Journal of the Neurological Sciences 355 (2015) 211–212
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Letter to the Editor A case of hypertensive brainstem encephalopathy presenting with severe headache and unilateral hearing loss Keywords: Brainstem encephalopathy Sensorineural hearing loss Hypertension
1. Introduction Hypertensive encephalopathy usually presents as posterior reversible encephalopathy syndrome (PRES), which is characterized by symmetrical vasogenic edema in the parieto-occipital areas where sympathetic innervation is relatively deficient compared with other areas [1,2]. The underlying pathological mechanism of PRES is the breakdown of cerebral autoregulation and endothelial dysfunction [2]. However, several reports have described a rare (about 4%) variant of PRES localized to the cerebellum and brainstem, rather than the posterior cerebrum [3–5]. This form of PRES is also referred to as hypertensive brainstem encephalopathy (HBE). As with typical PRES, severe hypertension, drugs, eclampsia, and chronic renal disease can cause HBE [3–5]. We herein describe an unusual case of HBE with severe headache and unilateral sensorineural hearing loss (SNHL) and discuss the potential pathomechanisms. 2. Case report A 32-year-old man was admitted with severe headache and suddenonset hearing impairment in the left ear. A mild headache had developed insidiously in the occipital area 1 week earlier and gradually progressed to severe, global head pain and discomfort. The patient said it was the most severe headache that he had ever experienced. The headache was pulsatile in nature and accompanied by nausea and vomiting. Hearing loss developed suddenly 2 days before visiting the hospital. He had been previously diagnosed with essential hypertension and treated with 50 mg atenolol and 30 mg sustained-release nifedipine per day for 15 years. He had stopped taking the medications of his own 1 month earlier. He had been smoking a pack of cigarettes per day for about 10 years. He was a social drinker. As for family history, the patient's father was diagnosed with essential hypertension in his twenties. The patient's blood pressure was 250/140 mm Hg. Prominent neck stiffness with Brudzinski's sign was observed. The Weber test lateralized to the right and the Rinne test was positive bilaterally. Pure tone audiometry revealed SNHL in the left ear (Fig. 1G). Brain MRI revealed severe vasogenic edema in the bilateral cerebellum, especially in the vermis and tonsil, which compressed the lower brain stem. Mild obstructive hydrocephalus developed due to narrowing of the fourth ventricle (Fig. 1A–D). Vestibular function test including the caloric reflex test revealed no abnormality.
http://dx.doi.org/10.1016/j.jns.2015.05.040 0022-510X/© 2015 Elsevier B.V. All rights reserved.
After administration of intravenous 20 mg nicardipine and 200 ml of 20% mannitol on the day of admission, his blood pressure dropped to 165/100 mm Hg and the headache was substantially relieved. On the second day of hospitalization, SNHL began to improve slowly and had almost returned to normal by the third day. A compatible finding was observed in the follow-up audiometry performed on the third day (Fig. 1H). A follow-up MRI performed on the same day revealed that vasogenic edema was much resolved and lower brainstem compression had subsided (Fig. 1E,F). A secondary hypertension work-up including measurement of serum renin/aldosterone, metanephrine, free T4, TSH, and urine vanillylmandelic acid levels revealed no abnormalities. After discharge, the patient has been treated with 50 mg atenolol, 10 mg amlodipine, and 40 mg olmesartan per day and has been symptom free for 5 months. 3. Discussion Patients with HBE require urgent medical care. Brainstem compression accompanied with elevated intracranial pressure may cause sudden neurological deterioration, irreversible neurologic damage, or death. However, symptoms are mostly reversible with proper treatment [3–5]. Therefore, early diagnosis and treatment are essential to avoid significant neurological damage. The development of SNHL in our patient was quite unusual. SNHL, especially unilateral SNHL, is rarely reported in patients with disorders causing increased intracranial pressure, such as idiopathic intracranial hypertension [6], hydrocephalus [7], cerebral venous sinus thrombosis [8], and Chiari malformation [9]. The exact mechanism of SNHL is uncertain in our case. However, it is possible that the elevated intracranial pressure was directly transferred to the cochlear perilymph, which is connected with subarachnoid CSF space via the cochlear aqueduct. The pressure difference between the perilymph and the endolymph may cause displacement of Reissner's membrane and thereby affect hearing [6–8]. Another possible mechanism is direct compression of the lower brainstem and vestibulocochlear nerve, which would cause hearing loss. Unfortunately, an auditory brainstem evoked response test was not performed during the hearing loss period, as it may have been helpful in differentiating between a cochlear, vestibulocochlear nerve, and brainstem lesion. Regardless of the pathogenesis, SNHL was reversed by acute reduction of blood and intracranial pressure. To the best of our knowledge, this is the first case report to describe a patient with unilateral SNHL associated with HBE. It is highly likely that the patient's willful cessation of chronic essential hypertension drugs initiated this unusual symptom of HBE. In patients with these symptoms, the possibility of HBE should therefore be considered. Rapid control of blood and intracranial pressure is crucial to prevent neurological deterioration and reverse SNHL. Conflict of interest/disclosures The authors have no conflict of interest in relation to this research and its publication.
