Popeye Sign in Hereditary Transthyretin Amyloidosis.

This case report describes a 63-year-old man who developed dysesthesia in his feet and was misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy.

Non-convulsive status epilepticus possibly induced by a rapid correction of severe hyperkalemia: a case report and literature review.

BACKGROUND: Patients with chronic kidney disease frequently develop neurological complications including confusion and altered consciousness. Non-convulsive status epilepticus, which is characterized by a change in behavior and/or mental process accompanied by epileptiform discharges on electroencephalogram in the absence of convulsive seizures, is one of the overlooked causes of altered consciousness. The incidence and precise pathophysiological mechanism of non-convulsive status epilepticus in patients with kidney disease, and especially in patients with electrolyte disturbances, remains unknown. We recently treated an older patient with chronic kidney disease and severe hyperkalemia in whom non-convulsive status epilepticus developed following a correction of severe hyperkalemia. CASE PRESENTATION: An 82-year-old male was admitted to our hospital at midnight because of weakness of all four limbs (Day 1). He underwent urgent hemodialysis for severe hyperkalemia (9.84 mEq/L) and his serum potassium concentration decreased to 4.97 mEq/L. He regained full consciousness and his limb weakness improved on the morning of Day 2, but he became confused in the evening. Electroencephalogram revealed repeated low-voltage ictal discharges in the right occipital region and a diagnosis of non-convulsive status epilepticus was made. Following medication with fosphenytoin and phenytoin, the patient became fully alert and orientated on Day 8. CONCLUSION: We speculate that a rapid correction of hyperkalemia was the possible cause of non-convulsive status epilepticus development. To our knowledge, this is the first report of non-convulsive status epilepticus from a potassium abnormality. We described a case of this condition in detail and summarized 78 previous case reports of non-convulsive status epilepticus with kidney disease or electrolyte disturbances.

Implications and limitations of magnetic resonance perfusion imaging with 1.5-Tesla pulsed arterial spin labeling in detecting ictal hyperperfusion during non-convulsive status epileptics.

Background: Recent our reports showed that 3-T pseudocontinuous arterial spin labeling (3-T pCASL) magnetic resonance perfusion imaging with dual post labeling delay (PLD) of 1.5 and 2.5 s clearly demonstrated the hemodynamics of ictal hyperperfusion associated with non-convulsive status epilepticus (NCSE). We aimed to examine the utility of 1.5-T pulsed arterial spin labeling (1.5-T PASL), which is more widely available for daily clinical use, for detecting ictal hyperperfusion. Methods: We retrospectively analyzed the findings of 1.5-T PASL with dual PLD of 1.5 s and 2.0 s in six patients and compared the findings with ictal electroencephalographic (EEG) findings. Results: In patients 1 and 2, we observed the repeated occurrence of ictal discharges (RID) on EEG. In patient 1, with PLDs of 1.5 s and 2.0 s, ictal ASL hyperperfusion was observed at the site that matched the RID localization. In patient 2, the RID amplitude was extremely low, with no ictal ASL hyperperfusion. In patient 3 with lateralized periodic discharges (LPD), we observed ictal ASL hyperperfusion at the site of maximal LPD amplitude, which was apparent at a PLD of 2.0 s but not 1.5 sec. Among three patients with rhythmic delta activity (RDA) of frequencies <2.5 Hz (Patients 4-6), we observed obvious and slight increases in ASL signals in patients 4 and 5 with NCSE, respectively. However, there was no apparent change in ASL signals in patient 6 with possible NCSE. Conclusion: The detection of ictal hyperperfusion on 1.5-T PASL might depend on the electrophysiological intensity of the epileptic ictus, which seemed to be more prominent on 1.5-T PASL than on 3-T pCASL. The 1.5-T PASL with dual PLDs showed the hemodynamics of ictal hyperperfusion in patients with RID and LPD. However, it may not be visualized in patients with extremely low amplitude RID or RDA (frequencies <2.5 Hz).

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.

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.

[Ischemic stroke associated with LH-RH analogue (leuprorelin) administration in a young woman].

