Tomoyuki Kamata

Cerebral ischemic attack caused by postprandial hypotension.

BACKGROUND Food ingestion sometimes induces systemic hypotension (postprandial hypotension). Although the possibility of stroke occurring postprandially has been suggested, no cases have been reported until now. CASE DESCRIPTION A 78-year-old man experienced repeated transient ischemic attacks after almost every ingestion of food and showed orthostatic and postprandial hypotension. An angiogram revealed occlusion of his left carotid artery and stenosis of his right middle cerebral artery. CONCLUSIONS Postprandial as well as orthostatic hypotension can be a risk factor for stroke in patients with severe occlusive cerebrovascular disease.

Spreading of amyotrophic lateral sclerosis lesions—multifocal hits and local propagation?

Objective To investigate whether or not the lesions in sporadic amyotrophic lateral sclerosis (ALS) originate from a single focal onset site and spread contiguously by prion-like cell-to-cell propagation in the rostrocaudal direction along the spinal cord, as has been hypothesised (the ‘single seed and simple propagation’ hypothesis). Methods Subjects included 36 patients with sporadic ALS and initial symptoms in the bulbar, respiratory or upper limb regions. Abnormal spontaneous activities in needle electromyography (nEMG)—that is, fibrillation potentials, positive sharp waves (Fib/PSWs) or fasciculation potentials (FPs)—were compared among the unilateral muscles innervated by different spinal segments, especially between the T10 and L5 paraspinal muscles, and between the vastus medialis and biceps femoris. Axon length and the proportion of muscle fibre types, which are both related to motoneuronal vulnerability in ALS, are similar in the paired muscles. Results Fourteen of 36 patients showed a non-contiguous distribution of nEMG abnormalities from the onset site, with skipping of intermediate segments. In eight of them, the non-contiguous pattern was evident between paired muscles with the same motoneuronal vulnerability. The non-contiguously affected lumbosacral lesions involved motoneuron columns horizontally or radially proximate to one another, appearing to form a cluster in four of the eight patients. FPs, known to precede Fib/PSWs, were shown more frequently than Fib/PSWs in all the lumbosacral segments but L5, suggesting that 2nd hits occur at L5 and then spread to other lumbosacral segments. Conclusions In sporadic ALS, the distribution of lower motoneuron involvement cannot be explained by the ‘single seed and simple propagation’ hypothesis alone. We propose a ‘multifocal hits and local propagation’ hypothesis instead.

Shakuyaku-kanzo-to induces pseudoaldosteronism characterized by hypokalemia, rhabdomyolysis, metabolic alkalosis with respiratory compensation, and increased urinary cortisol levels.

BACKGROUND Licorice, the primary ingredient of the Japanese herbal medicine shakuyaku-kanzo-to, can cause pseudoaldosteronism. Thus, shakuyaku-kanzo-to can cause this condition. CASE DESCRIPTION A 79-year-old woman was brought to the emergency room. She had been experiencing general fatigue, numbness in the hands, and weakness in the lower limbs and could not stand up without assistance. She presented with hypokalemia (potassium level, 1.7 mEq/L), increased urinary excretion of potassium (fractional excretion of K, 21.2%), abnormalities on an electrocardiogram (flat T waves in II, III, AVF, and V1-6), rhabdomyolysis (creatine kinase level, 28,376 U/L), myopathy, metabolic alkalosis with respiratory compensation (O(2) flow rate, 2 L/min; pH, 7.473; pco(2), 61.0 mm Hg; po(2), 78.0 mm Hg; HCO(3), 44.1 mmol/L), hypertension (174/93 mm Hg), hyperglycemia (blood glucose level, 200-300 mg/dL), frequent urination, suppressed plasma renin activity (0.1 ng/mL/hour), decreased aldosterone levels (2.6 ng/dL), and increased urinary cortisol levels (600.6 microg/day; reference range, 26.0-187.0 microg/day). CONCLUSIONS In this case, the observed reduction in the urinary cortisol levels, from 600.6 to 37.8 microg/day, led to a definitive diagnosis of pseudoaldosteronism instead of the apparent mineralocorticoid excess syndrome. Discontinuing shakuyaku-kanzo-to treatment and administering spironolactone and potassium proved effective in improving the patients condition. Medical practitioners prescribing shakuyaku-kanzo-to should take into account the association between licorice, which is its main ingredient, and pseudoaldosteronism.

