Subacute autonomic and sensory neuropathy closely related to cytomegalovirus infection preceded by frequent syncopal attacks.

A 73-year-old woman who had hypertension developed a slight fever and general malaise with laboratory-proven hepatic dysfunction as well as frequent syncopal attacks 3 months before admission to our hospital. One month later, she developed urinary retention and distal limb numbness. Upon admission, her neurological examination showed reduced limb tendon reflexes, glove and stocking-type numbness, and diminished senses of touch, temperature, pain, and distal leg vibration and position. Serum cytomegalovirus (CMV) IgM antibody and CMV IgG antibody were elevated on admission, and both decreased thereafter, confirming CMV infection. No serum anti-ganglioside antibody was detected. Cerebrospinal fluid revealed a mild pleocytosis and elevated proteins. Compound muscle action potential (CMAP) amplitudes of the tibial and peroneal nerve were slightly reduced. Sensory nerve action potential (SNAP) amplitudes of the median and ulnar nerves were reduced, and sural SNAP was not evoked. Systolic blood pressure dropped 48 mmHg when the patient assumed a standing position from a supine one, demonstrating orthostatic hypotension, and a cold pressor test was abnormal, both indicating an obvious hypofunction of the sympathetic nerve. The postganglionic autonomic nerve appeared to be damaged because the accumulation of [(123)I] meta-iodobenzylguanidine was reduced on myocardial scintigraphy. These findings combined together led us to make a diagnosis of subacute autonomic and sensory neuropathy associated with CMV infection in this case. Following an eventless administration of oral fludrocortisones, intravenous immuno-globulin (IVIg) was given after one month of the hospitalization with a remarkable reduction of the syncope. This case is instructive in two points. One is that there may be a couple of months with syncope alone before the sensory disturbance appearance, and the other is that IVIg may be considerably effective for the patient-annoying syncopes. To our knowledge, this is the first report of subacute autonomic and sensory neuropathy caused by CMV infection.

Intraoperative Neural Mobility and Postoperative Neurological Recovery in Anterior Cervical Decompression Surgery: Evaluation With Intraoperative Sonography.

STUDY DESIGN: A study using intraoperative sonography (IOS) was conducted for evaluating neural mobility in anterior cervical decompression surgery. OBJECTIVES: To analyze decompression status and mobility of the spinal cord and the nerve root during anterior cervical decompression and to clarify its relevance to the postoperative neurological recovery. SUMMARY OF BACKGROUND DATA: Several papers introduced the usefulness of IOS assessments; however, there have been no reports systematically evaluating the neural mobility in anterior cervical decompression surgery. METHODS: Eighty-four consecutive patients with compressive myelopathy who underwent anterior cervical decompression procedures were studied. The decompression status of the spinal cord was evaluated with IOS and classified into 3 grades according to the restoration pattern of the space ventral to the cord. Pulsatile motion of the spinal cord in cranio-caudal direction was named "sliding pulsation" and graded into 3 groups. The nerve root pulsation was also assessed using the IOS short-axis views. This study analyzed whether those neural mobility in anterior cervical decompression surgery had relevance to postoperative neurological recovery, which was assessed by the Japan Orthopaedic Association score. RESULTS: The mean recovery rate of the Japan Orthopaedic Association score was 59.1% in total. According to the decompression status in IOS, 67 patients who acquired space ventral to the spinal cord indicated 64.3% of the recovery rate which was significantly higher than 36.6% of the other patients on an average. As to the sliding pulsation of the cord, 10 patients who failed to show this particular motion indicated significantly lower recovery rate as 36.9%. In addition, 6 patients who did not exhibit nerve root pulsation indicated just 29.3% of recovery rate, and 4 of them failed to show the cord sliding motion. CONCLUSIONS: Sonographic evaluation during anterior cervical decompression surgery provided very useful information of neural decompression status that had significant correlation with postoperative neurological recovery.

A new performance test for cervical myelopathy: the triangle step test.

STUDY DESIGN: Our original performance test for evaluating the severity of cervical myelopathy, the triangle step test (TST), was introduced along with an assessment of its validity. OBJECTIVE: The TST was designed to evaluate the lower extremity motor function objectively and quantitatively. This study aimed to assess the validity of the test by analyzing the relation to the other analytic methods. SUMMARY OF BACKGROUND DATA: Several rating scales and performance tests have been proposed to evaluate the severity of cervical myelopathy. Simple walking test is useful; however, the test is limited for the patients who can walk safely. METHODS: Each subject sitting on a chair was instructed to step on marks at each apex of a triangular board and the number of steps in 10 seconds was counted for each foot. The subjects were 270 cervical myelopathy patients who had visited our hospital since 2002. As a control group, 60 healthy adults also underwent this test. All subjects were simultaneously evaluated by the Nurick score, the Japan Orthopedic Association score and the finger grip and release test. An assessment of the effect of surgery was analyzed for 94 patients who underwent surgical treatments. RESULTS: The mean of the lower count for each subject (TST score) in the control group was 25.4 +/- 3.7 steps, which was superior to 18.4 +/- 5.2 steps for myelopathy patients. TST score significantly correlated to the other analytic measures for cervical myelopathy. Regarding the effect of surgery, a performance of 16.7 +/- 4.5 steps before surgery improved to 21.2 +/- 4.9 steps at follow-up. Patients who could step more than 20 times before surgery, showed greater neurologic recovery. CONCLUSION: TST score correlated with other analytic methods for cervical myelopathy. This test is very useful to quantitatively evaluate lower extremity function and its improvement following surgical intervention.

