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Premonitory Symptoms of Stroke in Evolution to the Locked-In State

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Premonitory Symptoms of Stroke in Evolution to the Locked-In State J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.3.221 on 1 March 1983. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry 1983 ;46:221-226 Premonitory symptoms of stroke in evolution to the locked-in state JOHN LIU, STANLEY TUHRIM,* JESSE WEINBERGER,* SUN K. SONG,t PAUL J ANDERSONt From the Department ofNeurology* and Division ofNeuropathologyt The Mount Sinai School ofMedicine, City University ofNew York, and the Division ofNeuropathology, City Hospital Centre at Elmhurstt, New York, USA SUMMARY Three patients, who subsequently developed the locked-in state characterised by quadriplegia and mutism with an alert sensorium, initially had mild dysarthria and uncrossed hemisensory or hemimotor deficits involving the face and ipsilateral extremities. Case one ini- tially mimicked a left cerebral lesion with right hemisensory deficits, a mild right facial paresis and a right homonymous field deficit. Case two initially developed both left hemimotor and hemisen- sory deficits and later developed a paresis of right conjugate gaze. Case three presented with left hemimotor deficit, and mild paresis of conjugate gaze to the right. All three patients died. Rostral were brainstem infarctions found at necropsy in cases one and two. Case three had a radiolucent Protected by copyright. area of the brainstem demonstrated by CT Scan. Hemisensory and hemimotor deficits also have been noted to precede reported cases of pontine infarction with the locked-in state. Acute onset of uncrossed hemisensory and hemimotor deficits with dysarthria may be caused by infarction of the pons which may predispose to the locked-in state. The locked-in state is a devastating neurological ler infarction in the midventral pons was found. syndrome secondary to infarction or hemorrhage Both had infarctions involving the pyramidal tract in the ventral and rostral pons (-15). Patients are above the facial nucleus. This accounted for the ini- unable to move their extremities or speak, but are tial symptomatology involving the face and limbs on alert and responsive. They communicate with the same side. In the third case, a brainstem lesion vertical eye movements and lid movements. was revealed by computed tomography (CT). Horizontal eye movements usually are impaired. These cases illustrate that patients presenting with The locked-in state can occur acutely, but often symptoms of dysarthria and uncrossed hemisensory premonitory symptoms are present. or hemimotor deficits may have ischaemic lesions in We report three cases of patients who presented the ventral or rostral pons which may progress to the http://jnnp.bmj.com/ with mild neurological deficits preceding the locked-in state. development of the locked-in state. All three ini- tially had dysarthria and a mild uncrossed hemisen- Case reports sory or hemimotor deficit involving face and extremities on the same side. They were all felt to Case 1 A 75-year-old diabetic, hypertensive woman have had a mild cerebral or brainstem vascular acci- was admitted with slurred speech, dizziness and dent, until the locked-in state evolved. Post-mortem unsteadiness of gait of one day's duration. She had examination was obtained in two patients. In one, a noted circumoral numbness a few days earlier. She on September 30, 2021 by guest. large infarction of the rostral pons at the mesence- denied diplopia, vomiting, prior weakness or sen- phalic junction was identified. In the second, a smal- sory loss. Medications were tolbutamide and methyldopa. The blood pressure was 200/100 Address for reprint requests: Dr Jesse Weinberger, The Mount mmHg and pulse was 69/minute. She was alert, Sinai School of Medicine, 1 Gustave Levy Place, New York, New oriented and named objects well. Speech was dysar- York 10029, USA thric. There was a right homonymous hemianopia Received 3 July 1982 and in revised form 29 October 1982 but objects could be located in the affected field. Accepted 13 November 1982 Pupils were equal and reactive. Fundi were normal. 221 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.3.221 on 1 March 1983. Downloaded from 222 Liu, Tuhrim, Weinberger, Song, Anderson Ocular movements were normal. There was a mild maximal diameter. There were bilateral, wedge- right facial paresis with drooling from the right shaped recent infarcts of the medial and ventral sur- corner of the mouth. The right palpebral fissure was faces of the occipital lobes, each measuring 3 to 4 cm wider than the left. Right facial sensation was in maximal extent. Examination of the brain stem diminished to light touch. Strength was unimpaired disclosed extensive recent infarction of the crus bilaterally and reflexes were symmetrically active cerebri and tegmentum and base of pons bilaterally with flexor plantar responses. Vibration and light (fig 1). Within the pons, the zone of infarction touch sensation were impaired on the right side. measured 2*5 cm rostrocaudally, 2-0 cm trans- Pin-prick and position perception were normal. versely, and 