocognitive sequelae., Conclusion: This case series suggests that neurologic assessment-based prognosis of patients after cardiac arrest undergoing therapeutic mild hypothermia should be considered unreliable for at least the first 72 hours. Use of additional assessments such as brain injury markers or evoked potentials, in addition to clinical examination, should be strongly considered to help determine an estimated prognosis. Functional reversibility after a global insult could be an intrinsic potential of the brain, similar to myocardial stunning, and deserves investigation.

A 24-year-old male came to the emergency department with a diaphyseal fracture of the femur resulting from a motorcycle accident. Neurological deterioration was progressive, although a computed tomography scan was normal. Endotracheal intubation for mechanical ventilation was necessary. His condition progressed to sepsis and multiorgan failure before resolving. Magnetic resonance images of the brain suggested a fat embolism. The presence of a patent foramen ovale was investigated. The patient remained in a state of coma vigil for 3 months after the accident. After ruling out other more likely causes of neurological deterioration after trauma with fractures, fat embolism should be suspected. The prognosis for the neurological manifestations of fat embolism syndrome are generally good. Severe cases suggest massive (paradoxical) embolization of the brain and are associated with a patent foramen ovale. Early diagnosis will identify the patient at high surgical risk. A favorable course and outcome have been reported with preoperative closure of the foramen ovale.

Background: Abnormal motor posturing is often observed in children with cerebral malaria, but the aetiology and pathogenesis is poorly understood. This study examined the risk factors and outcome of posturing in Kenyan children with cerebral malaria., Methods: Records of children admitted to Kilifi district hospital with cerebral malaria from January, 1999 through December, 2001 were reviewed for posturing occurring on or after admission. The clinical characteristics, features of raised intracranial pressure, number of seizures and biochemical changes in patients that developed posturing was compared to patients who did not., Results: Of the 417 children with complete records, 163 (39.1%) had posturing: 85 on admission and 78 after admission to hospital. Decorticate posturing occurred in 80, decerebrate in 61 and opisthotonic posturing in 22 patients. Posturing was associated with age > or = 3 years (48.1 vs 35.8%, p = 0.01) and features of raised intracranial pressure on funduscopy (adjusted OR 2.1 95%CI 1.2-3.7, p = 0.009) but not other markers of severity of disease. Unlike decorticate posturing, decerebrate (adjusted OR 1.9 95%CI 1.0-3.5) and opisthotonic posturing (adjusted OR 2.9 95%CI 1.0-8.1) were, in addition, independently associated with recurrence of seizures after admission. Opisthotonus was also associated with severe metabolic acidosis (OR 4.2 95%CI 3.2-5.6, p < 0.001). Thirty one patients with posturing died. Of these, 19 (61.3%) had features suggestive of transtentorial herniation. Mortality and neurological deficits on discharge were greatest in those developing posturing after admission., Conclusion: Abnormal motor posturing is a common feature of cerebral malaria in children. It is associated with features of raised intracranial pressure and recurrence of seizures, although intracranial hypertension may be the primary cause.

We reported a 77-year-old woman with atrial fibrillation. She was admitted to our hospital because of bradycardia and disturbance of consciousness. She regained consciousness soon after the admission, however on the 3rd day of admission, she abruptly fell into a coma. Neurological examination revealed decerebrate rigidity, conjugate eye deviation to the right, and bilateral Babinski signs, but remaining oculocephalic reflex in both vertical and horizontal directions. Diffusion-weighed MR image of the brain on the same day demonstrated extensive hyperintense lesions in the bilateral hemispheres, sparing the brainstem. On the duplex carotid ultrasonography just after the MR study, oscillating intraluminal thrombi occluded the right common carotid and the left internal carotid artery. We diagnosed the patient as having bilateral carotid occlusions by cardioembolic mechanism.

