|Year : 2022 | Volume
| Issue : 1 | Page : 72-74
Stupor resolving into rapidly deteriorating locked-in syndrome: Early magnetic resonance imaging brain in diagnosis
Professor, Department of Psychiatry, Malla Reddy Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Submission||01-Apr-2021|
|Date of Acceptance||15-Apr-2021|
|Date of Web Publication||04-Mar-2022|
Dr. Bhogaraju Anand
Department of Psychiatry, Malla Reddy Institute of Medical Sciences, Suraram, Hyderabad - 500 055, Telangana
Source of Support: None, Conflict of Interest: None
Locked-in syndrome (LiS) is a rare condition characterized by quadriplegia, lower cranial nerve paralysis, and mutism with preservation of consciousness, vertical gaze, and upper eyelid movement. Here, we present a case that had initial mild neurological symptoms, stupor, fully conscious state, and computed tomography-brain showing old cerebral infarcts. There was a rapid deterioration of the condition into LiS and magnetic resonance imaging (MRI) brain showing infarcts bilaterally in the cerebellum, pons, midbrain, and thalami and finally leading to death. Relevant studies for discussion are presented. This case highlights the importance of MRI brain at the earliest even for cases with initial mild neurological symptoms and stupor to make an early diagnosis.
Keywords: Early diagnosis, locked-in–syndrome, magnetic resonance imaging brain, stupor
|How to cite this article:|
Anand B. Stupor resolving into rapidly deteriorating locked-in syndrome: Early magnetic resonance imaging brain in diagnosis. Arch Ment Health 2022;23:72-4
|How to cite this URL:|
Anand B. Stupor resolving into rapidly deteriorating locked-in syndrome: Early magnetic resonance imaging brain in diagnosis. Arch Ment Health [serial online] 2022 [cited 2022 Jun 26];23:72-4. Available from: https://www.amhonline.org/text.asp?2022/23/1/72/339121
| Introduction|| |
Plum and Posner first defined locked-in syndrome (LiS) with signs of quadriplegia, lower cranial nerve paralysis, and mutism with preservation of consciousness, vertical gaze, and upper eyelid movement. Haig et al. preferred the term anarthria over mutism as the term mutism implied unwillingness to speak. Occasional case series or case reports show that this is a rare condition.
| Case Report|| |
A 70-year-old female known hypertensive, type 2 diabetes mellitus, and hypothyroidism presented to medical outpatient with history of dizziness and leaning toward the left side while walking of 1-week duration. Two years back, she was admitted with a diagnosis of cerebrovascular accident with right hemiparesis; she was treated with good response to treatment and was able to perform activities of daily living and instrumental activities of daily living. Her symptoms were progressively worsening for the last 1 week and were admitted to intensive care unit. Her vital functions were stable and Ryle's tube feeding was given and Foley's catheter was in place. She was not moving from the bed and would open her eyes as if looking around. A condition of stupor was entertained and a psychiatrist was called to evaluate. Noncontrast computed tomography (CT) scan of the brain showed: Acute lacunar infarct in right corona radiata, chronic infarcts in right capsuloganglionic, right corona radiate and right cerebellar hemisphere and age-related cerebral atrophy [Figure 1]a and [Figure 1]b.
|Figure 1: (a and b) Noncontrast computed brain: Acute lacunar infarct in right corona radiate, chronic infarcts in right capsulo ganglionic , right corona radiata and right cerebellar hemisphere, and age-related cerebral atrophy|
Click here to view
There was no previous history of psychiatric illness. On examination, the patient was lying in the bed, not moving, eyes opened occasionally, but no eye contact, she was not following commands and no purposive movements. Reaction to deep pain was not nonpurposive. Her plantars were flexor. A condition of organic stupor or LiS was made. No metabolic causes would explain the condition nor was CT scan of the brain helps to explain the condition. Magnetic resonance imaging (MRI) brain was done which showed multiple scattered nonhemorrhagic infarcts bilaterally in cerebellum, pons, midbrain, and thalami. Chronic infarcts with gliosis and gradient recalled echo blooming in right capsuloganglionic region seventh and eighth nerve complexes normally seen, with no mass lesions at the angle region on either side. Age-related diffuse cerebral loss [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e. The patient became progressively unconscious, Glasgow Coma Scale 4 (E1V1M3), Plantars became extensor bilaterally. She was started on intravenous mannitol patient died in 4 days after admission.