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Letter to the Editor
Fig. 1. Initial (A–D) and follow-up (E, F) MRI of the patient and initial (G) and follow-up (H) pure tone audiometry. (A) Diffusion-weighted imaging showed patchy and subtle high signal intensities in the cerebellum. (B) An ADC image showed patchy and confluent high signal intensities in the bilateral cerebellum. (C) T2 weighted image showed diffuse high signal intensities in the bilateral cerebellum and a small and oval-shaped high signal intensity in the right pons. (D) T1 sagittal image showed anterior displacement of the lower brainstem and diffuse edema of the cerebellar vermis and tonsil. (E) On the follow-up image, diffuse edema of bilateral cerebellum decreased, the pontine lesion disappeared, and the fourth ventricular narrowing subsided after treatment. (F) Anterior displacement of the brain stem subsided compared to the initial MRI. Sensorineural hearing loss in the left ear was initially observed at admission (G), and this was much improved after treatment (H). R, right.
References [1] W.S. Bartynski, Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema, AJNR Am. J. Neuroradiol. 29 (2008) 1043–1049. [2] J.E. Fugate, D.O. Claassen, H.J. Cloft, D.F. Kallmes, O.S. Kozak, A.A. Rabinstein, Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings, Mayo Clin. Proc. 85 (2010) 427–432. [3] A.M. McKinney, B.D. Jagadeesan, C.L. Truwit, Central-variant posterior reversible encephalopathy syndrome: brainstem of basal ganglia involvement lacking cortical or subcortical cerebral edema, AJR 201 (2013) 631–638. [4] R. Bălaşa, S. Maier, E.G. Baubec, Z. Bajkó, A. Bălaşa, Cerebellar and brainstem variant of posterior reversible encephalopathy syndrome, Acta Neurol. Belg. (2015) (in press). [5] I. Karakis, J.A. Macdonald, M. Stefanidou, C.S. Kase, Clinical and radiological features of brainstem variant hypertensive encephalopathy, J. Vasc. Interv. Neurol. 2 (2009) 172–176. [6] P.J. Dorman, M.J. Campbell, A.R. Maw, Hearing loss as a false localizing sign in raised intracranial pressure, J. Neurol. Neurosurg. Psychiatry 58 (1995) 516. [7] V.J. Sammons, E. Jacobson, J. Lawson, Resolution of hydrocephalus-associated sensorineural hearing loss after insertion of ventriculoperitoneal shunt, J. Neurosurg. Pediatr. 4 (2009) 394–396. [8] A.C. Fonseca, L. Albuquerque, J.M. Ferro, Reversible bilateral hearing loss in a woman with cerebral venous thrombosis, J. Neurol. 255 (2008) 1825–1826.
[9] H. Dolgun, E. Turkoglu, H. Kertmen, E.R. Yilmaz, Z. Sekerci, Chiari type I malformation presenting with bilateral hearing loss, J. Clin. Neurosci. 16 (2009) 1228–1230.