A 59-year-old man presented with severe dyspnea caused by advanced laryngeal cancer. As he had disregarded the dyspnea for a month, we did not have enough information about the extent of the tumor. The pulse oximeter showed 88% with oxygen inhalation. Because of severe dyspnea, he could not maintain supine position. Fiberoptic laryngoscopy showed tumor bulk obstructing airway directly. In the operating room, at first, a cricothyroid membrane puncture was attempted under local anesthesia but the procedure was abandoned when the patient became hypoxic and unconscious. Immediately tracheostomy and cardiopulmonary resuscitation were performed. Tumor bulk had displaced the trachea and surrounding structures, making a tracheostomy difficult. Nine min after loss of consciousness, a secure airway was obtained. However, he was still unconscious and developed characteristic decerebrate rigidity. Therefore the patient was treated with infusion of thiamylal and free radical scavenger and mild hypothermia therapy (bladder temperature 34 degrees C). On the fifth day of this treatment, after rewarming and discontinuation of thiamylal, the patient responded to command. He recovered with no neurological deficits. This case suggests that combined treatment with barbiturate, free radical scavenger, and mild hypothermia therapy is effective to minimize ischemic brain damage after cardiopulmonary resuscitation.

We experienced a case of dissociative stupor with decorticated posture in a 71-year-old woman after neck clipping of the brain aneurysm. Decorticated posture is observed with severe midbrain disorder caused by brain herniation. In this case, therefore, severe brain stem damage was suspected, although light reflex was observed and respiration was stable. In addition, in this case, the aneurysm was located at the junction of the internal carotid artery and the posterior communicating artery. Therefore, surgical damage to midbrain was not likely to have happened. Postoperative computed tomography showed no abnormality in this region. Two days later, her symptom disappeared, and she could clearly recollect memory of these two days just after surgery. However, she could not follow directions because of a sense of fear. Thus her symptom might be based on a kind of mental disorder. Patients with conversion disorder react abnormally to stimulus or stress. Stupor was diagnosed as due to defect of reaction to stimulus of sound, light and touch. She was diagnosed as dissociative stupor of the conversion disorder type. In this case, decorticated posture was caused by mental disorder. We have to pay attention not only to brain tissue damage but also to psychological damage of patients.

A 9-year-old female presented with daily episodes of medically refractory paroxysmal bilateral arm posturing, which had long been thought to be epileptic seizures. She also had other types of episodes, including daily staring spells and infrequent generalized tonic-clonic convulsions. Neurologic examination was normal except for delayed cognitive development. The results of previous electroencephalograms (EEG) were normal, and magnetic resonance imaging of the head revealed a Chiari I malformation. Video EEG monitoring revealed no EEG changes during the attacks, and magnetic resonance imaging of the spine revealed a large cervical syrinx associated with the Chiari malformation. The episodes of paroxysmal bilateral dystonic arm posturing resolved after surgical intervention for the syrinx. This report illustrates that cervical cord disease is an unusual although potentially treatable condition to be considered in the differential diagnosis of paroxysmal episodes with dystonic movements of the arms, even in the absence of other physical findings of myelopathy.

Anesthesia is an essential element during the induction of ischemia/reperfusion and cerebral blood flow (CBF) measurement in most animal models. Cerebral neuroprotection and intrinsic effects on CBF afforded by anesthetics are confounding variables in those models. A new model of global forebrain ischemia/reperfusion (GFIR) in awake rats is presented and characterized. Rats underwent permanent occlusion of the basilar, and the paired pterygopalatine, external carotid, and occipital arteries. Inflatable balloon occluders were inserted around both common carotids, the nine-vessel occlusion (9VO) preparation. A subgroup of 9VO rats underwent placement of a laser Doppler flowmetry (LDF) probe for measurement of cortical CBF. Twenty-four hours later, while awake, 9VO rats were subjected to 10 min of ischemia by occluding both common carotid arteries. Blood gases, glucose and hematocrit were analyzed before and during ischemia, and for up to 90 min during reperfusion. Behavioral observations and continuous LDF CBF and mean arterial blood pressure determinations during ischemia and reperfusion were made. Rats were rendered comatose and decerebrate rigidity was observed during 9VO. Following balloon deflation, rats immediately regained the righting reflex and achieved complete recovery in the next 24 h. Moderate hyperglycemia was observed at 5 min of ischemia and up to 90 min reperfusion in 9VO rats. LDF CBF decreased to 5% of baseline and remained unchanged during ischemia. The 9VO is a reproducible recovery model of GFIR. Behavioral and LDF CBF correlates are consistent and survival studies are feasible.

