Search

A 64-year-old male came to our hospital emergency department with fever and consciousness disturbance. Culture tests of blood and spinal fluid samples revealed meningococci (Neisseria meningitidis), and we made a diagnosis of meningococcal meningitis. Brain magnetic resonance imaging (MRI) findings revealed ventriculitis. Ceftriaxone was administered for 17 days, however, relapse was noted after that was discontinued, with neutropenia and renal impairment thought to be adverse reactions to the beta-lactam antibiotic. Hence, treatment was switched to oral administration of moxifloxacin for a total of 12 weeks, including in an outpatient setting. After moxifloxacin was discontinued, no side effects or relapse were seen, and treatment was ended. Although antibacterial agents generally show favorable effects for meningococcal meningitis, we consider that sufficient antimicrobial therapy is difficult in cases complicated with ventriculitis.

We report a 63-year-old right-handed man who presented an alien hand syndrome (AHS). He complained of clumsiness of his left hand and admitted to our hospital. On the first examination, he presented left homonymous hemianopia, left spatial neglect and left limb ataxia, but neither paralysis nor sensory impairment. A few days after, he complained that his left hand was controlled by someone else, and we considered this phenomenon as AHS. At that time, he lost sensation of almost all modalities including deep sensation on his left upper and lower limb. Magnetic resonance image examination was performed, and it showed acute cerebral infarction at right posterior cerebral artery territory including right thalamus (ventral posterior lateral nucleus). Generally, AHS is caused by left mediofrontal and callosal lesion (frontal type AHS), or by callosal with bilateral frontal or without frontal lesion (callosal type AHS). However, some cases were reported that they presented AHS after damage of the basal ganglia, right thalamus, right occipital or inferior parietal lobe. Some authors described this phenomenon as "sensory" or "posterior" type AHS. In such cases, included our case, we speculate that sensory impairment causes AHS. Especially in our case, AHS might be caused by not only the sensory impairment but also by left homonymous hemianopia and left spatial neglect. So, because of these symptoms, our patient could not recognize the motion of the left hand, and presented AHS. We think that this "sensory" or "posterior" type AHS should be distinguished from frontal and callosal type AHS.

A 36-year-old woman suffered from steroid-responsive relapsing myelitis associated with hypocomplementemia, thrombocytopenia, and anti-cardiolipin antibody. At the second attack of paraplegia, neck flexion in a supine position induced uncomfortable dysesthesia radiating into the ulnar side of the bilateral forearms. Both truncal and neck flexion resulted in painful dysesthesia down into both lower limbs also. Cervical MRI showed 2 gadolinium-enhanced dorsal-dominant lesions in the spinal cord at C 5/6 and C 6/7 disc levels. Enhancement of Lhermitte sign by truncal flexion might be useful to detect multiple lesions in the dorsal column.

A seventy-six-year-old man suddenly suffered from paraplegia and pain in both legs. He had been maintained on hemodialysis and committed a suicide attempt by cutting the shunt at the paraplegic attack. He was brought to the emergency ward for the treatment of hemorrhagic preshock. Neurological examination demonstrated flaccid paraplegia, loss of tendon reflex in the lower extremities, dissociated sensory loss below the fourth lumbar level; and incontinence in defecation. MRI showed T2 shortening in the ventral spinal cord caudal below the level of the eleventh thoracic cord. Postmortem examination confirmed ischemic infarct in the central area of the spinal cord, associated with disseminated cholesterin emboli in the small arteries. This case was the first MRI demonstration of central necrosis caused by cholesterin emboli, and may emphasize the significance of cholesterin emboli in the spinal arterial disorders in the aged.