1-5 cm dorsoventrally. Rapid alternating movements were slightly impaired Microscopic examination disclosed multiple bilaterally, more on the right. The heel to knee to recent infarcts in the calcarine cortex of both occipi- shin manoeuvre was clumsy on the right. She walked tal lobes. Focal infarcts were also present in the with an unsteady shuffling gait. Spinal fluid, blood thalamus. The most extensive zone of infarction chemistries, haematocrit and sedimentation rate involved most of the base of the rostral and mid- were normal. portions of the pons. At the level of the middle Twelve hours later, she was found to be unrespon- cerebellar peduncle, the infarction extended dor- sive with Cheyne-Stokes respirations. Blood pres- sally to destroy most of the pontine tegmentum. sure was 200/90 mmHg and pulse was 60/min. Eyes Rostrally, the infarct extended into both crus cerebri were deviated downward without response to where extensive portions of the substantia nigra and oculocephalic manoeuvre or to cold water caloric fiber tracts of the crus were destroyed. Recent focal testing. Pupils were 1-5 mm bilaterally and mini- infarcts were also present in the central white matter mally reactive to light. Corneal responses were of the cerebellum. The medulla contained irregular depressed bilaterally. There was a quadraparesis zones of rarefaction and there was descending upper in both corticospinal tracts. Swollen with slight extensor posturing of the degeneration Protected by copyright. extremities to noxious stimuli. Reflexes were 3+ axonal fragments and reactive hyperplastic astro- bilaterally with bilateral extensor plantar responses. cytes were observed in the medullary pyramids She was placed on a respirator. Computed tomogra- and the lateral funiculi of the cervical spinal cord. phy revealed a left sided pontine lucency. Two days Sections of the basilar artery showed severe later, the patient had upper facial grimacing with atherosclerotic narrowing of the lumen, but no areas crying when spoken to by her sister. Pupils were at 2 of occlusion were found. All of the above described mm and reactive to light. Resting gaze was disconju- infarcts were of similar recent age, while the focal gate with slight right eye adduction. The patient infarct in the right caudate nucleus was considerably looked down and upward to the midline from a older. down position upon command. Gaze above the mid- The neuropathological diagnoses were: (1) line was impaired. She had no horizontal eye move- infarct, recent, extensive, of pons, mid-brain, cere- ments on passive head turning or ice water caloric bellum, thalamus, and occipital lobes bilaterally, (2) testing. Opticokinetic response to a striped drum descending degeneration, recent, of corticospinal was absent in all directions. Corneal reflexes were tracts, (3) infarct, old, focal, right caudate nucleus, present bilaterally. She could not raise her eyebrows (4) atherosclerosis, severe, basilar artery. was or forcefully close her eyelids to command. She http://jnnp.bmj.com/ quadraparetic with extensor posturing of the arms Case 2 A 67-year-old white male complained of and slight withdrawal of the lower extremities to three days of pain in his left ear and episodic painful stimuli. She developed a fever and died three unsteadiness of increasing frequency lasting approx- weeks later. imately fifteen minutes. He also complained of Necropsy disclosed severe atherosclerosis involv- episodes of numbness and tingling of the left arm ing aortic, renal iliac, coronary and carotid arteries. and face associated with slurred speech. Initial There was bilateral bronchopneumonia of recent neurologic examination was unremarkable. Upon brain disclosed severe awakening seven days after onset of symptoms, the origin. Examination of the on September 30, 2021 by guest. atherosclerotic plaque formation within the ver- patient was noted to have slurred speech, a right tebral and basilar arteries. Multiple cross-sections conjugate gaze paresis, left central facial nerve palsy through these vessels disclosed varying degrees of and a mild left hemiparesis. The patient reported narrowing of the lumen, most pronounced in the decreased sensation to pinprick over the entire left mid-portion of the basilar artery overlying the base side of the body, including the face. Position sense of the pons. Dissection of the cerebrum disclosed a was preserved. Over the next twenty-four hours the well circumscribed old infarct in the head and body patient became quadriplegic with bilateral extensor of the right caudate nucleus, measuring 0*5 cm in posturing and bilateral extensor plantar responses, J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.3.221 on 1 March 1983. Downloaded from Premonitory symptoms ofstroke in evolution to the locked-in state 223 Protected by copyright. Fig 1 Case 1. Transverse sections through mid and caudal levels ofmidbrain (upper left and centre) and rostral level ofpons (lower right). Extensive recent infarct destroys most ofthe tegmentum and base ofthe pons and extends rostrally to destroy portions ofthe crus cerebri. but he remained responsive to verbal stimuli.
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