Neurology
A Rare Case of Diencephalic Seizures Secondary to Hemorrhagic Stroke Muhammed Zohaib Ghatala*, Swamikannu Murugan†, Shriraam Mahadevan‡, Soundarajan Booma#
Abstract Diencephalic seizure or sympathetic storm is a rare condition characterized by paroxysmal episodes of acute increase in heart rate, blood pressure, respiratory rate, temperature and diaphoresis with extensor posturing that can occur, following brain injury. Here we report a case of a 45-year-old male with hemorrhagic stroke who had symptoms of diencephalic seizure during his hospital stay.
Keywords: Diencephalic seizure, sympathetic storm, brain injury, hemorrhagic stroke
D
iencephalic seizure was first described by Penfield in 1929, in a patient with a cerebral disorder who had paroxysmal episodes of acute increases in blood pressure (BP), heart rate and respiratory rate with lacrimation and extensor posturing. Since then, there have been several reports of similar cases and the suggestion that these seizures were associated with cerebral autonomic dysfunction secondarily because no evidence of seizure activity was detected by electroencephalography (EEG). We report on a patient with an intracerebral hemorrhage who developed characteristics of diencephalic seizures. The patient responded well to sodium valproate and adrenergic blockers.
Case Report A 45-year-old male presented with acute onset left hemiplegia, progressive decrease in the level of consciousness and one episode of generalized tonicclonic seizure. His past medical history was significant for hypertension and residual right upper and lower limb weakness from a hemorrhagic stroke at the age
*Senior House Officer †Consultant Physician Dept. of Internal Medicine ‡Consultant Endocrinologist Sundaram Medical Foundation, Shanthi Colony, Anna Nagar, Chennai, Tamil Nadu #Senior Registrar Dept. of General Medicine St. Martha’s Hospital, Bengaluru, Karnataka Address for correspondence Dr Muhammed Zohaib Ghatala #67, AJ Block, 3rd Street Anna Nagar, Chennai - 600 040, Tamil Nadu E-mail:
[email protected]
of 31 years. He was also an alcoholic but there was no history suggesting substance abuse. He was on regular antihypertensive medication with long-acting calcium channel blockers. On examination, the patient had a BP of 150/90 mmHg, heart rate of 88/min, normal body temperature and as he was not able to maintain airway, he was intubated. The patient was in post-ictal state with a Glasgow coma scale (GCS) of 6/15, he had paucity of movements of the left upper and lower limb and his left pupil was 2 mm in diameter but reactive to light. Given the patient’s history and clinical exam, an urgent computed tomography (CT) scan of the brain was done, which showed hemorrhage in the right lentiform nucleus (2.5 × 5.6 × 3.9 cm) with intraventricular extension, moderate midline shift, contralateral obstructive hydrocephalus and ipsilateral edema. There was also evidence of left middle cerebral artery (MCA) gliosis and encephalomalacia. The patient was admitted in the intensive care unit and he was managed with labetalol for his BP, loading dose and then maintenance dose of phenytoin for seizure prophylaxis which was later changed to sodium valproate, anti-edema measures were taken with hypertonic fluids and nasogastric tube feeding was started. On Day 3, his sensorium improved, and he was opening eyes to painful stimulus with paucity of movements of limbs. On Day 4, tracheotomy was done and he was weaned off the ventilatory support. On Day 11, the patient dropped his BP because of which labetalol was discontinued. However, later on during his hospital course, his BP was high again and was managed with short-acting metoprolol and amlodipine.
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Neurology Discussion
Table 1. Vitals of Patient on Day 54 Showing Episodic Sympathetic Surge Time
BP
PR
T
RR
12 am
190/100
138
100.2
34
02 am
120/80
60
98.6
20
04 am
110/80
62
98.6
20
06 am
110/70
60
98.6
20
08 am
130/80
76
98.6
22
10 am
120/80
70
98.6
20
12 pm
120/80
61
98.6
22
02 pm
120/80
72
98.4
22
04 pm
130/90
76
98.4
18
06 pm
130/70
86
98.6
22
08 pm
186/110
126
99.4
32
10 pm
130/80
68
99
20
11 pm
160/90
104
99
38
The above table shows vitals on a randomly selected day (Day 54), for a better understanding of the episodic sympathetic surge. BP = Blood pressure in mmHg; PR = Pulse rate per minute; T = Temperature in degree Fahrenheit; RR = Respiratory rate per minute.
