Locked in syndrome secondary a multiform glioblastoma in brain stem. ARTURO AYALA-ARCIPRESTE MD FAANS

LOCKED-IN SYNDROME SECONDARY TO MULTIFORM GLIOBLASTOMA ON LOWER PONS, MEDULLA AND CERVICAL SPINAL CORD IN ADULT AGE.

Arturo Ayala Arcipreste, Eduardo Díaz Juárez, Rafael Mendizabal Guerra, Rubén Acosta Garcés, Luis Delgado Reyes, Gervith Reyes Soto, Carlos Delgado Hernández, Ignacio Félix, Durdica López Vujnovic.

Neurosurgery Department. Hospital Juárez de México. México City.

Background

Locked-in syndrome was described for Plum and Posner on 1966.

The most common etiology is due to stroke on perforator branches of basilar trunk affecting ventral pons, however desmielinizating pathology and tumors has been reported.

Clinical Case

48 years old Mexican male with evolution of 2 months with progresive lossweight, headache, posterior cervical pain, diplopia, dysmetria, dysfagia, ataxia, dizziness and cough reflex decreased, the motor evaluation was normal only with bilateral hyperreflexia and bilateral Babinski reflex. He arrived to emergency service.

72 hours later, the ventilatory frecuency was irregular , multidirectional nystagmus was evident, and development cuadriplegia,absence of lower cranial nerves activity but he was totally awake and he reponsed with “Yes” or “No” blinking and with vertical movements of the eyes.

Clinical case.

He was intubated with orotracheal cannula and mechanical assistance.

The result of MRI demostrated a irregular tumor localizated on superior cervical spinal cord, medulla and lower pons.

The lession enhanced with contrast on heterogenic features.

Images of Magnetic Resonance.

Sagittal and axial T1 enhanced with gadolineum showed a tumor infiltrating the higher medulla and higher cervical spinal cord.

Operative Technique

Midline Suboccipital approach was done to make a open biopsy of tumor. The transoperative view show a medulla increased of volume with abnormal vessels on the surface.

Results

Hystopathological report describe a high grade astrocytoma with necrosis areas with pseudoempalisading pattern, anaplasic cells, atypical mytosis, giant cells and glomeruloids vessels.

The patient died one week later.

Typical Pseudoempalisading pattern of necrosis, with anaplasic cellularity, atypical mytosis and giant cells on multiform glioblastoma of brain stem.(H&E).

Discussion The Locked-in syndrome is the result of a

lesion on the ventral region of the pons or medulla: the injury paralized the piramidal long tracts to the extremities and lower cranial nerves, but the tegmental area is not involucrated , preserving the supranuclear tracts of the oculomotor mechanism. The consciusness depends of the preservation of the superior tegment of pons, above the trigeminal nuclei.

The ascending reticular activating system running through the mid-brain, pons and medulla and connects to areas in the thalamus, hypothalamus and cortex.

Discussion Patterson and Grabois

analized 139 cases of locked-in syndrome and the most common was vascular etiology on basis pontis infartc and only 3 of 139 cases was for tumor of brain stem.

The mortality is 67% on vascular group and 47% for another etiologies including tumor.

Bauer describe three varities of the locked-in Syndrome:

Classic: (Plum & Posner)

Incomplete: classic + few voluntary movements of the body.

Total: Totally paralizated preserving conscius.

Conclusion

The incidence of locked-in syndrome due to tumors are anecdotic , the main factor is vascular or desmielinizating pathology.

The presence of a primary tumor on brain stem like multiform glioblastoma on a adult age is a very rare case, and the clinical presentation with a locked-in syndrome make to this case, unique in world wide´s medical literature.

References

Inci, Ôzgen: Locked-in Syndrome Due to metastatic pontomedullary tumor. Neurol Med Chir (Tokio) 43, 497-500, 2003.

Cherintong, Stears, Hodges: Locked-in Syndrome caused by a tumor. Neurology 26: 180-182, 1976.

Patterson-Grabois: Locked-In Syndrome A Review of 139 cases. Stroke 17, 4 758-764. 1986

Plum F, Posner JB. The diagnosis of stupor and coma. Philadelphia, FA:Davis; 1966,p.92-3.