Objective: Although Japanese encephalitis (JE) has been reported to be associated with seizures, there is no report on its frequency, pattern and severity and their correlation with electroencephalography (EEG) and radiological findings. In this communication, the analysis of seizures in 65 JE patients seen during 1991-1999 is presented., Methods: The diagnosis of JE was based on clinical, radiological and serological criteria. The patients were subjected to a detailed clinical evaluation. Frequency and pattern of seizures and other neurological findings were recorded. Cranial CT scan or MRI was carried out in all the patients. Electroencephalograph was recorded using the 10-20 system of electrode placement. Patients' outcome was defined, on the basis of clinical status for 3 months, into poor, partial and complete recovery. The relationship between seizures and other clinical, radiological and EEG parameters was evaluated by the Z test of proportion using the two-tail approach., Results: The patient's age ranged between 2 and 57 years, 24 were females and 19 were below 13 years of age. Thirty patients had seizures within the first week of encephalitis. The seizures were generalised tonic-clonic in 17 patients and partial motor with secondary generalization in 13 patients. Eleven patients had a single seizure, 8 had two seizures and 11 had multiple seizures. Two patients were presented with status epilepticus. Interictal EEG in the patients with seizures revealed theta to delta slowing in all patients and epileptiform discharges in four patients only. MRI revealed thalamic lesions bilaterally in 24, cortical in 7, basal ganglia in 8 and brainstem in 3 patients out of 26 patients with seizure. In the seizure group, three patients died, nine had poor, eight had partial and nine had complete recovery. Upon comparison of the JE patients with and without seizures, Glasgow coma scale (GCS) score, focal weakness, EEG slowing and cortical and thalamic lesion on CT or MRI were significantly related to the occurrence of the seizures; however, it was not associated with poor outcome., Conclusions: JE is associated with seizures in 46% of the patients in the acute stage of encephalitis which is easily controlled by monotherapy. Patients with severe encephalitis were associated with higher frequency of seizures.

A 72-year-old male presented with a post-traumatic false aneurysm of the right proximal M2 artery with massive subarachnoid hemorrhage after closed head injury. Serial computed tomography (CT) and angiography showed the development of the aneurysm which was verified at autopsy. He was admitted in a drowsy state just after a motorcycle accident. Initial brain CT showed subarachnoid hemorrhage without skull fracture. Follow-up brain CT showed a huge hematoma in the right temporal lobe. He died 47 hours after the accident. Histological examination of the aneurysm showed a false aneurysm. Delayed diagnosis of traumatic aneurysms leads to high mortality, so early surgical treatment is essential to save such patients.

A 46-yr-old man with dysaesthesia (burning sensation) following herpes zoster in the left upper chest region was treated with a single thoracic (T2/T3) epidural injection (1.0% lidocaine 3 ml + 0.125% bupivacaine 3 ml) as an outpatient. Twenty minutes after the injection, a nurse noticed the patient to be unconscious with dilated pupils, apnoea and cardiac arrest. Following immediate cardiopulmonary resuscitation, the patient was treated with an i.v. infusion of thiamylal sodium 2-4 mg kg-1 h-1 and his lungs were mechanically ventilated. When the patient developed a characteristic decorticate posture, mild hypothermia (oesophageal temperature, 33-34 degrees C) was induced. On the 17th day of this treatment, after rewarming (35.5 degrees C) and discontinuation of the barbiturate, the patient responded to command. Weaning from the ventilator was successful on the 18th day. About 4 months after the incident, the patient was discharged with no apparent mental or motor disturbances. We suggest that mild hypothermia with barbiturate therapy may have contributed to the successful outcome in this case.

A case is described in which, following a motorcycle accident resulting in a closed head injury, a young woman was rendered unconscious. On examination she was found to have unilateral decerebrate rigidity. Further clinical and radiological examination revealed that there was no damage to the limbs, vertebral column, or spinal cord. Readers are expected to be able to make an anatomical diagnosis on the basis of the facts presented.

A 56-year-old female presented with chronic subdural hematoma due to spontaneous (primary) intracranial hypotension (SIH). She was admitted in a deep coma. Computed tomography showed bilateral chronic subdural hematomas with tight basal cisterns. Percutaneous aspiration of the chronic subdural hematoma with the twist drill revealed negative pressure. The neurological symptoms rapidly improved postoperatively, but the chronic subdural hematoma recurred three times, requiring further aspiration procedures. Patients with SIH may develop subdural hematoma requiring surgical treatment.

