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Astrocytoma

Astrocytomas are intracranial tumors derived from astrocytes cells of the brain. They can have narrow or diffuse zones of infiltration. more...

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Grading

Astrocytomas have great variation in their presentation. WHO acknowledges the following grading system for astrocytomas:

  • WHO Grade 1 — pilocytic astrocytoma - accounts for 5% of all reported brain tumors, with a median age at diagnosis of 12 years. This brain tumor is primarily pediatric, although it is also found in adults.
  • WHO Grade 2 — diffuse astrocytoma
  • WHO Grade 3 — anaplastic (malignant) astrocytoma - accounts for 7% of all primary brain tumors, with the median age at diagnosis of 51 years of age.
  • WHO Grade 4 — glioblastoma multiforme (most common) - accounts for 45% of all reported brain tumors, with the median age at diagnosis of 64 years of age.

In addition to these four tumor grades, astrocytomas may combine with oligodendrocytes to produce oligoastrocytoma. Unique astrocytoma variants have also been known to exist.

Symptoms

Although there is variation in initial presentation, in many cases, the first symptom of an astrocytoma is the onset of seizure activity or severe headache. Presentation will vary depending upon the astrocytoma grade, the location of the tumor, among other factors. A Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) scan is necessary to characterize the anatomy of this tumor (size, location, heter/homogeneity). However, final diagnosis of this tumor, like most tumors, relies on histopathologic examination (biopsy examination).

Treatment

A surgeon will typically remove as much of the tumor as he or she can without damaging other critical, healthy brain structures. Often, surgery is followed up by chemotherapy, radiation, or a mix of both. Therapy may be more or less aggressive, depending upon the tumor behavior and patient condition.

Astrocytomas often reappear - the reoccurrence of the tumor are often visible on MRI. The recurrent tumors are then treated similarly as the initial tumor, with sometimes more aggressive chemo or radiation therapy.

There is great life expectancy variation between different subsets of brain tumor. Age and initial diagnosis are often related to survival time.

The prognosis is worst for Grade 4 gliomas, with an average survival time of 14-18 months. Overall, the five year survival rate is 5%.

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Astrocytoma presenting with apnea and sinus arrest
From CHEST, 10/1/94 by Natarajan Rajagopoalan

Although some vascular lesions of the central nervous system are known to cause abnormalities in the control of breathing, association between astrocytoma and combined cardiorespiratory abnormalities in the adult is distinctly unusual. We present a case of a 52-year-old man whose only features of astrocytoma consisted of episodes of apnea and sinus arrest followed by prolonged alveolar hypoventilation. These abnormalities resolved after resection of the tumor. We conclude that in patients presenting with respiratory and sinus arrest, the search for a cause should not be limited to the cardiac and pulmonary systems, but it must include a possibility of a brain tumor. (Chest 1994; 106:1301-03)

In a patient presenting with apnea, alveolar hypoventilation, and cardiac arrhythmia, the cause is usually assumed to be either a vascular lesion in the central nervous system, drug overdose, or primary alveolar hypoventilation. Limbic system seizures are not frequently considered in this setting. We present a patient with apnea and sinus arrest as the only physiologic signs of the limbic system seizures secondary to astrocytoma.

CASE REPORT

A 52-year-old man presented to the emergency department because of two episodes of loss of consciousness at work. These episodes lasted a few seconds, were not preceded by any neurologic or cardiac symptoms, and were not associated with exertion. There were no positictal symptoms or signs, although on one occasion the patient complained of a mild headache. The patient denied any substance abuse (including alcohol), was not taking medications, and stopped smoking 20 years ago.

After arrival to the emergency department, the patient was observed to have two more similar episodes. The first one was very brief; the patient looked vacantly into space for about 1 s. The second episode lasted about 4 s, during which the patient was unresponsive, apneic, and pulseless. An ECG showed sinus arrest, and arterial blood gases on room air were [Po.sub.2] of 67 mm Hg, [Pco.sub.2] of 64 mm Hg, and pH of 7.27. The patient was given supplemental oxygen. His sinus arrest ended with a junctional escape beat followed by a sinus rhythm of 70/min. Simultaneously, respiration resumed and the patient became responsive.

Physical examination revealed a slightly drowsy, but fully oriented man in no distress. His BP was 120/70 mm Hg, heart rate of 70/min, and respiratory rate of 14/min. There were no abnormal respiratory, cardiac, or necrologic findings. Laboratory investigations--serum electrolytes, glucose, calcium, CBC count, electrocardiogram, and chest radiograph--were all normal. His arterial blood gases 8 h after the initial episode still showed marked alveolar hypoventilation with [Pco.sub.2] of 58 mm Hg, [Po.sub.2] of 62 mm Hg, and pH of 7.29.

A temporary venous pacemaker was inserted strictly as a prophylactic measure. The patient had no further episodes of apnea or sinus arrest. Electroencephalogram performed the day after showed focal slow waves over the right temporal area. Computed tomography of the brain revealed a large mass in the right temporo-occipital area. This was eventually resected surgically and the disease revealed an astrocytoma. At the time of discharge from the hospital, arterial blood gases were pH 7.40, [Po.sub.2] 86 mm Hg, and [Pco.sub.2] 42 mm Hg.