Jung-Ju Lee Soohyun Cho Jong-Moo Park Department of Neurology, Eulji General Hospital, Eulji University College of Medicine, Hangeulbisekro 68, Noweongu, Seoul, Republic of Korea Kyung-Il Park Department of Neurology, Seoul Paik Hospital, Inje University College of Medicine, Mareunnae-ro 9, Jung-gu, Seoul, Republic of Korea Corresponding author at: Department of Neurology, Seoul Paik Hospital, Inje University, Mareunnae-ro 9, Jung-gu, Seoul 100-032, Republic of Korea. E-mail address: [email protected] (K.-I. Park). 16 April 2015
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Letter to the Editor A case of hypertensive brainstem encephalopathy presenting with severe headache and unilateral hearing loss Keywords: Brainstem encephalopathy Sensorineural hearing loss Hypertension
1. Introduction Hypertensive encephalopathy usually presents as posterior reversible encephalopathy syndrome (PRES), which is characterized by symmetrical vasogenic edema in the parieto-occipital areas where sympathetic innervation is relatively deficient compared with other areas [1,2]. The underlying pathological mechanism of PRES is the breakdown of cerebral autoregulation and endothelial dysfunction [2]. However, several reports have described a rare (about 4%) variant of PRES localized to the cerebellum and brainstem, rather than the posterior cerebrum [3–5]. This form of PRES is also referred to as hypertensive brainstem encephalopathy (HBE). As with typical PRES, severe hypertension, drugs, eclampsia, and chronic renal disease can cause HBE [3–5]. We herein describe an unusual case of HBE with severe headache and unilateral sensorineural hearing loss (SNHL) and discuss the potential pathomechanisms. 2. Case report A 32-year-old man was admitted with severe headache and suddenonset hearing impairment in the left ear. A mild headache had developed insidiously in the occipital area 1 week earlier and gradually progressed to severe, global head pain and discomfort. The patient said it was the most severe headache that he had ever experienced. The headache was pulsatile in nature and accompanied by nausea and vomiting. Hearing loss developed suddenly 2 days before visiting the hospital. He had been previously diagnosed with essential hypertension and treated with 50 mg atenolol and 30 mg sustained-release nifedipine per day for 15 years. He had stopped taking the medications of his own 1 month earlier. He had been smoking a pack of cigarettes per day for about 10 years. He was a social drinker. As for family history, the patient's father was diagnosed with essential hypertension in his twenties. The patient's blood pressure was 250/140 mm Hg. Prominent neck stiffness with Brudzinski's sign was observed. The Weber test lateralized to the right and the Rinne test was positive bilaterally. Pure tone audiometry revealed SNHL in the left ear (Fig. 1G). Brain MRI revealed severe vasogenic edema in the bilateral cerebellum, especially in the vermis and tonsil, which compressed the lower brain stem. Mild obstructive hydrocephalus developed due to narrowing of the fourth ventricle (Fig. 1A–D). Vestibular function test including the caloric reflex test revealed no abnormality.
http://dx.doi.org/10.1016/j.jns.2015.05.040 0022-510X/© 2015 Elsevier B.V. All rights reserved.
After administration of intravenous 20 mg nicardipine and 200 ml of 20% mannitol on the day of admission, his blood pressure dropped to 165/100 mm Hg and the headache was substantially relieved. On the second day of hospitalization, SNHL began to improve slowly and had almost returned to normal by the third day. A compatible finding was observed in the follow-up audiometry performed on the third day (Fig. 1H). A follow-up MRI performed on the same day revealed that vasogenic edema was much resolved and lower brainstem compression had subsided (Fig. 1E,F). A secondary hypertension work-up including measurement of serum renin/aldosterone, metanephrine, free T4, TSH, and urine vanillylmandelic acid levels revealed no abnormalities. After discharge, the patient has been treated with 50 mg atenolol, 10 mg amlodipine, and 40 mg olmesartan per day and has been symptom free for 5 months. 3. Discussion Patients with HBE require urgent medical care. Brainstem compression accompanied with elevated intracranial pressure may cause sudden neurological deterioration, irreversible neurologic damage, or death. However, symptoms are mostly reversible with proper treatment [3–5]. Therefore, early diagnosis and treatment are essential to avoid significant neurological damage. The development of SNHL in our patient was quite unusual. SNHL, especially unilateral SNHL, is rarely reported in patients with disorders causing increased intracranial pressure, such as idiopathic intracranial hypertension [6], hydrocephalus [7], cerebral venous sinus thrombosis [8], and Chiari malformation [9]. The exact mechanism of SNHL is uncertain in our case. However, it is possible that the elevated intracranial pressure was directly transferred to the cochlear perilymph, which is connected with subarachnoid CSF space via the cochlear aqueduct. The pressure difference between the perilymph and the endolymph may cause displacement of Reissner's membrane and thereby affect hearing [6–8]. Another possible mechanism is direct compression of the lower brainstem and vestibulocochlear nerve, which would cause hearing loss. Unfortunately, an auditory brainstem evoked response test was not performed during the hearing loss period, as it may have been helpful in differentiating between a cochlear, vestibulocochlear nerve, and brainstem lesion. Regardless of the pathogenesis, SNHL was reversed by acute reduction of blood and intracranial pressure. To the best of our knowledge, this is the first case report to describe a patient with unilateral SNHL associated with HBE. It is highly likely that the patient's willful cessation of chronic essential hypertension drugs initiated this unusual symptom of HBE. In patients with these symptoms, the possibility of HBE should therefore be considered. Rapid control of blood and intracranial pressure is crucial to prevent neurological deterioration and reverse SNHL. Conflict of interest/disclosures The authors have no conflict of interest in relation to this research and its publication.
