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Clinical Neuroscience News, June 2013

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Case Study: Severe Headache Resulting from Craniocervical Instability

Harold L. Rekate, MD, FACS, FAAP and
Salvatore Insinga, DO


This 46-year-old female came to us with a long history of health problems consistent with fibromyalgia and chronic fatigue syndrome. She reportedly managed well enough with exercise until 2007 when she hit her head on the edge of a cabinet and suffered the first of her chronic severe prolonged headaches. Since then, the headaches began to interfere with her ability to work. She also reported gastroesophageal reflux, irritable bowel syndrome, lightheadedness, pervasive fatigue, burning pain in neck and shoulders, tingling and numbness in extremities and nystagmus. Her pain reportedly spiked with the Valsalva maneuver and was relieved when lying down.

Consulting neurosurgeons near home, she was assured that she did not have Chiari I. She then sought treatment at a renowned, out-of-state headache institute, had Botox® injections to reduce muscle tension, and took various pain relief medications, none of which brought relief. In 2010, coincident with a lumbar puncture test, she experienced two weeks of low-pressure headaches followed by a week of improvement, leading another specialist to diagnose pseudotumor cerebri or idiopathic intracranial hypertension. The patient was urged to try therapeutic lumbar punctures every 4-6 weeks. She then sought our expertise.

We began by taking a full patient history. We also supplemented MRIs from her previous physicians with our own dynamic MRI and identified a severe degree of tonsillar descent. Her images indicated an abnormally acute retroflexed odontoid process causing distortion of the upper end of the spine into the brainstem. We also detected the absence of a cisterna magna between the cerebellum and the medulla oblongata where CSF normally drains.

Surgical intervention consisted of performing an occipitocervical decompressive craniectomy to relieve the area of neural compression. Subsequently, using ultrasound guidance, traction and realignment proceeded to establish CSF flow to a newly created cisterna magna anterior to the brainstem. Intraoperative fluoroscopic 3D imaging showed that the cervical spine was now in normal alignment. The operation concluded with fixation and fusion of the cranium to the cervical spine in this newly formed angle. Postoperatively the patient was in an improved state and returned home. 

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Case Study: A.P.

Preoperative sagittal brain and cervical MRI showing length of 9mm Grabb

Case Study: A.P.

Postoperative MRI of the cervical spine showing that the elements of the cervical spine in front the brainstem are no longer pushing into the brain

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