Toshihide Kanesaka

Serum matrix metalloproteinase-3 levels correlate with disease activity in relapsing-remitting multiple sclerosis

Background: Adhesion molecules and matrix metalloproteinases (MMPs) are known to be relevant to the ongoing development and disappearance of areas of demyelination in the white matter of the CNS of multiple sclerosis (MS) patients. This study examined whether serum matrix metalloproteinase-3 (MMP-3) levels correlate with disease activity in MS. Methods: Serum MMP-3 levels in 47 consecutive patients with relapsing-remitting MS were measured by immunoassay every 4 weeks over a 15 month period. Results: During the study period, 48 clinical relapses occurred. Serum MMP-3 levels within 1 month of relapse were significantly higher than during the remission phase. Sequential analysis showed that serum MMP-3 levels had increased transiently at the time of clinical relapse but returned to the normal range within a month. Conclusions: Circulatory MMP-3 levels are correlated with disease activity in relapsing-remitting MS. This may contribute to the breakdown of the blood-brain barrier at the time of relapse.

Emotional sweating response in a patient with bilateral amygdala damage

Sweat output on the palm or sole is distinct from thermoregulatory sweating and has been designated emotional sweating. The amygdala has been implicated in this phenomenon, but the role it plays remains unclear. We had the chance to evaluate emotional sweating in a 21-year-old female with bilateral restricted amygdala lesions caused by idiopathic subacute limbic encephalitis. At the peak of the illness, sweat responses in the palm were not evoked in this patient in conjunction with any sympathetic activation procedures, including deep inspiration, mental arithmetic, isotonic exercise, and tactile stimulation. After neurological improvement associated with diminution of amygdala lesions on the magnetic resonance imaging, normal sweat responses were incited. This indicates that the amygdala does play an important role in emotional sweating.

Successful immune treatment for non-paraneoplastic limbic encephalitis

A 21-year-old woman subacutely developed memory loss subsequent to gastroenteritis. Brain MRI with gadolinium enhancement showed symmetric involvement of the amygdala. The CSF was acellular with increased protein level. There was no evidence suggestive of neoplasm or viral infection. Combined treatment with plasmapheresis and immunoglobulin improved her clinical symptoms and lessened abnormalities manifested in the MRI. This case suggests the presence of immune-mediated limbic encephalitis without association with neoplasms or infections.

Dopamine agonist-induced antecollis in Parkinson's disease†

Few cases of dopamine agonist‐induced antecollis in Parkinsons disease (PD) have been reported. Literature review of 16 PD patients including our 3 cases with dopamine agonist‐induced antecollis showed predominance of (1) Japanese, (2) women, and (3) Hoehn‐Yahr stage of ≥3. We experienced three Japanese PD patients who subacutely exhibited antecollis following increased dopamine agonist dose that improved just after withdrawal of the agonist. One patient developed antecollis during increasing pramipexole dose in combination with cabergoline. Antecollis in another patient appeared during increasing pramipexole dose; it worsened after substituting pergolide for pramipexole, but improved after withdrawal of pergolide. Our cases indicate that there is no specific dopamine agonist causing antecollis, and it is possibly caused by a number of single dopamine agonists or a combination of them. Dopamine agonist‐induced antecollis should be considered when encountering antecollis in PD patients being treated with dopamine agonists and withdrawal of the agonist can improve symptoms.

Truncal polyradiculopathy due to sarcoidosis

We report the case of a 43-year-old woman who developed multiple cranial nerve palsy, the symptoms of which included hyposmia, visual loss, facial hypoesthesia, facial weakness, dysphagia, gustatory disturbance, and sensory disturbance of the trunk and ulnar side of the bilateral arms. The clinical features included swelling of the bilateral hilar lymph nodes, uveitis, an elevated serum angiotensin-converting enzyme level, and negative tuberculin reactions, which led to a diagnosis of neurosarcoidosis. Her symptoms improved after administration of steroids. An elevated cerebrospinal fluid cell count and protein level, a low-frequency F-wave and slightly decreased sensory nerve action potentials in bilateral ulnar nerves by nerve conduction studies, and normal findings in the spine by magnetic resonance imaging suggested that truncal hypoesthesia was caused by polyradiculopathy. Although rare, in patients with neurosarcoidosis, truncal polyradiculopathy is noteworthy findings in addition to cranial nerve palsy.