A 56-year-woman with type 2 respiratory failure due to diaphragmatic dysfunction in Charcot-Marie-Tooth disease (CMT) is reported. The patient, who had a 50-year history of CMT, was referred to our hospital because of nocturnal dyspnea. Arterial blood gas analysis on admission showed marked hypoxia with hypercapnia, and physical examination revealed thoracoabdominal paradoxus in the supine position. Chest radiography revealed elevation of both sides of the diaphragm. The vital capacity and arterial blood gas pressure in the sitting position were markedly higher than those in the supine position. Electrical phrenic nerve stimulation failed to produce any convincing muscle action potential in the diaphragm. These findings suggested that her respiratory failure was induced by both diaphragmatic dysfunction caused by bilateral phrenic nerve palsy due to CMT. Treatment of this patient was started at home with a pressure support ventilator, resulting in satisfactory clinical improvement. In general, respiratory muscle impairment is a rare phenomenon in a patient with CMT. However when a patient with CMT complains of dyspnea or if unexpected heart failure develops, it is important to keep in mind that CMT may be associated with phrenic nerve palsy.

We report four patients with a new type of familial parkinsonism and dementia consisting of an autosomal dominant inheritance, dopa-responsive parkinsonism, severe dementia, variable myoclonus and autonomic disturbances. Autopsy of two patients revealed symmetrical cerebral atrophy with fronto-temporal dominant distribution, and marked depigmentation in the substantia nigra and locus ceruleus. Neuronal loss and gliosis were observed in the deep cerebral cortex and amygdala as well as in the areas vulnerable to Parkinson's disease. In the cerebral cortex, swollen neurons with frequent granulovacuolar changes were observed, consisting of ballooned neurons and those with argyrophilic intracytoplasmic inclusions, in addition to neuropil threads. Atypical neurofibrillary tangles, which barely stained with tau antibodies, were numerous in the upper cortical layers, consisting of 15-nm straight tubules. In addition, tau-negative astrocytic fibrillary tangles were also frequent. Electron microscopically, the ballooned neurons and argyrophilic neuronal inclusions contained filamentous structures coated with fuzzy electron-dense deposits. The inclusions showed immunohistochemical features different from those of cortical Lewy bodies and Pick bodies. Occasional Lewy bodies were present in the brain stem lesions of both patients. In two of our patients, the pathology in the brain stem was similar to that of Parkinson's disease, whereas their cerebral pathology was unusual and has not been reported previously.

In a case of neurosarcoidosis with bilateral facial nerve palsy and hydrocephalus, contrast-enhanced magnetic resonance imaging (MRI) study and angiotensin converting enzyme (ACE) activities in cerebrospinal fluid (CSF) were valuable for the diagnosis and the follow up. Facial nerve lesions were demonstrated on gadolinium-DTPA enhanced MRI. The disappearance of enhancement was concomitant with the amelioration of facial nerve palsy after corticosteroid therapy.

A rare point mutation at nucleotide position 8356 in the transfer RNA gene in mitochondrial DNA was found in a Japanese family. Our proband had migraine and dementia associated with lactic acidosis in addition to myoclonic epilepsy with ataxia and ragged-red fibres in a muscle biopsy specimen consistent with the clinical characteristics of myoclonic epilepsy with ragged-red fibres (MERRF). His mother, who had the same point mutation, also had migraine but without myoclonus or ataxia. His aunt, who had the same point mutation and migraine, developed diabetes mellitus, encephalomyopathy and several stroke-like episodes associated with lactic acidosis (MELAS). This is the third family with the rare mutation seen in American and Italian families. The mutation may not be specific to Caucasians, and is probably closely related to the MERRF/MELAS overlap syndrome.

A patient presenting as severe respiratory failure due to alveolar hypoventilation resulting from respiratory muscle weakness is described. Diagnosis of polymyositis was established by electromyography and muscle biopsy. Steroid therapy was initiated and it ameliorated respiratory failure remarkably. Selective respiratory muscle involvement due to polymyositis has been suggested in this patient. Although fatal respiratory failure is a very rare complication in polymyositis, polymyositis should be considered as one of underlying diseases causing severe respiratory failure.