Spinal cord morphology and dynamics during cervical laminoplasty: evaluation with intraoperative sonography.

STUDY DESIGN: An intraoperative sonographic study for evaluating spinal cord decompression status was conducted. OBJECTIVES: To analyze the spinal cord decompression status using intraoperative sonography and to evaluate the relation to postoperative neurologic recovery following cervical laminoplasty. SUMMARY OF BACKGROUND DATA: Since the 1980s, several papers have introduced that the intraoperative ultrasound allowed assessment of the adequacy of decompression and configuration of the spinal cord in compressive myelopathy. However, there have been no reports systematically evaluating the decompression status. METHODS: Spinal cord decompression status of 80 consecutive patients with cervical compressive myelopathy was evaluated by intraoperative sonography during cervical laminoplasty. The decompression status was classified into 4 grades according to the restoration pattern of the space ventral to the cord. In addition, amplitude of the cord pulsation and compression type in axial view were also assessed. This study analyzed whether those findings from intraoperative sonography had relevance to preoperative spinal cord conditions evaluated by magnetic resonance images (MRI) and postoperative neurologic recovery. RESULTS: The mean neurologic recovery rate was 48.3% at the final follow-up. According to intraoperative sonographic evaluation, 50 cases who acquired the space ventral to the cord showed significantly higher recovery rate (59.2%) than 30 cases who failed to acquire the space (recovery rate, 31.0%) in total. Twenty-seven of 60 cases with intramedullary T2 high lesion on preoperative MRI more frequently failed to restore the ventral space, and their neurologic recovery rate indicated 30.2%. The amplitude of spinal cord pulsation or compression type did not correlate with the neurologic recovery. CONCLUSION: Intraoperative sonography during laminoplasty appears to be very useful for evaluating spinal cord decompression status. Our original classification system based on restoration patterns of the space ventral to the spinal cord is considered to be practical for predicting neurologic improvement in cervical compressive myelopathy.

Congenital anomaly of cervical vertebrae is a major complication of Rubinstein-Taybi syndrome.

Rubinstein-Taybi syndrome (RTS; MIM# 180849) is a well-known malformation syndrome, characterized by broad thumbs and halluces, a characteristic facies, short stature, and mental retardation. RTS is accompanied by a variety of morbid complications, particularly of the skeleton. Based on the experience of five RTS patients with malformation of the craniovertebral junction, we draw attention to previously unrecognized life-threatening complications of RTS, including instability of C1-C2, os odontoideum, hypoplasia of the dens, and fusion of the cervical vertebrae. One patient developed severe cervical myelopathy. Malformation of the cervical spine may be a common syndromic constituent of RTS, to which special attention should be paid to prevent its neurologic sequelae.

Combined anterior-posterior fusion for cervical spondylotic myelopathy in patients with athetoid cerebral palsy.

OBJECT: Cervical spondylotic myelopathy (CSM) or myeloradiculopathy, frequent in adults with athetoid cerebral palsy, is a serious secondary disability in patients with an existing congenital handicap. Although several surgical procedures have been described for CSM in adults with athetoid cerebral palsy, none has had satisfying long-term results. The object of this study was to evaluate the effectiveness and safety of combined anterior-posterior fusion with wave-shaped rods and its influence on the stability of other spinal segments. METHODS: Twenty-three patients with CSM and athetoid cerebral palsy underwent posterior fusion with wave-shaped rods combined with anterior interbody fusion with internal fixation; 20 patients, 17 men and three women, were followed for more than 5 years. This procedure yielded good results. The mean follow-up period was 8.7 years (range 5-17 years). At 1-year follow-up examination, ambulation had improved in 12 patients. Upper-extremity pain, deltoid muscle weakness, and ability to self-feed improved in almost all patients. Myelopathy recurred in one patient 8.5 years after surgery. The mean motion angle at the adjacent level to the fixed segment did not change postoperatively, but the mean motion between C-1 and C-2 increased and slight atlantoaxial subluxation occurred postoperatively in five patients. CONCLUSIONS: Combined anterior-posterior fusion can effectively improve neurological function in patients with CSM and athetoid cerebral palsy, even in those with severe involuntary movements. Postoperative rigid external fixation is not required.