Age: 59.0
Sex: M
Vaccine: LYME
Symptoms: Hypokinesia
Clinical narrative: On 10/16/00 the pt was diagnosed with "spastic motor skills" by a neurologist. Onset from time of immunization was not provided.  A 15-day follow up report received 11/14/2001 adds: Report 2000034909-1 describes Creutzfeldt-Jakob disease in a 58-year-old male who received Lyme disease vaccine (Lymerix). Concurrent medical conditions included hypertension, coronary artery disease, hypercholesterolemia, chronic constipation, and a kidney stone "removed some years ago". The vaccine provider indicated that there were no concurrent medications at the time of immunization. The pt received his first 2 injections of Lymerix on 03/18/1999 and 04/16/1999, respectively. No adverse events were reported following receipt of these 2 injections. The pt received his 3rd injection of Lymerix on 03/20/2000. In May 2000 approximately 2 months post-immunization, he experienced severe tiredness and developed a moderately unbalanced gait with some paresthesia. He gradually worsened over time and was referred to a neurologist. The pt was seen by the neurologist on 10/06/2000. The pt reported that he "had trouble walking for the past few months. He loses his balance and falls backwards or forwards. He states his handwriting is scribbly but not small." At the time of the consult, the pt was taking nortriptyline (Pamelor) for unspecified pain, verapamil (Calan), stenolol (Tenormin) and fluvastatin (Lescol). On physical examination, the neurologist noted that the pt "has widened palpebral fissures bilaterally and a questionable lid lag...There is increased tone with spastic paraparesis bilaterally." The neurologist's impression was "spastic paraparesis etiology unknown." An MRI of the cervical spine, with and without contrast, performed 10/11/2000 revealed "Two tiny foci of signal abnormality in the spinal cord at the T1 level, seen only on the proton density images." The neurologist saw the pt on 10/13/2000. At that time, the pt reported that "he has had an episode of incontinence on occasion." Physical examination revealed "a spastic gait with paraparesis and bilateral Babinski's but no memory loss". An MRI of the brain, with and without contrast, performed 10/18/2000 revealed "Non-specific white matter disease". The pt was seen by the neurologist on 11/10/2000. At that time, the neurologist noted "EMG (electromyelography) really showed no evidence of motor neuron disease." The neurologist prescribed a quad cane "because he has so much difficulty walking." An MRI of the thoracic spine, with and without contrast, performed 11/13/2000 revealed "No significant abnormality". The previously seen areas of abnormal increased signal at the T1 level, noted in the 10/11/2000 MRI were considered to possibly "have represented a component of artifact." Analysis of cerebrospinal (CSP) fluid obtained 11/22/2000 was normal. Borrelia DNA was not detected by polymerase chain reactin (PCR) assay. Angiotonsin-1-converting enzyme level was normal. The neurologist saw the pt on 12/01/2000. The pt "continues to have significant spastic paraparesia which is clearly motor." The neurologist stated that he reviewed the history with the pt, and the pt reported that his symptoms began in March 2000, rather than May 2000, as reported by the vaccine provider. The pt was referred for physical therapy and a second neurology opinion. The pt returned to the office of the first neurologist on 12/29/2000, after being evaluated by the second neurologist. The second neurologist expressed concern "about the possibility of primary lateral sclerosis. He said he saw some fasciculations." The first neurologist was unable to appreciate any fasciculations. The first neurologist prescribed baclofen at an initial dosage of 5 mg three times daily, increased to 10 mg three times daily. The pt underwent repeat EMG and nerve conduction studies preformed by the second neurologist on 01/18/2001. Physical examination prior to the start of the studies revealed occasional fasciculations in the thights. There was no evidence on the EMG of acute denervation and no fasciculations. The impression was "Normal electrodiagnostic study of right upper extremity and both lower extremities, except for some minor findings of neuropathic recruitment in the right extensor digitorum brevis muscle, and relative slowing of right median sensory conductin across the wrist segment. The latter finding may indicate an early right sided carpal tunnel syndrome. No evidence to suggest motor neuron disease found." The pt returned to the first neurologist's office on 01/26/2001. At that time, the neurologist noted, "some cogwheel rigidity in all four extremities, and slightly masked facias. He has a lot of trouble turning with very small steps." The neurologist stated, "Although