Posterior Cerebral Artery Laterality on Magnetic Resonance Angiography Predicts Long-Term Functional Outcome in Middle Cerebral Artery Occlusion

Background and Purpose— Prominent posterior cerebral artery (PCA) laterality upon 3-dimensional time-of-flight magnetic resonance angiography is often encountered in patients with middle cerebral artery occlusion. We hypothesized that this sign is correlated with improved functional outcome in patients with middle cerebral artery occlusion treated with intravenous recombinant tissue plasminogen activator. Methods— Fifty acute ischemic stroke patients with middle cerebral artery occlusion were treated with intravenous recombinant tissue plasminogen activator from April 2007 to October 2009. All patients routinely underwent initial (first 3 hours) magnetic resonance scans on admission, and additional follow-up (14–21 days after stroke onset) computed tomography scans. Two film readers blinded to all clinical information assessed the presence or absence of PCA laterality on magnetic resonance angiography. We retrospectively analyzed the clinical and radiologic data on all patients. Results— Out of 50 patients, 20 showed PCA laterality on magnetic resonance angiography. National Institute of Health Stroke Scale score 7 days after stroke onset was significantly lower (P=0.007), and infarct volume on follow-up computed tomography was significantly smaller (P=0.009) in patients with PCA laterality than in patients without this sign. Multivariate logistic regression analyses showed an adjusted odds ratio of 8.49 for a favorable outcome (modified Rankin Scale score 0–1 at 6 months) in patients with PCA laterality (95% CI: 1.82 to 55.8, P=0.005). Conclusions— The presence of PCA laterality on magnetic resonance angiography before intravenous recombinant tissue plasminogen activator can be used as a predictor of favorable functional outcome in patients with middle cerebral artery occlusion, probably due to improvement of recanalization rate.

Serum amyloid A level is increased in neuromyelitis optica and atypical multiple sclerosis with smaller T2 lesion volume in brain MRI

Serum amyloid A (SAA) is known to promote the development of T helper 17 cells (Th17) and can be a critical mediator of disease pathogenesis. We analyzed SAA levels in 40 patients with multiple sclerosis (MS) and related disorders, and 10 with non-inflammatory neurological disease (NIND) as controls. We found that SAA levels were significantly increased in neuromyelitis optica (NMO) patients and relapsing and remitting MS (RRMS) patients showing atypical phenotype with spinal cord lesions and smaller T2 lesion volume in brain MRI, resembling NMO. Therefore, SAA levels can be associated with clinical phenotypes in MS and NMO.

Acute bacterial myositis due to Staphylococcus aureus septicemia

Staphylococcus aureus is a causative organism of many diseases, but acute bacterial myositis is very rare in temperate climates.’ It is characterized by single or multiple suppurative abscesses, and in most cases septicemia does not accompany its course. We present an adult case of acute bacterial myositis caused by S aureus septicemia. Case report. A 58-year-old man was admitted to our hospital because of a consciousness disturbance and myalgia. The patient had been well until 1 week previously, when he experienced slight fever. A few days later, myalgia developed in his right leg and subsequently became generalized. He was discovered by his daughter muttering incomprehensible words and was admitted as an emergency. The patient was a train sweeper. He had no personal or family history of illness. His body temperature was 38.8 “C, his pulse 90, and his blood pressure 130/90 mmHg. He was lethargic and disoriented to place and time. His skin was generally dry, and numerous red rashes were present on his trunk and extremities. There was a small cut in his right thumb that he had sustained at work about 10 days before. On neurologic examination, muscle tone was generally reduced, and there was diffuse muscle tenderness, especially severe in both legs. The deep tendon reflexes were diminished or absent in the lower extremities, but no other neurologic symptoms were found. The results of the urine and stool examinations were normal. The 1-hour sedimentation rate was 55 mm. The peripheral blood count showed leukocytosis (14,600/mm3) and thrombocytopenia (93,000/mm3). Blood chemistry studies revealed elevated CK activity (1,588 IUA [normal range, 10 to 601), LDH (897 IU/l [normal range, 250 to 5001), GOT (147 UA [normal, <401), glutamate pyruvate transaminase (44 UA [normal, <30] ), aldolase (17.7 IUA [normal range, 1.2 to 7.61), BUN (34 mg/dl [normal range, 7 to 201), and myoglobin (640 ng/ml [normal, <601). Creactive protein was strongly positive and fibrin degradation products were slightly elevated. Exotoxin was also positive. A