212
Letter to the Editor
Fig. 1. Initial (A–D) and follow-up (E, F) MRI of the patient and initial (G) and follow-up (H) pure tone audiometry. (A) Diffusion-weighted imaging showed patchy and subtle high signal intensities in the cerebellum. (B) An ADC image showed patchy and confluent high signal intensities in the bilateral cerebellum. (C) T2 weighted image showed diffuse high signal intensities in the bilateral cerebellum and a small and oval-shaped high signal intensity in the right pons. (D) T1 sagittal image showed anterior displacement of the lower brainstem and diffuse edema of the cerebellar vermis and tonsil. (E) On the follow-up image, diffuse edema of bilateral cerebellum decreased, the pontine lesion disappeared, and the fourth ventricular narrowing subsided after treatment. (F) Anterior displacement of the brain stem subsided compared to the initial MRI. Sensorineural hearing loss in the left ear was initially observed at admission (G), and this was much improved after treatment (H). R, right.
References [1] W.S. Bartynski, Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema, AJNR Am. J. Neuroradiol. 29 (2008) 1043–1049. [2] J.E. Fugate, D.O. Claassen, H.J. Cloft, D.F. Kallmes, O.S. Kozak, A.A. Rabinstein, Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings, Mayo Clin. Proc. 85 (2010) 427–432. [3] A.M. McKinney, B.D. Jagadeesan, C.L. Truwit, Central-variant posterior reversible encephalopathy syndrome: brainstem of basal ganglia involvement lacking cortical or subcortical cerebral edema, AJR 201 (2013) 631–638. [4] R. Bălaşa, S. Maier, E.G. Baubec, Z. Bajkó, A. Bălaşa, Cerebellar and brainstem variant of posterior reversible encephalopathy syndrome, Acta Neurol. Belg. (2015) (in press). [5] I. Karakis, J.A. Macdonald, M. Stefanidou, C.S. Kase, Clinical and radiological features of brainstem variant hypertensive encephalopathy, J. Vasc. Interv. Neurol. 2 (2009) 172–176. [6] P.J. Dorman, M.J. Campbell, A.R. Maw, Hearing loss as a false localizing sign in raised intracranial pressure, J. Neurol. Neurosurg. Psychiatry 58 (1995) 516. [7] V.J. Sammons, E. Jacobson, J. Lawson, Resolution of hydrocephalus-associated sensorineural hearing loss after insertion of ventriculoperitoneal shunt, J. Neurosurg. Pediatr. 4 (2009) 394–396. [8] A.C. Fonseca, L. Albuquerque, J.M. Ferro, Reversible bilateral hearing loss in a woman with cerebral venous thrombosis, J. Neurol. 255 (2008) 1825–1826.
[9] H. Dolgun, E. Turkoglu, H. Kertmen, E.R. Yilmaz, Z. Sekerci, Chiari type I malformation presenting with bilateral hearing loss, J. Clin. Neurosci. 16 (2009) 1228–1230.
Jung-Ju Lee Soohyun Cho Jong-Moo Park Department of Neurology, Eulji General Hospital, Eulji University College of Medicine, Hangeulbisekro 68, Noweongu, Seoul, Republic of Korea Kyung-Il Park Department of Neurology, Seoul Paik Hospital, Inje University College of Medicine, Mareunnae-ro 9, Jung-gu, Seoul, Republic of Korea Corresponding author at: Department of Neurology, Seoul Paik Hospital, Inje University, Mareunnae-ro 9, Jung-gu, Seoul 100-032, Republic of Korea. E-mail address: [email protected] (K.-I. Park). 16 April 2015