Posterior spinal artery syndrome presenting with sensory and motor disturbances of unilateral lower limb

Sirs: Posterior spinal artery (PSA) syndrome is a very rare condition that is due to infarcts in the PSA territories. Patients with PSA syndrome exhibit symptoms and signs caused by lesions in the posterior third of the spinal cord, namely the posterior column, the posterior horn, and the posterolateral portion of the lateral column [1–4, 6, 7]. Lesions in PSA syndrome are usually bilateral, and only a few reports have described a unilateral lesion [2, 4, 7]. A 63-year-old man had a sudden onset of a tingling sensation and paresthesia on his right lower limb at around 3:00 a. m. on 1 January 2003. Upon examination, he was mildly obese and his blood pressure was 200/120 mmHg with sinus rhythm. He had almost complete loss of vibration and position senses in his entire right lower limb and a marked loss of pain and touch senses at right L2 to S1 spinal segments, especially in L5 segment. He also had muscular weakness of his right iliopsoas, quadriceps and anterior tibial muscles. Tendon reflexes were absent in his right lower limb, while those in his left lower limb were normal. Saddle anesthesia was not present and he was able to urinate. He could not stand or walk, and therefore, he was admitted. Laboratory examinations showed hypercholesterolemia. A sagittal T2-weighted MRI of the tholacolumbar cord showed a T2elongated lesion in the posterior cord at T12 to L1 vertebral levels (Fig. 1A). Axial images at the T12 level revealed right posterior intramedullary lesions that seemed to involve the posterior column, the posterior horn, the posterolateral portion of the lateral column, and a part of the anterior horn (Fig. 1B). Neither CT nor conventional spinal angiography was performed because of the patient’s active bronchial asthma. Muscular strength recovered gradually, and he became able to walk with a cane about two months after onset. Tingling sensations and paresthesia with moderate sensory decrease in all modalities remained unchanged. A follow-up MRI about one year after the onset showed a remaining intramedullary lesion in the right lumbar cord. This lesion was less apparent than it had been at onset, and the cord was slightly atrophic (Fig. 1C). In this case, abrupt onset together with multiple vascular risk factors strongly suggests a vascular accident as the cause of the disease. Neurological examinations showed sensory motor disturbances in the entire right lower limb with absent tendon reflexes. This suggested that the lesion was in the right lumbar spinal cord. MRI scans revealed a right posterior intramedullary lesion at the T12 to L1 vertebral levels, namely at the lumbosacral cord. These clinical and MRI findings strongly suggested the diagnosis of PSA syndrome, which is also supported by gradual improvement of clinical symptoms and the sequential changes of MRI findings. In this patient, the loss of pain and touch sensations and areflexia were thought to be due to ipsilateral L2 to S1 segmental involvement of the posterior horn, whereas loss of vibration and position senses were thought to be due to damage of the ipsilateral posterior column or horn at L2 to S1 spinal levels. The partially recovered right leg monoparesis was probably due to partial involvement of ipsilateral lumbar pyramidal tract and/or ipsilateral anterior horn. Blood perfusion of the posterior third portion of the spinal cord is supplied by a pair of PSA, which become part of a complicated anastomotic network [5]. PSA usually originate from radiculo-pial arteries branching from the lumbar artery, iliolumbar artery, and lateral sacral artery, respectively at the L1L4, L5-S1, and S2-S5 levels [5]. These anatomical features remind us that a posterior third portion of the spinal cord is supplied by “a single vascular network” usually originating from two radiculo-pial arteries, rather than a pair of independent PSA. This concept makes it possible to understand the facts that: 1) PSA syndrome usually occurs bilaterally, and 2) unilateral PSA syndrome is very rare.