Challenges when diagnosing locked in syndrome following TBI: The Story of U.P. a Clinical Puzzle

Abstract

Mistry N, Wilson BA and Rose A

Background and aims: Locked-in syndrome (LIS) is a rare neurological disorder; patients with LIS are awake, conscious with normal or nearly normal cognitive functioning. They cannot produce speech, facial or limb movements with complete paralysis of all voluntary muscles except for those controlling eye movements. LIS is associated with lesions of the brain stem and the pons, with 60% of people having sustained a stroke. LIS following traumatic brain injury (TBI) is rare; brain stem lesion plus cortical damage makes diagnosis of LIS challenging.

Method: We describe U.P, a 42 year old man who sustained a TBI. A CT scan showed traumatic subarachnoid haemorrhage with a pre-pontine bleed. Awake, conscious, presenting with right sided paralysis and severe left sided paresis;U.P could produce voluntary horizontal eye movement, tracking people and stimuli of interest. Vertical eye movements emerged later, reading simple instructions and following commands.

Results: U.P could use eyebrow movements for “yes” and a slight head shake for “no”. He could read some written instructions; non-verbal responses were inconsistent and sometimes unreliable.

Discussion: Based on assessments from speech and language therapy and neuropsychology with U.P, we discuss LIS plus additional cognitive problems and the difficulties with diagnosing LIS following TBI.

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