I cannot really make a diagnosis of Parkinsonism there are some Parkinsonian symptoms which I felt were worthwhile treating." He prescribed carbidopa/levodopa (Sinemet) 25/100 one tablet four times daily, gradually increasing to two tablets four times daily. The vaccine provider considered the tiredness, unbalanced gait, and paresthesia to be possibly related to Lymerix administration. These events persisted as of 12/19/2000. Info received from the neurologist on 02/26/2001 indicated that the spastic paraparesis persisted as of 01/26/2001. The pt was hospitalized at a tertiary care center on an unspecified date. A brain biopsy was performed. Based on the pathologist's examination of the biopsy specimen, a diagnosis of Creutsfeldt-Jakob disease was made. On 01/26/2001 the neurologist stated, "I still feel that this myelopathy may be related to the Lyme's vaccine." However on 11/07/2001, the neurologist stated he did not know what may have caused Creutsfeldt-Jakob disease in this pt. As a result of the most recent info, received 11/07/2001, this report has been reclassified as serious - unexpected. The neurologist stated that as of 11/02/2001, the Creutsfeldt-Jakob disease was ongoing. A 15-day follow up report received 12/12/2001 adds: The pt was hospitalized at a tertiary care center on 03/12/2001. His attending physician wrote, "During the last 9 months he has had progressive weakness, dysarthria, dysphagia, intermittent shortness of breath, and a poor memory with progressive gait abnormalities. He has become totally dependent on activities of daily living. Also, he has had episodes of urinary incontinence. He has not had any seizures, myoclonus or other deficit." On physical examiniation, "he was alert, oriented in person, but not in time or place. He had a severe dysarthria. He denied any delusions or hallucinations. On examination of his cranial nerves he has diminished voluntary saccades and an increased jaw jerk with severe dysarthria. On examination of his gait he was able to stand up with help and had a severe bilateral spasticity with increased tone and was unable to ambulate without assistance. On examination of his deep tendon reflexes they were aclonic. The plantar responses were extensive and on sensory exam he did not have any abnormalities." A small bowel biopsy ruled out Whipple disease. CSF was sent to a different tertiary care center for electrophoresis. The CSF was "markedly and unequivocally positive" for prion disease. A brain biopsy performed 03/21/2001 produced "pieces of neocortical brain tissue exhibiting mild spongiform change within the gray matter. No evidence of senile plaques, neurofibrillary tangles, Lawry bodies, amyloid angiopathy or vasculitis seen. No significant gliosis seen. No significant pathology is seen in the subcortical white matter...Western immunoblot analysis was performed on the frozen biopsy tissue by the ...National Prion Disease Pathology Surveillance Center...The immunoblot reveals the presence of protease-resistant, scrapie prion protein confirming the diagnosis of prion disease based on current criteria. The immunoblot pattern is consistent with the diagnosis of Creutzfeldt-Jakob disease. DIAGNOSIS: Spongiform encephalopathy, consistent with Creutzfeldt-Jakob disease (brain biopsy)." Magnetic resonance imaging (MRI) of the head "showed consistent findings with Jakob-Creutzfeldt disease". An electroencephalogram (EEG) "showed diffuse abnormalities with burst suppression periodicity." The pt was discharged from the hospital on 03/23/2001; "he was slightly more confused than on admission and totally disabled". Discharge diagnosis was "progressive dementia due to Jakob-Creutzfeldt disease".... The first neurologist saw the pt on 10/22/2001. At that time, the pt was hospitalized for an upper respiratory tract infection (non-event). The neurologist noted that the pt had been "on some experimental treatment with quinacrine, although he has not done very well; he has been on the medication for about the past one month or so...He now has severe myoclonus, he is nonverbal and he just makes mumbling noises which do not make sense. He does not follow any command. The pt is confined to home." On physical examination, "the pt looks alert, but he is totally noncommunicative. The pt mumbles, but does not make any sense...The pt has some mouth movement and significant myoclonus of all four extremities. There is increased tone in all four extremities. There are brisk and equal reflexes. The plantar responses are equivocal bilaterally." The neurologist's impression was "This pt had end-stage Creutzfeldt-Jakob disease." As a result of the info received on 11/07/2002, this report has been reclassified as serious, unexpected. The neurologist stated that, as of 11/27/2001, the Creutzfeldt-Jakob disease was ongoing.