Four months after surgery, the patient presented with headaches and episodes of "staring into space," without any further episodes of respiratory or sinus arrest. He was found to have a recurrence of the tumor that was surgically debulked and the patient was discharged to a convalescent hospital on steroids (Dexamethasone) and anticonvulsants.

DISCUSSION

We describe a patient with apnea and sinus arrest as the initial presentation of astrocytoma, and we postulate that these physiologic abnormalities were the only manifestations of limbic system seizures in this patient.

In 1899 Jackson(1) described respiratory arrest as a part of complex partial seizure. This was subsequently confirmed by ictal EEG monitoring which showed that initial apnea may be an integral manifestation of partial seizures often originating in the limbic system.(2)(3)

Autonomic nervous system manifestations of seizures have been studied experimentally during electrical and mechanical stimulation of different areas of the limbic system in animals and in humans. Respiratory arrest has been noted after stimulation of posterolateral frontal cortex, anterior insular cortex, anterior cingulate gyrus, amygdala, uncus, and fornix, which consist of most of what we now know as the limbic system.(4)

Kaada and Jasper(4) pointed out that during experimentally induced respiratory arrest, patients could still be conscious and responsive and, in fact, can overcome apnea when commanded to speak. Their articulation, however, was poor because of poor control of breathing. Consciousness was not necessarily lost during apneic seizures, which was later confirmed by Watanabe et al.(5) They reported five children who had apneic attacks without loss of consciousness as manifestation of limbic system seizures. Rovener and Barron(6) observed intermittent respiratory arrest in a patient who had epilepsia partialis continua due to occlusion of right anterior cerebral artery. electroencephalographic recordings showed periodic spike discharge from the right frontocentral area accompanied by respiratory arrests lasting from 5 to 8 s. There are case reports of epileptics with prolonged respiratory insufficiency not associated with tonic-clonic movements and with simultaneous EEG evidence of seizure activity.(7)

Apnea is unusual as the initial manifestation of seizure beyond the neonatal period. Initial reports of apneic seizure described tachycardia as the associated cardiovascular response. Fenichel et al(8) described bradycardia accompanying as favoring nonconvulsive apnea in infants. Subsequently Coulter(9) described partial complex seizures with apnea and bradycardia. Van Buren et al(10) made careful observations in both spontaneous and induced epileptic attacks and noted apnea and bradycardia.

Our patient is unusual in that he had episodes of sinus arrest and apnea without other overt features of seizure activity. Interictal EEG seldom shows seizure activity; slow waves showed in our patient merely reflect the presence of the tumor. We did consider a possibility that cardiac events (sinus arrest) led to apnea; however, no cardiac disease was shown in our patient, the pacemaker was never used and later removed, and after tumor resection there were no further episodes of either apnea or sinus arrest. To our knowledge, the combination of autonomic features that includes respiratory and sinus arrest, has not been previously reported as a manifestation of limbic system epilepsy. Kelly et al(11) described an infant who had episodes of cyanosis and apnea and was later found to have astrocytoma in the left temporal lobe. Valente et al(12) recently described a young patient with astrocytoma who had repeated episodes of coma and apnea. This patient had stable alveolar hypoventilation with well-compensated respiratory acidosis and polycythemia, suggesting that the abnormal respiratory control was probably long standing. Our patient had only transient hypoventilation that was probably because of an electrical disturbance in the limbic system resulting in abnormal regulation of breathing.

REFERENCES

(1)Jackson JH. On asphyxia in slight epileptic paroxysms. Lancet 1899; 1:79-80

(2)Johnson LC, Davidoff RA. Autonomic changes during paroxysmal EEG activity. Electroencephalogr Clin Neurophysiol 1964; 17:25-35

(3)Mirsky AF, van Buren JM. On the nature of the "absence" in centrencephalic epilepsy. Electroencephalogr Clin Neurophysiol 1965; 18:334-38

(4)Kaada BR, Jasper H. Respiratory responses to stimulation of temporal pole, insula, and hippocampal and limbic gyri in man. Arch Neurol Psychiatr 1952; 68:609-19

(5)Watanabe K, Hara K, Hakamada S, Negoro T, Sugiura M, Matsumoto A, et al. Seizures with apnea in children. Pediatrics 1982; 79:87-90

(6)Rovener RN, Barron KD. A case of respiratory epilepsy. J Neurol 1966; 16:328

(7)Nelson DA, Wilmington D, Ray CD. Respiratory arrest from seizure discharges in limbic system: report of cases. Arch Neurol 1968; 19:199-207

(8)Fenichel GM, Olson BJ, Fitzpatrick JE. Heart rate changes in convulsive and non-convulsive neonatal apnea. Ann Neurol 1980; 7:577-82

(9)Coulter DL. Partial seizures with apnea and bradycardia. Arch Neurol 1984; 41:173-74

(10)Van Buren J, Jonone A, Marsan C. A correlation of autonomic and EEG components in temporal lobe epilepsy. Arch Neurol 1960; 91:683-703

(11)Kelly DH, Krishnamoorthy KS, Shannon DC. Astrocytoma in an infant with prolonged apnea. Pediatrics 1980; 66:429-31

(12)Valente S, De Rosa M, Culla G, Corbo GM, Ciappi G. An uncommon case of brainstem tumor with selective involvement of the respiratory centers. Chest 1993; 103:1909-10

COPYRIGHT 1994 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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