|Figure 2: (a-c) Upper row magnetic resonance imaging head and brain diffusion-weighted and lower row (d and e) is fluid-attenuated inversion recovery imaging: shows multiple scattered nonhemorrhagic infarcts bilaterally in the cerebellum, pons, midbrain, and thalami. Chronic infarcts with gliosis and gradient recalled echo blooming in right capsulo ganglionic region. Seventh and eighth nerve complexes normally|
Click here to view
This case highlights confusion between Stupor and LiS. Brain stem infarcts were detected on MRI brain although CT of the brain could not explain the condition. Stupor was only a transition state. With no history of psychiatric presentation, one has to entertain LiS.
| Discussion|| |
From a state of full consciousness, this patient went into a stupor, with akinesis but occasional movements and mutism but her eyes were following movements alone. There was no preceding history of depression or schizophrenia or mania. CT brain showed old infarcts. Further, the patient had to be put on tube feeding and urinary catheter raised the suspicion of an organic cause of stupor and at this stage, MRI brain was ordered which showed recent onset of infarcts in pons, cerebellum, and midbrain. The evolution of the symptoms was rapid in this case, not responding to antiplatelet therapy eventually leading to death.
Bauer et al. described in 12 cases LiS and classified them into classic LiS with Quadriplegia, anarthria, preserved consciousness, vertical eye movement, and blinking. Incomplete liS is similar to classic, but with some voluntary movement other than vertical eye movement are present. Lui et al. observed in three cases the initial presentation was dysarthria, mild uncrossed Hemi sensory or Hemi motor deficit involving face and extremities on the same side. This was thought of as a mild cerebral or brainstem vascular accident but soon evolved into a locked-in state. Li et al. described in a 34 year old woman initial complaints of unsteady feeling which progressed to slurred speech and left-sided weakness, decreased ability to communicate and being frustrated. CT scan of the head was found normal. MRI of the brain without contrast showed a large acute pontine infarct involving the width of the pons. MR angiography showed occlusion at the proximal area of the basilar artery with some flow being maintained at the uppermost portion of the basilar artery. Ayas et al. reported a case of incomplete LiS, in a 62-year-old man presenting with right-sided numbness, limited response to verbal stimuli and somnolence, right hemi hypoesthesia, CT head was normal, but diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) MRI revealed an area of acute infarction in the left mesencephalon. Later, the patient developed quadriplegia with preserved horizontal and vertical eye movements and preserved motor functions. Repeat brain DWI and ADC MRI showed infarction with severe limitation of flow in the region of the left medial mesencephalon and pons.
Hindman et al. in two cases of stupor, the lesions were extending from thalamus to posterior hypothalamus and midbrain. In 12 patients, lesions entirely confined to the thalamus had no problems in arousal. In 17 patients with intermediate extension into the midbrain, partial impairments in the level of arousal in the form of obtundation, somnolent or lethargic were seen. Patterson and Grabois in a review of 139 cases of LiS found that the cause's mortality in the nonvascular group (41%) was less than in the vascular group (67%). Transient ischemic attacks precede posterior circulation infarcts in 18%–57% of cases.
| Conclusion|| |
This case highlights the importance of MRI brain at the earliest even for cases with initial mild neurological symptoms and stupor to make an early diagnosis.
Thanks to the Chief Medicine Department for helping with case material.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Plum F, Posner JB. The Diagnosis of Stupor and Coma. Philadelphia, PA: FA Davis; 1966.
|2.|Haig AJ, Katz RT, Sahgal V. Mortality and complications of the locked-in syndrome. Arch Phys Med Rehabil 1987;68:24-7.
Bauer G, Gerstenbrand F, Rumpl E. Varieties of the locked-in syndrome. J Neurol 1979;221:77-91.
Liu J, Tuhrim S, Weinberger J, Song SK, Anderson PJ. Premonitory symptoms of stroke in evolution to the locked-in state. J Neurol Neurosurg Psychiatry 1983;46:221-6.
Li W, Brandon O, Smith DV, Petersen E. A conversion disorder or a stroke? A proximal basilar artery thrombosis induced 'locked-in' syndrome in a young Caucasian woman. BMJ Case Rep 2013;2013:1-3.
|6.|Ayas ZO, Kotan D, Akdogan M, Gunel ME. Serum prolidase enzyme activity level: Not a predictive biomarker for epilepsy. Eurasian J Med 2019;51:27-30.
|7.|Hindman J, Bowren MD, Bruss J, Wright B, Geerling JC, Boes AD. Thalamic strokes that severely impair arousal extend into the brainstem. Ann Neurol 2018;84:926-30.
|8.|Patterson JR, Grabois M. Locked-in syndrome: A review of 139 cases. Stroke 1986;17:758-64.
Bernasconi A, Bogousslavsky J, Bassetti C, Regli F. Multiple acute infarcts in the posterior circulation. J Neurol Neurosurg Psychiatry 1996;60:289-96.
[Figure 1], [Figure 2]