Taste impairment in Miller Fisher syndrome

Miller Fisher syndrome (MFS) was first described by Charles Miller Fisher in 1956 [1]; its cardinal clinical features are external ophthalmoplegia, ataxia, and areflexia. Taste impairment is considered to be an extremely rare clinical feature of MFS [2]. Here, we report a case of MFS with taste impairment and review the previously reported similar cases. A 53-year-old male was referred to our department because of taste impairment, diplopia, and gait disturbance. About 1 month before these symptoms emerged, he had experienced symptoms of an upper respiratory infection which spontaneously resolved. On admission, he was fully alert, his body temperature was 36.5 C and his blood pressure was 171/113 mmHg. He complained of diplopia, but neurological examination did not reveal obvious ocular movement abnormalities. He also complained of dysesthesia around his mouth and taste impairment, but did not have facial nerve palsy. He did not recognize sourness well, especially on his palate. He did not complain of lacrimal hyposecretion. He had no weakness in his extremities, but deep tendon reflexes were absent. He had ataxia in his extremities and trunk, and performed tandem gait with difficulty. Immunoglobulin (Ig) G anti-GQ1b antibodies were detected in the patient’s serum. Cerebrospinal fluid examination revealed a mild elevation of protein without pleocytosis: cell 1.3/ll, protein 50.2 mg/ dl, glucose 59 mg/dl, and oligoclonal bands were present. Brain magnetic resonance imaging operated on days 2 and 5 after disease onset revealed no abnormalities which could explain his neurological symptoms. Nerve conduction studies of his upper and lower extremities were normal. Electrogustometry revealed elevated thresholds in his right and left soft palates, but normal thresholds in the anterior two-thirds and posterior one-third of his tongue, suggesting dysfunction of the bilateral greater petrosal nerves. Four days after admission, neurological examination revealed obvious ocular movement abnormalities, impairment of abduction, adduction and supraduction of both eyes, resulting in a diagnosis of MFS. Intravenous immunoglobulin (0.4 g/kg/day) was immediately administered and his symptoms, including taste impairment, gradually improved. On day 35 after disease onset, electrogustometry revealed normal thresholds in his right and left soft palates. The frequency of taste impairment in patients with Guillain–Barré syndrome has been reported to be 0.6–2 % [3, 4]. Since only two other cases with MFS presenting with taste impairment have been reported in the literature [2, 5], taste impairment is a very rare clinical feature of MFS. Clinical features of these two patients and the present case are summarized in Table 1. Taste impairment in MFS could independently occur without facial nerve palsy and could be an initial symptom of MFS. Uchibori et al. [2] postulated that the presence of specific antibodies, including anti-GQ1b antibodies, might affect peripheral nerves associated with taste and taste buds in MFS patients with taste impairment. Since taste disturbance emerged without facial nerve palsy, a similar mechanism might be associated with the symptom in the present case. Because a continuous spectrum was suggested to exist between MFS Y. Yagi (&) H. Yokote Y. Watanabe T. Amino T. Kamata Department of Neurology, Musashino Red Cross Hospital, Kyonancho 1-26-1, Musashino, Tokyo 180-8610, Japan e-mail: yyagi-tmd@umin.ac.jp

Tremor in Klinefelter’s syndrome improved by testosterone administration

Tremor in Klinefelter’s syndrome is believed to be essential tremor since the publication of “Klinefelter’s syndrome and essential tremor” in 1969. However, the author also stated that tremor in Klinefelter’s syndrome might differ from essential tremor. A 71-year-old man with Klinefelter’s syndrome who suffers from postural hand tremor is described. The electromyogram indicated lower motor neuron disturbance and chronic neurogenic change. The muscle biopsy indicated neurogenic muscle atrophy. Upon testosterone administration, the amplitude of tremor was reduced and a gradual improvement in handwriting was observed. The tremor in this patient was different from essential tremor. The foresight by Baughman in 1969 proved to be true in this patient. This case report provides new insights into the pathogenesis and treatment of tremor in Klinefelter’s syndrome, which would benefit patients who suffer from the tremor.

Cervical dystonia in an Alzheimer's disease patient treated with donepezil.

