A systematic evaluation of cognitive function is an integral part of the neurological examination. Behavioral measures could detect functional impairment that is too subtle to be detected by current neurological procedures. Neuropsychological examination should cover both general and specific cognitive processes. The level of general attention or emotional change may affect variety of cognitive processes. Impairment of a functional system due to a focal cerebral damage results in a specific cognitive dysfunction such as aphasia, apraxia, agnosia, etc. Simplified but systematic methods to assess basic neuropsychological functions are suitable for everyday clinical examination by neurologists. A relatively short form of the cognitive status examination was devised, which covers general attention, language, calculation, praxis, visuospatial ability, directional attention and memory. Not only quantitative but also qualitative assessment of patients' performance would give clinicians valuable clues for the neuronal bases of cognitive functions.

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Oral disintegrating tablets (hereafter, ODT) can be ingested without water. We conducted a videoscopic examination to determine whether they are also useful as internal agents for patients experiencing difficulty with eating and swallowing. Normal tablets and dummy preparations of ODT were orally administered to six patients with neurological diseases who were either diagnosed with or aware of difficulty in eating and swallowing, and observations were conducted using a videoscope. Two subjects were able to ingest both the normal tablet and the dummy preparation without any problem; two subjects were able to ingest the normal tablet without any problem but the dummy preparation remained in their pharynx; and two subjects had both the normal tablet and the dummy preparation remained in the pharynx. There was no feeling of residue in the four cases in which the dummy preparation remained in the pharynx. ODT is not necessarily easy to ingest for patients with neurological diseases who have difficulty eating and swallowing, and it was believed that repeated swallowing or alternate swallowing of a thick liquid is required for ingestion.

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A 58-year-old man was admitted to our hospital with fever, vomiting and disturbance of consciousness after common cold-like symptoms for 2 days. Physical examination showed high fever, moderate hypertension and tachycardia. There were no superficial lymph nodes swelling nor skin rashes. Cerebrospinal fluid (CSF) examination revealed increased protein level (467mg/dl) and pleocytosis (508cells/mm³), but no glucose was detected. CSF smear test detected the pneumococcus. Intravenous cefotaxime was administered along with intravenous immunoglobulins and steroid pulse therapy. However, DIC developed, so FOY therapy was started. With these treatments, level of consciousness gradually improved and he became able to eat. At 11th days after the onset, the patient suddenly developed left facial palsy and paresis of the left arm. Head T2-weighted magnetic resonance imaging demonstrated tumor-like hyperintensity signal lesions (28×16.6mm) with ring enhancements in the right frontal lobe. Acute disseminated encephalomyelitis (ADEM) was diagnosed based on MRI and CSF findings, and then additional corticosteroid pulse therapy was administered twice. Herpes simplex virus and herpes zoster virus DNA in the CSF were undetectable by PCR. After 6 days of treatment with corticosteroid pulse therapy, left facial palsy and paresis of the left arm gradually improved and MRI showed the disappearance of tumor-like hyperintense signals. Although ADEM usually develops as a complication after viral infection such as measles, rubella, mumps and herpes zoster, this case suggests that ADEM complication should be considered even after pneumococcal meningoencephalitis.

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A 32-year-old homosexual man was admitted because of acute headache, fever, and lymphoadenopathy. The neurological examination revealed nuchal rigidity and positive Kernig's sign. The cell count of cerebrospinal fluid (CSF) at the time of admission, however, was four per microliter and subsequently increased up to 31 per microliter in three days. The serum antibody for human immunodeficiency virus (HIV) was positive in ELISA and the cell number of CD4 positive population decreased to 280. The RT-PCR for HIV RNA was 7.6×10⁵ copies per milliliter, which gradually decreased, leading to the diagnosis of meningitis due to HIV itself. The Western blotting for HIV antibodies were positive for p24, p40 and p55, whereas that for gp 41 was negative in serum and CSF, suggesting that the meningitis occurred during the seroconversion in this patient. We surmise that aseptic meningitis during HIV primary infection usually results in mild CSF pleocytosis and sometimes leads to even normocytosis shown as in this patient, probably because cellular immunity is temporally suppressed in acute HIV infection.