On Day 39, the hypertonic fluid infusion was discontinued. From Day 51, the patient was noticed to have repeated episodes of sudden onset tachypnea (RR-24-42/min), tachycardia (90-146/min), increased BP (180/100-200/130), diaphoresis with extensor posturing. These episodes occurred at an average of 3-4 times/day and lasted for 10-150 minutes. These episodes did not respond to conventional antiepileptic benzodiazepines. Diagnostic investigations for paroxysmal hypertension, arrhythmias and seizure activity were negative. Twenty-four hour urine for metanephrines was within normal limits. CT scan of the abdomen showed no evidence of an adrenal gland mass. EEG showed diffuse bilateral slow wave dysfunction with mild accentuation to right side. Dexamethasone suppression test ruled out hypercortisolism. Holter recording was unyielding. His thyroid profile was within normal limits. With this background, the patient was diagnosed with diencephalic seizure secondary to cerebral hemorrhage. These episodes of diencephalic dysautonomia responded moderately to α1-blocker, but had a significant response when we replaced the shortacting β1-blocker that the patient was already getting with a long-acting β1-blocker.
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Indian Journal of Clinical Practice, Vol. 24, No. 11, April 2014
Diencephalic seizure is a rare condition. It presents with autonomic dysregulation. Symptoms include intermittent hyperthermia, hypertension, tachypnea, tachycardia, diaphoresis and extensor posturing. In the literature, various terms have been used in reference to this condition like paroxysmal sympathetic storms, midbrain dysregulatory syndrome and paroxysmal autonomic instability with dystonia.1 This condition results from traumatic brain injury, intracranial hemorrhage, brain tumors, hydrocephalus and hypoxic brain injury. However, in our extensive literature search we found that traumatic brain injury was by far the most common cause of diencephalic seizure. It is still unclear as to what causes the sympathetic storm in these patients. Some of the possible causes include dysfunction of autonomic centers in the diencephalon (thalamus or hypothalamus) or their connection to cortical, subcortical and brainstem loci that control autonomic function and loss of parasympathetic inhibitory response to sympathetic surge produced under stress.2,3 It is primarily a diagnosis of exclusion. With our patient, we checked his thyroid profile, did an EEG, dexamethasone suppression test, 24 hours urine metanephrines and Holter monitoring before concluding that the sympathetic surge was because of diencephalic seizures. In one study of 814 patients with diencephalic seizures, the EEG findings were consistent with paroxysmal slow waves and 6 or 14 c/sec positive spikes.4 This condition can be managed with bromocriptine, clonidine, dantrolene, lorazepam, morphine sulfate and nonselective β-blockers such as propranolol.5 Usually, a combination of these drugs is used. Authors of various published studies in the past have stated that selective β1-blockers are not as effective as nonselective β-blockers or combined α1- and β-blockers but, interestingly, our patient responded well to a long-acting selective β1-blocker (metoprolol) in combination with sodium valproate. Conclusion Diencephalic seizure is a relatively rare condition characterized by sympathetic surge. As many physicians rarely see this condition during their medical practice, this is an often misdiagnosed condition. It is very important to recognize the symptoms and start the appropriate treatment. We suggest that these patients be treated with long-acting β-blockers in combination
Neurology with an anti-epileptic medication. In our case, the patient responded well to long-acting metoprolol and sodium valproate. References 1. Blackman JA, Patrick PD, Buck ML, Rust RS Jr. Paroxysmal autonomic instability with dystonia after brain injury. Arch Neurol 2004;61(3):321-8. Erratum in: Arch Neurol 2004;61(6):980. 2. Ramdhani NA, Sikma MA, Witkamp TD, Slooter AJ, de Lange DW. Paroxysmal autonomic instability with
dystonia in a patient with tuberculous meningitis: a case report. J Med Case Rep 2010;4:304. 3. Lemke DM. Riding out the storm: sympathetic storming after traumatic brain injury. J Neurosci Nurs 2004;36(1):4-9. 4. Shimoda Y. The clinical and electroencephalographic study of the primary diencephalic epilepsy or epilepsy of brain stem. Acta Neuroveg (Wien) 1961;23:181-91. 5. Levy ER, McVeigh U, Ramsay AM. Paroxysmal sympathetic hyperactivity (sympathetic storm) in a patient with permanent vegetative state. J Palliat Med 2011;14(12):1355-7.
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