The cholinesterase inhibitor donepezil is a safe, effective, and widely used drug for the long-term symptomatic treatment of Alzheimer’s disease (AD) [1]. Dystonia is a rare complication of donepezil treatment, and some authors have reported dystonia, including Pisa syndrome, in patients treated with this drug [2]. Here, we report an AD patient treated with donepezil who developed cervical dystonia. A woman in her late 70s was referred to our department because of progressive forgetfulness. She had impaired episodic memory for recent events and her mini-mental state examination score was 12. Eye movement was normal, and she did not have akinesia or rigidity in her extremities that would suggest parkinsonism. Brain magnetic resonance imaging showed mild brain atrophy, and 99mTc-ECD single-photon emission computed tomography showed diminished blood flow in both temporal and parietal lobes, but this was more severe in the left hemisphere. She was diagnosed with AD and was started on a 5-mg dose of donepezil. Within 1 month of beginning the donepezil regimen, she developed severe torticollis and complained of difficulty in walking and eating. Physical examination revealed no abnormality other than torticollis to the left side. Physical examination and imaging studies did not provide any evidence of Parkinson’s disease or dementia with Lewy bodies, and she did not take other dopaminergic or psychotropic drugs. Therefore, we suspected that her torticollis was a side effect of donepezil. Treatment with memantine did not affect her torticollis, and we decided to discontinue donepezil treatment. Several weeks later, she experienced relief of her torticollis. She was then treated with memantine alone and had a good outcome. Dystonia is considered a rare complication of treatment with donepezil, and previous case reports and the present findings provide evidence that this complication can be due to drugs that act on the cholinergic system. Kwak et al. [2] previously reported Pisa syndrome in an AD patient treated with donepezil, and Huvent-Grelle et al. [3] reviewed Pisa syndrome in AD patients treated with 3 cholinesterase inhibitors: donepezil, rivastigmine, and galantamine. Villarejo et al. [4] suggested that cholinergic excess may induce Pisa syndrome. Although dystonia is a rare complication of cholinesterase inhibitors, clinicians must be aware of this phenomenon because of the increasing number of dementia patients and the growing use of cholinesterase inhibitors to treat them. Because cholinergic excess induced by cholinesterase inhibitors seems to be a cause of dystonia, treatment with the noncompetitive N-methyl-D-aspartate receptor antagonist memantine [5] is an alternative for AD patients who experience side effects on cholinesterase inhibitors.

A sporadic case of late-onset familial amyloid polyneuropathy with Bence-Jones proteinuria.

Sirs: Peripheral neuropathies are found to be associated with primary (AL) and familial amyloidoses [2]. In AL amyloidosis, clonal plasma cells produce immunoglobulin light chains that are amyloidogenic and detected as serum M protein or urinary free monoclonal light chains, Bence-Jones proteins (BJP). AL amyloidosis has a wide spectrum of organ involvement, including the heart, kidney, and peripheral nervous system; autonomic and sensory neuropathy is a relatively common feature. The most common form of familial amyloidosis is caused by mutant transthyretin (TTR). More than 50 different substitutions of single amino acids in TTR cause familial amyloid polyneuropathy (FAP). The most common is a substitution of methionine for valine at position 30 (Met-30), which was first described in Portugal and has since been reported to occur in persons of almost every ethnic background (FAP type I). FAP I with the TTR Met-30 is characterized by onset between the ages of 20 and 40 years of selective loss of pain and temperature sensation (dissociated sensory loss) with painful paresthesiae and early autonomic dysfunction, and later development of muscle weakness and loss of tendon reflexes and other sensory modalities. Here we describe a sporadic case of lateonset amyloid neuropathy with urinary BJP in which the neuropathy later proved to be FAP I with the TTR Met-30. The patient was a 71-year-old Japanese man with a 2-year history of numbness of the feet. He had occasional constipation without diarrhea or orthostatic dizziness. There was no family history of neuropathy. Physical examination was normal. Neurologically, there was no muscle weakness. Deep tendon reflexes were normal in the upper extremities and diminished in the knee and ankle jerks. Sensory examination showed impairment of all sensory modalities in the feet. The tilting test showed an orthostatic decrease in blood pressure with no symptoms. Immunoelectrophoresis of the urine revealed a BJP of κ type; no M protein was detected in the serum immunoelectrophoresis. There was no increase in bone marrow plasma cells. No albumin was detected in the urine, although the urine contained BJP. Nerve conduction studies showed a decrease in the amplitude of sensory nerve action potentials. Electrocardiography, echocardiography, and renal function test were normal. Biopsies of the sural nerve and rectum revealed amyloid deposits. The presence of the BJP-κ in this sporadic case of amyloid neuropathy first suggested AL amyloidosis. However, in immunohistochemical studies of the sural nerve, the amyloid deposits were not immunoreactive with an antibody to the κ-chain, but with an antibody to TTR (Fig. 1). Therefore exon 2 of the TTR gene was amplified using DNA extracted from blood by polymerase chain reaction (PCR). Restriction fragment length polymorphism analysis of the PCR product with BalI revealed a Met-30 mutation in the TTR gene, which led to the diagnosis of FAP I with the TTR Met-30 mutation. In our experience of six FAP I patients with the TTR Met-30 mutation from unrelated Japanese families, later onset of the disease tends to be associated with a lack of family history of neuropathy or amyloidosis, mild autonomic symptoms, and no obvious dissociation in impairment of sensory modalities (data not shown), which differ from the typical FAP I presentation. Atypical features have been reported in lateonset cases of FAP I [1, 4, 6]. In neuropathy associated with AL amyloidosis that commonly shows onset at an older age, a disproportionate impairment of pain and temperature sensation is found in about one-half of patients, and bladder and rectal disturbance is more delayed than in typical FAP I [3]. On the basis of family history and clinical feaLETTER TO THE EDITORS J Neurol (1999) 246 :726–727