Bilateral craniectomy in a woman comatose and decerebrate after a subarachnoid hemorrhage, resulted in normal mentation in 3 days. The dramatic recovery is unmatched in our experience. The rationale rested on clinicopathologic studies showing that in such cases brain swelling was the cause of death, the brain being otherwise intact. Emergency surgery to relieve the tamponade seemed reasonable. The question is whether the procedure has a role in those patients who fail to respond to current therapeutic measures. The arguments in favor, presented herein, are illustrated by three pathologic studies selected from our longtime experience.

A case of treatment-related leukoencephalopathy is presented. A patient with medulloblastoma was postoperatively treated with craniospinal axis irradiation. One month after irradiation, weekly intrathecal administration of methotrexate was performed 4 times to treat cerebrospinal fluid dissemination of the tumor. Two months after the initiation of intrathecal chemotherapy, the patient became somnolent and developed decerebrate posturing. Magnetic resonance imaging showed diffuse leukoencephalopathy. Positron emission tomography revealed a diffuse decrease in glucose uptake in the deep white matter. Auditory evoked potential also showed diffuse abnormalities, not only in the cerebrum, but also in the brain stem. High dose intravenous leucovorin rescue was attempted without any neurologic improvement.

Decerebrate rigidity is one of several reversible neurological abnormalities which have been observed in the setting of metabolic coma. We present the case of a patient who recovered fully from prolonged decerebrate rigidity associated with hypoglycemic coma. This case emphasizes the possibility of recovery from severe, prolonged hypoglycemia.

A case of stable decerebrate posture in the upper limbs following sudden loss of consciousness and prolonged coma is described. The patient recovered most of her cognitive functions and gait, without clinical, neurophysiological or neuroradiological evidence of brainstem lesion. MRI shows borderzone infarcts. It is suggested that anoxic-ischemic cortical damage, affecting specially corticoreticular neurons, could explain the development of decerebrate rigidity in patients without apparent brainstem lesion.

Certain decerebrate lesions of brain stem or hypothalamus induce pharmacologically reversible hypertension and hyperthermia in animals. We observed three young patients with episodic decerebration, hyperthermia, hypertension, and hyperventilation during recovery from comas of different etiologies. The shared pathology on neurologic examinations and computed tomographic scans was hypothalamic-mesencephalic dysfunction, suggesting a diencephalic-brain-stem disconnection syndrome or brain-stem release mechanism. Propranolol was the most effective drug tested, but only two patients responded, one dramatically. This novel clinical syndrome may have localizing and therapeutic significance in pediatric coma that needs to be further defined in future studies.

Nearly drowned children sustaining cardiac or respiratory arrest or altered neurologic status developed anoxic encephalopathy (30%), died in the ensuing days (36%), or were discharged neurologically normal (34%). In encephalopathic children, spasticity was more malignant than in children with other causes of spasticity. The most common musculoskeletal problem was contracture, especially equinus, hip adductor, hamstring, and quadriceps. Hip subluxation or dislocation occurred in 34% as early as 1 month after injury. Scoliosis developed in 18%, with some requiring bracing and surgery. Thirty-one percent ambulated, and the rest were quadriplegic. Independent sitting or better function by discharge was a predictor of ambulation potential. Of the quadriplegics, 66% were dependent sitters.

From these 196 aneurysms of high grade at the admission 61% die, but 16% have a good recovery. The high mortality of early surgical operations must be moderate by the seriousness of rebleeding in attempting a further surgery.

The behaviour of the motor activity was investigated in a selected group of neurosurgical patients with diverse cerebral and spinal lesions, as well as in a group of healthy controls. Under standard conditions the EMGs were simultaneously recorded from six muscles, and the time-voltage integral of the muscle action potentials were continuously recorded. In the foregoing work the behaviour of the motor activity in the decerebrate state and its sequelae is described.

Causes of head injury in childhood depend on age. In contrast to the adults there are differences in pathophysiological mechanisms and in the site of lesions. Brain stem disorders are more frequent, superficial brain damage is less frequent than in children. Multiple lesions of the limbic system are as well important as those ones of the mesencephalon. Most of the cases of apallic syndromes in childhood can be due to these lesions.