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Neurocritical Care

, Volume 27, Issue 1, pp 108–114 | Cite as

Reverse Locked-In Syndrome

  • Pooja Raibagkar
  • Ram V. Chavali
  • Tamara B. Kaplan
  • Jennifer A. Kim
  • Meaghan V. Nitka
  • Sherry H.-Y. Chou
  • Brian L. EdlowEmail author
Practical Pearl

Abstract

Background

Basilar artery occlusion can cause locked-in syndrome, which is characterized by quadriplegia, anarthria, and limited communication via eye movements. Here, we describe an uncommon stroke syndrome associated with endovascular recanalization of the top of the basilar artery: “reverse locked-in syndrome.”

Methods

We report the case of a patient with atypical neurological deficits caused by acute ischemic stroke of the midbrain tegmentum. We perform neuroanatomic localization of the patient’s infarcts by mapping the magnetic resonance imaging (MRI) data onto a brainstem atlas.

Results

A 61-year-old man presented with acute coma and quadriplegia due to top of the basilar artery occlusion. He underwent emergent endovascular thrombectomy, with successful recanalization of the basilar artery at 4 h and 43 min post-ictus. The patient regained consciousness and purposeful movement in all four extremities, but the post-procedure neurological examination demonstrated bilateral ptosis with complete pupillary and oculomotor paralysis. MRI revealed infarction of the bilateral oculomotor nuclei in the midbrain tegmentum. At 9-month follow-up, he had anisocoria and dysconjugate gaze, but was living at home and required minimal assistance in performing all activities of daily living.

Conclusions

Since the patient’s deficits were the exact opposite of those described in locked-in syndrome, we propose the term “reverse locked-in syndrome” to describe this neurological entity characterized by bilateral ptosis, non-reactive pupils, and ophthalmoplegia with preservation of consciousness and extremity motor function.

Keywords

Top of the basilar artery Locked-in syndrome Midbrain tegmentum Endovascular thrombectomy Ophthalmoparesis 

Notes

Funding

This work was supported by the National Institutes of Health (K23NS094538), American Academy of Neurology/American Brain Foundation, and the James S. McDonnell Foundation.

Compliance with ethical standards

Conflict of interest

Sherry H.-Y. Chou: National Institutes of Health/National Institute of Neurological Disorders and Stroke (K23-NS073806), University of Pittsburgh Physicians Research Grant, serves as the site principal investigator for Newton II clinical trial funded by Edge Therapeutics. Pooja Raibagkar, Ram V. Chavali, Tamara B. Kaplan, Jennifer A. Kim, Meaghan V. Nitka, Brian L. Edlow: nothing to disclose.

