Information has been received from a health professional concerning a 66 year old female was vaccinated with PNEUMOVAX 23. Concomitant therapy included influenza virus vaccine (unspecified). Subsequently the patient experienced miller-fisher variant of guillain barre syndrome and was hospitalized. The patient was presented to the hospital on October 21, 2007, after progressively worsening neurological complaints. The patient stated that she had onset of headaches a week or so before admission, soon associated with some numbness and tingling in her hands and feet, and later associated with dysarthria and swallowing difficulties, prompting her to get further medical attention in the emergency room. The patient had no viral-type illnesses; however, a week or so before symptom onset, she had a flu shot as well as PNEUMOVAX 23. The patient was evaluated by neurology with multiple tests. MRI and MRA scans demonstrated no significant abnormality that could have caused her symptoms. With the patient's history, physical, and diagnostic testing done, she was diagnosed with Miller-Fisher variant of Guillain-Barre syndrome. The patient was treated with intravenous immunoglobulin. At this point, the patient noted stability in her course, no worsening, but had not noted much improvement either. Overall, she felt somewhat weaker on the left versus the right, but noted significant difficulties with coordinating her movements. She also noted residual numbness and tingling involving her face, the inside of her mouth, in her lower extremities in a knee sock-type distribution and in a glove distribution in the upper limbs. She did admit to facial weakness with some dysarthria; however, noted improvement in her face and in her speaking. She was experiencing some intermittent bouts of pain. She had pain in her limbs, particularly her legs, described as a combination of burning pain, cramping, and irritating numbness and tingling. The patient was on gabapentin. The patient also developed what she called "spells," where she experienced an autonomic instability giving her a 3- to 10-minute period of dizziness, change in vision, headache, and hypotension. The patient was previously fully independent with activities of daily living as well as ambulation. She had no medical limitations. On physical examination, the patient had difficulty in her feet with digit identification as well as proprioception, which was absent at her toes. Light touch sensation was decreased beginning at about mid thigh level and gradually worsening down to her toes. Upper extremity sensation was not as markedly decreased as in her feet, but there was decreased light touch sensation in bilateral hands as well as decreased vibratory sensation. Myoclonus was present bilaterally in both lower extremities. Rotational dystonia was also present in her ankles, more pronounced on her left side. Digic identification was intact in her hands. With manual muscle testing in general she had 4/5 strength in her limbs, with hand intrinsic being weaker, but she was able to give some minimal resistance with intrinsic testing. Reflexes were obtained at her ankles, 1+ at the knees, and 1+ in her upper extremities. Extraocular movements were intact. No nystagmus was noted. Mild facial weakness was noted bilaterally. Her speech was mildly dysarthric. here was significant ataxia with attempts at gross as well as fine motor movements, more pronounced in the lower extremities versus the upper extremities. Comment: It should be clear from the case that the diagnosis is Guillain-Barre syndrome (GBS) from the findings of her rapidly progressive ascending limb weakness, accompanied by paresthesias, pain, and cranial nerve dysfunction. However, there are many subdivisions of GBS, all of which appear to be autoimmune diseases resulting from aberrant immune responses against various components of peripheral nerve fibers. The acute symptoms of ataxia, ophthalmoplegia, and decreased reflexes confirmed her history of the rare variant Miller Fisher syndrome (MFS), which is observed in about 5% of all GBS cases. 'Acute onset of external ophthalmoplegia is a cardinal MFS feature.' The ataxia tends to be out of proportion to the degree of sensory loss. Patients may also have mild weakness, prosis, facial palsy, or bulbar palsy. With MFS, the underlying pathology is unclear because of the affected portions of the nervous system cannot be biopsied and autopsies do not occur since it is a benign disorder. The usual course is one of gradual and complete recovery over weeks to months. A close association exist between antiganglioside antibodies and the Miller Fisher variant. Ant-GQ1b antibodies have a relatively high specificity and sensitivity for the disease. Dense concentrations of GQ1b ganglioside are found in the oculomotor, uochlear, and abducens nerves, which may explain the relationship between anti-GQ1b antibodies and ophthalmoplegia. Our patient's risk factor that was believed to have led to her disease was the influenza and/or PNEUMOVAX vaccination a week prior to the symptoms. Recent influenza immunization has been associated with GBS. One study reviewed the cases of GBS during the 1992-1993 and 1993-1994 influenza seasons and found an adjusted relative risk of 1.7 cases per 1 million influenza vaccination. The Vaccine Adverse Event Reporting System, which is administered by the Centers for Disease Control and Prevention and the US Food and Drug Administration co monitor reports of suspected adverse events after immunization, concluded in its 2003 surveillance summary that the risk of developing vaccine-associated GBS is less than the risk of severe influenza. In addition, despite increasing doses of administered vaccine during the 4 previous influenza seasons, the number of GBS reports has remained stable. In conclusion GBS (acute inflammatory demyelinating polyradiculoneuropathy) is the most common cause of acute progressive flaccid paralysis. The variant MFS is a rare disorder with rapidly evolving ataxia, areflexia, mild limb weakness, and ophthalmoplegia. Sensory loss is unusual, but proprioception may be impaired. Demyelination and inflammation of cranial nerves III and VI, spinal ganglia, and peripheral nerves occur. Also, reduced or absent sensory nerve action potentials, tibal H reflex is usually absent. Resolution occurs in 1 to 3 months.' No further information is available. A copy of the literature article is attached as further documentation of the patient's experiences.