Editor:
A 49-yr-old female suddenly experienced intense lower neck pain irradiating to her right arm and trunk. Within a few minutes, she developed right-sided weakness in her arm and leg. She had recently been diagnosed with hypertension but was on no treatment. On admission to the emergency hospital, she was afebrile with high systolic (190–210 mmHg) and diastolic (130–150 mmHg) blood pressure (BP). A myocardial infarction or a ruptured aortic aneurysm was initially suspected; however, laboratory investigations including normal troponin and creatine kinase levels as well as electrocardiography, echocardiography and thorax computed tomography scan excluded these conditions. A few hours later, the patient began complaining of intense headache and blindness and within a few minutes a prolonged tonic-clonic seizure occurred. On admission to our hospital, she was confused and somnolent. BP was 185/130 mmHg. Neurological examination showed severe right-sided paresis and sensory deficit sparing the face, enhanced tendon reflexes in both upper and lower limbs (right > left), and bilateral upgoing plantars. Her strength was 2/5 in the right upper extremity, 0/5 in the right lower extremity and 4/5 in the left upper and lower extremities.
Brain magnetic resonance (MR) scan showed multiple focal, mainly posterior, white and grey matter hyperintensities on fluid-attenuated inversion recovery (FLAIR)-sequences and diffusion-weighted images (DWI), suggesting vasogenic oedema (Fig. 1a, b). Posterior reversible encephalopathy syndrome (PRES) was diagnosed and intravenous valproate and clonidine were administered. The patient’s neurological function slowly deteriorated with increasing weakness of extremities. We performed an MR scan of the spine. It showed a large extraspinal mass lesion compressing the dorsolateral surface of the medulla at the levels C8–T2 (Fig. 1c–e). The lesion was slightly hyperintense on T1-weighted images and hyperintense on T2-weighted images, strongly suggestive of spinal epidural hematoma. No abnormally enlarged vessels were observed on T2-weighted or post-contrast T1-weighted images, thus excluding the presence of an underlying arteriovenous malformation.
Figure 1 (a–e). Fluid-attenuated inversion recovery (a) brain MR image showing increased white/grey matter predominantly affecting the parieto-occipital areas and (b) diffusion-weighted image showing elevated intensity signal in a pattern typical for posterior reversible encephalopathy syndrome. (c) Sagittal T2-weighted, (d) axial T1-weighted and (e) T2-weighted spine MR images showing the spindle-shaped epidural hematoma on the cervicothoracic spine with characteristic signal intensity.
The patient was transferred to the neurosurgery department where she underwent a C4–C7 laminectomy followed by evacuation of a thick blood clot. Over the following days, her neurological condition rapidly improved and brain and spine MR scans repeated 2 weeks later showed almost complete remission. She was discharged to the ward with no further complications.
PRES is a clinico-radiological syndrome associated with several conditions including hypertension, preeclampsia/eclampsia, treatment with immunosuppressive drugs, haemolytic uraemic syndrome, acute glomerulonephritis, blood transfusion, i.v. globulin or erythropoietin administration, acute intermittent porphyria and severe hypercalcaemia [Reference Hinchey, Chaves and Appignani1,Reference Servillo, Bifulco and De Robertis2]. The favoured pathogenetic theory suggests autoregulatory disturbance with hyperperfusion resulting in blood–brain barrier breakdown with reversible oedema without infarction [Reference Hinchey, Chaves and Appignani1,Reference Servillo, Bifulco and De Robertis2]. Accordingly, MR study typically reveals hyperintensity on both echoes of a dual-echo T2-weighted sequence and either iso- or hypointensity on T1-weighted image brain abnormalities, including both grey and white matter. Parietal–occipital lobes are mainly involved although other cerebral structures are also frequently involved [Reference Hinchey, Chaves and Appignani1–Reference Covarrubias, Luetmer and Campeau3]. DWI sequences detect white matter oedema and also reliably differentiates between vasogenic and cytotoxic oedema. Moreover, high DWI signal abnormalities and apparent diffusion coefficient pseudonormalization often correlate with patient outcome and may represent the earliest sign of nonreversibility as severe vasogenic oedema progresses to cytotoxic oedema, which carries a worse prognosis [Reference Servillo, Bifulco and De Robertis2,Reference Covarrubias, Luetmer and Campeau3].
One of the distinctive characteristics of PRES is the reversibility of radiological abnormalities once that treatment is instituted. Lowering BP, removal or significant reduction of the causative medication as well as the treatment of seizures are mandatory [Reference Hinchey, Chaves and Appignani1,Reference Servillo, Bifulco and De Robertis2].
Spinal epidural haematomaseh accounts for 0.3–0.9% of the epidural space-occupying lesions, more often occurring in the cervicothoracic and thoracolumbar regions. It has been associated with anticoagulant therapy, vascular malformation, venous epidural plexus defects, inherited or acquired bleeding disorders and hypertension [Reference Hsieh, Chiang, Tang, Sun and Ju4,Reference Chang, Lirng and Luo5]. It typically begins with a sudden, localized, intense pain in the neck or spine that progresses rapidly and often shows a radicular radiation. Neurological signs may appear asymmetrically and develop within minutes or even hours, corresponding to the level of the spinal cord compression. Clinical diagnosis is often difficult and it may be easily misdiagnosed as the differential diagnosis includes several chest and abdominal diseases [Reference Hsieh, Chiang, Tang, Sun and Ju4,Reference Chang, Lirng and Luo5]. Furthermore, progressing myelitis and polyradiculitis, myelocompressive diseases, including tumours and disc herniations should also be considered. MR scan is the diagnostic tool of choice as it allows the correct position and the extent of the haematoma, cord compression and to determine the presence of spinal cord oedema. Spinal epidural haematom may result in permanent neurological deficit or death if not properly treated. The standard management is emergency evacuation of the haematoma and spinal cord decompression. The more rapid is the surgical decompression, the more favourable is the outcome [Reference Hsieh, Chiang, Tang, Sun and Ju4–Reference Shin, Kuh and Cho6].
After the exclusion of coagulopathies, vascular malformations and other sources of haemorrhage, we suggest that the hypertension was the cause of both PRES and SEH in this patient. Moreover, persisting high BP values were documented at admission. The high BP and the increased systemic pressure exceeding the autoregulatory mechanisms of the cerebral vasculature might have been sufficient to overcome the blood–brain barrier and allow extravasation of fluid into the brain as well as of blood in the epidural space. Thus, not only the brain but also the spinal cord should be considered as a possible, albeit less common, site of hypertension-induced nervous tissue damage.