References

  1. 1.
    Ferbert A, Bruckmann H, Drummen R. Clinical features of proven basilar artery occlusion. Stroke. 1990;21:1135–42.CrossRefPubMedGoogle Scholar
  2. 2.
    Plum F, Posner JB. The diagnosis of stupor and coma. Contemp Neurol Ser. 1972;10:1–286.PubMedGoogle Scholar
  3. 3.
    Brandt T. Diagnosis and thrombolytic therapy of acute basilar artery occlusion: a review. Clin Exp Hypertens. 2002;24:611–22.CrossRefPubMedGoogle Scholar
  4. 4.
    Baird TA, Muir KW, Bone I. Basilar artery occlusion. Neurocrit Care. 2004;1:319–29.CrossRefPubMedGoogle Scholar
  5. 5.
    Karp JS, Hurtig HI. “Locked-in” state with bilateral midbrain infarcts. Arch Neurol. 1974;30:176–8.CrossRefPubMedGoogle Scholar
  6. 6.
    Zakaria T, Flaherty ML. Locked-in syndrome resulting from bilateral cerebral peduncle infarctions. Neurology. 2006;67:1889.CrossRefPubMedGoogle Scholar
  7. 7.
    Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R, Perez-Santamaria FJ. The locked-in syndrome: a syndrome looking for a therapy. Brain Injury. 2002;16:571–82.CrossRefPubMedGoogle Scholar
  8. 8.
    Leon-Carrion J, Van Eeckhout P, Dominguez-Morales MR. The locked-in syndrome: a challenge for therapy. Acta Neurochir Suppl. 2005;93:213–5.CrossRefPubMedGoogle Scholar
  9. 9.
    Goyal M, Demchuk AM, Menon BK, et al. Randomized assessment of rapid endovascular treatment of ischemic stroke. N Engl J Med. 2015;372:1019–30.CrossRefPubMedGoogle Scholar
  10. 10.
    Saver JL, Goyal M, Bonafe A, et al. Stent-retriever thrombectomy after intravenous t-PA vs. t-PA alone in stroke. N Engl J Med. 2015;372:2285–95.CrossRefPubMedGoogle Scholar
  11. 11.
    Berkhemer OA, Fransen PS, Beumer D, et al. A randomized trial of intraarterial treatment for acute ischemic stroke. N Engl J Med. 2015;372:11–20.CrossRefPubMedGoogle Scholar
  12. 12.
    Campbell BC, Mitchell PJ, Kleinig TJ, et al. Endovascular therapy for ischemic stroke with perfusion-imaging selection. N Engl J Med. 2015;372:1009–18.CrossRefPubMedGoogle Scholar
  13. 13.
    Jovin TG, Chamorro A, Cobo E, et al. Thrombectomy within 8 hours after symptom onset in ischemic stroke. N Engl J Med. 2015;372:2296–306.CrossRefPubMedGoogle Scholar
  14. 14.
    Edlow BL, Takahashi E, Wu O, et al. Neuroanatomic connectivity of the human ascending arousal system critical to consciousness and its disorders. J Neuropathol Exp Neurol. 2012;71:531–46.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Edlow BL, Giacino JT, Hirschberg RE, Gerrard J, Wu O, Hochberg LR. Unexpected recovery of function after severe traumatic brain injury: the limits of early neuroimaging-based outcome prediction. Neurocrit Care. 2013;19:364–75.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Edlow BL, McNab JA, Witzel T, Kinney HC. The structural connectome of the human central homeostatic network. Brain Connect. 2016;6:187–200.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Lindsberg PJ, Mattle HP. Therapy of basilar artery occlusion: a systematic analysis comparing intra-arterial and intravenous thrombolysis. Stroke. 2006;37:922–8.CrossRefPubMedGoogle Scholar
  18. 18.
    Marinkovic S, Milisavljevic M, Kovacevic M. Interpeduncular perforating branches of the posterior cerebral artery. Microsurgical anatomy of their extracerebral and intracerebral segments. Surg Neurol. 1986;26:349–59.CrossRefPubMedGoogle Scholar
  19. 19.
    Kubik CS, Adams RD. Occlusion of the basilar artery; a clinical and pathological study. Brain. 1946;69:73–121.CrossRefPubMedGoogle Scholar
  20. 20.
    Duvernoy HM. Human brain stem vessels: including the pineal gland and information on brain stem infarction. 2nd completely rev. and expanded ed. Berlin: Springer; 1999.Google Scholar
  21. 21.
    Facon E, Steriade M, Wertheim N. Prolonged hypersomnia caused by bilateral lesions of the medial activator system; thrombotic syndrome of the bifurcation of the basilar trunk. Rev Neurol (Paris). 1958;98:117–33.Google Scholar
  22. 22.
    Finocchi C, Del Sette M, Croce R, Giberti L, Serrati C, Gandolfo C. Bilateral ophthalmoplegia: an unusual sign of the top of the basilar artery syndrome. Ital J Neurol Sci. 1996;17:301–4.CrossRefPubMedGoogle Scholar
  23. 23.
    Thurtell MJ, Halmagyi GM. Complete ophthalmoplegia: an unusual sign of bilateral paramedian midbrain-thalamic infarction. Stroke. 2008;39:1355–7.CrossRefPubMedGoogle Scholar
  24. 24.
    Inocencio FP, Ballecer R. Tuberculosis granuloma in the midbrain causing wall-eyed bilateral internuclear ophthalmoplegia (Webino). J Clin Neuroophthalmol. 1985;5:31–5.PubMedGoogle Scholar
  25. 25.
    Kim JS, Jeong SH, Oh YM, Yang YS, Kim SY. Teaching NeuroImage: wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) from midbrain infarction. Neurology. 2008;70:e35.CrossRefPubMedGoogle Scholar
  26. 26.
    Pullicino P, Lincoff N, Truax BT. Abnormal vergence with upper brainstem infarcts: pseudoabducens palsy. Neurology. 2000;55:352–8.CrossRefPubMedGoogle Scholar
  27. 27.
    Caplan LR. “Top of the basilar” syndrome. Neurology. 1980;30:72–9.CrossRefPubMedGoogle Scholar
  28. 28.
    Bender MB. Brain control of conjugate horizontal and vertical eye movements: a survey of the structural and functional correlates. Brain. 1980;103:23–69.CrossRefPubMedGoogle Scholar
  29. 29.
    Schmahmann JD. Vascular syndromes of the thalamus. Stroke. 2003;34:2264–78.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Pooja Raibagkar
    • 1
  • Ram V. Chavali
    • 2
  • Tamara B. Kaplan
    • 1
  • Jennifer A. Kim
    • 1
  • Meaghan V. Nitka
    • 3
  • Sherry H.-Y. Chou
    • 4
  • Brian L. Edlow
    • 1
    • 5
    Email author
  1. 1.Department of NeurologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Department of RadiologyBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Department of Emergency MedicineLowell General HospitalLowellUSA
  4. 4.Departments of Critical Care Medicine, Neurology, and NeurosurgeryUniversity of Pittsburgh School of MedicinePittsburghUSA
  5. 5.Athinoula A. Martinos Center for Biomedical ImagingMassachusetts General Hospital, Harvard Medical SchoolCharlestownUSA

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