Fujio Fujiki

Non-herpetic limbic encephalitis associated with relapsing polychondritis

Relapsing polychondritis is a generalised disorder characterised by recurrent inflammation of cartilaginous structures throughout the body, without recognised collagen disease or infectious disorders. Central nervous system involvement is rarely reported in this disorder. We describe the cases of two patients with relapsing polychondritis who presented with severe memory impairment and psychiatric features such as euphoria and hyperactive behaviours, leading to a diagnosis of non-herpetic limbic encephalitis. Limbic encephalitis is caused by the herpes simplex virus (HSV) or by heterogeneous non-herpetic disorders (non-herpetic viruses, Hashimoto’s encephalopathy, central nervous system lupus, gliomatosis cerebri, intravascular malignant lymphomatosis, and paraneoplastic conditions). Clinical characteristics include cognitive dysfunction, severe memory impairment, seizures, depression, anxiety, and hallucinations. Magnetic resonance imaging (MRI) studies reveal selective unilateral or bilateral involvement of the limbic system, particularly the medial temporal lobe regions. Our first patient was a 45 year old man referred to us because of subacute progressive mental confusion, euphoria, hyperactive behaviour, disorientation, and forgetfulness of recent episodes. He had a history of right sided conjunctivitis successfully treated with steroid and antibiotic ointment, shoulder stiffness, headache, low grade fever, and recent weight loss (6 kg). On admission, the patient was disoriented, with an inappropriately jocular affect, disjointed speech, confabulation, attention deficits, and memory impairment including anterograde and 1 year retrograde amnesia. His Mini Mental Status Examination (MMSE) score was 11 (of a possible 30). He had no …

Magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease.

Hirayama disease is a nonprogressive, asymmetric amyotrophy of the hands and forearms, possibly caused by compression of the lower cervical cord on neck flexion. The authors used phase-contrast MR angiography to study a patient with this disorder and observed abnormal spinal epidural venous dilation on neck flexion. In addition to mechanical compression of the lower cervical cord, venous congestion in the spinal canal may have a role in promoting anterior horn damage.

Hypertrophic cranial nerve roots in CIDP

A 61-year-old man presented with painful dysesthesia/dysarthria (3 months) and sensorimotor impairment (13 years). Chronic inflammatory demyelinating polyradiculoneuritis (CIDP) was confirmed 4 years earlier by …

Acute motor sensory axonal neuropathy associated with Henoch-Schönlein purpura

A 41-year-old man was admitted with progressive tetraparesis with hypoesthesia. He also presented with purpura in both legs. After admission, joint pain, gastrointestinal tract bleeding, and renal dysfunction developed. A nerve conduction study revealed reduced amplitude of the motor and sensory nerve action potential, with normal conduction velocity. A skin biopsy showed leukocytoclastic vasculitis, indicating Henoch-Schönlein purpura (HSP). After administration of corticosteroids, the symptom completely disappeared. The present case is the first report in Japan of HSP associated clinically and electrophysiologically with confirmed acute motor sensory axonal neuropathy. Common pathogenesis might have a role for development for two distinct disorders.

Aseptic meningitis as initial presentation of acute disseminated encephalomyelitis

BACKGROUND It is well known that upper respiratory infections or vaccinations are etiologic factors in the majority of acute disseminated encephalomyelitis (ADEM) cases. However, it is less well known that aseptic meningitis may be an initial manifestation of ADEM. OBJECTIVES To compare the clinical and laboratory findings between aseptic meningitis associated with ADEM (AM-ADEM) and isolated aseptic meningitis (AM-alone), and to determine possible predictive factors for progression to ADEM. METHODS Twenty-five adults initially diagnosed as having aseptic meningitis were included in the present study. Clinical features, CSF, and laboratory parameters were retrospectively analyzed and compared between those with AM-alone and those who went on to develop AM-ADEM. RESULTS Twenty patients were diagnosed as AM-alone, whereas five were AM-ADEM. Neurological features associated with ADEM including somnolence, diplopia, ataxia, paresis, and bladder disturbance developed 5-19 days after the first symptoms of aseptic meningitis. Sustained high fever >38 degrees C and hyponatremia <135 mEq/l were seen more frequently in cases with AM-ADEM compared with those with AM-alone. CONCLUSIONS Patients with an initial diagnosis of aseptic meningitis may develop ADEM during hospitalization. Sustained high fever and hyponatremia upon admission might be useful predictive factors for the subsequent development of ADEM in patients with aseptic meningitis.