A 33-year-old woman suffering from endometriosis was admitted to our hospital because of acute-onset diplopia and gait instability. She had a low-grade fever and cough 7 days before admission. On admission, she had left eye deviation downward on the inside, bilateral upper gaze impairment, and left Barrés sign with myoclonic-like movement of the left hand. MRI with diffusion-weighted images (DWI) showed high signal intensity in the right thalamus. The lesion had low signal intensity on an apparent diffusion coefficient (ADC) map, indicating cytotoxic edema. Although the presence of antecedent infection and moderate inflammatory changes shown by blood analysis prevented exclusion of inflammatory disorders such as cerebral angitis or acute disseminating encephalomyelitis, cerebral infarction was strongly suspected. The patient was treated with the free radical scavenger edaravone and high-dose dexamethasone; antiplatelet treatment was not used because of menstrual bleeding. Activation of coagulo-fibrinolytic system such as the elevated plasma level of D-dimer was present, and a luteinizing hormone-releasing hormone (LH-RH) analogue (leuprorelin) had been administered 4 days before hospitalization. This circumstance suggests the possibility that treatment with an LH-RH analogue may cause ischemic stroke in young women.

[A case of pseudo Tolosa-Hunt syndrome with bacterial infection and literature review].

We report a case with recurrent orbital pain and unilateral cranial nerve paresis mimicking Tolosa-Hunt syndrome. However, these features were most likely caused by bacterial infection because of beneficial response to antibiotics therapy. A 32-year-old man developed severe right orbital pain and diplopia. Neurological examination revealed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction. MR imaging revealed thickness of right cavernous sinus region with marked gadolinium enhancement. Cerebrospinal fluid (CSF) examination was initially normal. Treatment with steroid showed marked improvement. However soon after tapering of steroid dosage, his symptoms recurred and deteriorated. He was referred to our hospital because of second opinion. Neurological examination still showed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction with orbital and retro-orbital pain. Re-examination of CSF showed pleocytosis with neutrocytes dominancy and elevated protein concentration. Intravenous treatment with penicillin was initiated with marked improvement. There have been reported cases with bacterial infection resulting pseudo Tolosa-Hunt syndrome, which have good response to antibiotics treatment with excellent prognosis. It is speculated that bacterial infection might cause clinical features mimicking Tolosa-Hunt syndrome in our case.

[Garcin syndrome in a patient with rhinocerebral mucormycosis].

Garcin syndrome is characterized by an unilateral cranial nerves involvement without sensory or motor long-tract disturbances. It is usually caused by tumor infiltrating in the skull base with osteolytic changes on radiological study. We report a case of 64-year-old man with history of alcohol overintake, who admitted local hospital, because of right periorbital edema and facial swelling. He noted right ptosis 2 weeks prior to admission. Neurological examination revealed right multiple cranial nerves involvement including II, III, IV, V, and VI cranial nerves. MR imaging of the brain showed marked paranasal sinusitis and abnormal infiltration of right orbital fat. Orbital apex syndrome related to paranasal sinusitis was diagnosed, and antibiotics was administered. But a few days after admission, he developed a right VII, IX, X cranial nerve palsy. He was transferred to our hospital because of acute development of left hemiparesis and deteriorated consciousness. MR imaging of the brain showed right internal carotid artery (ICA) occlusion, and infarction in right middle cerebral artery (MCA)'s territory. The diagnostic biopsy of the paranasal sinus showed mucorales hyphae, indicating that the pathological diagnosis was mucormycosis. Despite of antibiotic therapy included of amphotericin-B administration and strict control of diabetic mellitus, his sinusitis was gradually spread. His condition progressively deteriorated, and finally died of sepsis. Post-mortem examination revealed a widespread mucor infiltration in the dura mater without skull bone invasion. This case presented with unilateral multiple cranial nerve involvements (Garcin syndrome) followed by left hemiparesis associated with rhinocerebral mucormycosis. It is suggested that mucormycosis should be considered in case of Garcin syndrome without osteolysis in the skull base.

[Thyrotoxic encephalopathy showing reversible diffusion-weighted imaging abnormalities].

A 31-year-old man was transferred to our emergency room (ER) with acute onset of high-grade fever and consciousness disturbance. His consciousness at ER was severely disturbed with restlessness. No apparent focal neurological signs were seen. MRI with diffusion-weighted images (DWI) showed high signal intensities at the corpus callosum, left cerebellar hemisphere, left deep white matter and right middle cerebellar peduncle. These lesions were low signals in apparent diffusion coefficient(ADC) map, indicating cytotoxic edema. EEG showed enhanced fast waves seen in predominantly frontal regions. CSF examination was normal except elevated initial pressure of 210 mmH2O. He was treated with high dose dexamethasone and acyclovir. His consciousness and high-grade fever with systemic inflammatory responses were dramatically improved after these treatments. Subsequent data showed hyperthyroidism with anti-thyroid stimulating hormone receptor antibodies. This case was thought to be a thyrotoxic encephalopathy with beneficial response to corticosteroid therapy. Abnormalities seen in DWI and EEG were normalized ten days later.