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A 63-year-old man was admitted because of sudden transient consciousness disturbance and left-side hemiparesis 110 minutes after the onset. Typical symptoms of aortic dissection, such as chest pain, back pain, neck pain, laterality of blood pressure or hypotension were not found. Brain magnetic resonance imaging (MRI) showed multiple acute brain infarction of the right middle cerebral artery territory. Carotid duplex ultrasonography demonstrated a subintimal dissection with a false channel of the right common carotid artery (CCA) and the right internal carotid artery (ICA). Thoracoabdominal computed tomographic (CT) scan demonstrated the false lumen in ascending and descending thoracic aorta. Cervical CT scan showed a dissection with a false channel of the right CCA. Intravenous administration of recombinant tissue plasminogen activator (rt-PA) is a contraindicant therapy in patients of brain infarction with aortic dissection. Thus our patient showed thoracic aortic dissection with extension of the dissection toward the right internal carotid artery. And the patient complained of neither the pain in the chest, the back nor the neck. So we emphasize the necessity of carotid duplex ultrasonography examination before intravenous administration of rt-PA in the treatment of the cerebral infarction, regardless of having chest pain, back pain, neck pain or not.

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A 68-year-old man was admitted to our hospital in the mid-October of 2006 because of a one-month history of peculiar movements of the left hand, which had been preceded by one month by awkward motions in the left leg. Upon neurological examination, spontaneous involuntary movement of the left hand was found. His left hand with his index finger stretched moved toward his right side spontaneously. He could not control his left leg freely. Although he showed mild ataxia in his left hand, there was no weakness, no dystonia, and no apraxia. No sensory abnormality was detected except for mild deep-sensation impairment in his left foot. The results of magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI) were normal; however, single-photon emission computed tomography (SPECT) showed hypoperfusion in the right hemisphere. At the time of admission, although a clinical diagnosis could not be made, we considered that the involuntary movements of his left hand were consistent with alien hand sign (AHS). Two and a half months after its onset, with the development of rapidly progressive dementia and generalized myoclonus, AHS gradually disappeared. Three months after the AHS onset, MRI with DWI showed restricted diffusion within the cortex involving the cingulated gyrus and bilateral temporal lobes, which was more prominent on the right than on the left side. Four months after the AHS onset, 14-3-3 protein level of the cerebrospinal fluid was elevated, and EEG recordings showed diffuse slowing of basic activity with periodic complexes. The patient was clinically diagnosed as having CJD. The patient died of pneumonia four and a half months after the AHS onset. AHS has rarely been reported in patients with CJD. Our case illustrates the importance of considering CJD in the differential diagnosis, if the patient showed AHS, even with normal MRI findings.

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A 65-year-old woman presenting with multiple autoimmune disorders including incomplete CREST overlapping with aquaporin 4 (AQP4) antibody-positive recurrent myelitis was reported. She also clinically suffered from Sjögren syndrome and primary biliary cirrhosis (PBC). She had dysesthesia below C4 level, mild motor weakness and hyperreflexia without pathological reflexes on bilateral lower extremities. A T2-weighted MRI indicated multiple discontinuous spinal cord lesions at C1-5 and T7/8. A visual evoked potential study disclosed bilateral prolonged latency of P100. She clinically manifested not only incomplete CREST syndrome (facial teleangiectasia, sclerodactyly in bilateral fingers, and Raynaud's phenomenon), but also Sjögren (sicca syndrome) and PBC (jaundice). Immunoserological study showed that she was positive for anti-nuclear, anti-centromere, and anti-AQP4 (=NMO-IgG) antibodies. A combination therapy with corticosteroid and plasmapheresis was effective for all clinical symptoms. Therefore, this case stresses on the relevance of anti-AQP 4 antibody to the other overlapping autoimmune disorders, such as CREST syndrome, when recurrent myelitis is clinically diagnosed.

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A 35-year-old man suffered from myalgia and joint pain on walking for 5 months. Physical and neurological examinations revealed dermal sclerosis, skin swelling, redness of forearms, Raynaud's phenomenon, joint pain, myalgia and muscle weakness. Eosinophilia was not found and serum creatine kinase activity was normal, while aldolase was markedly elevated. Abnormal signals suggesting synovitis and myofasciitis were found on MRI images. Biopsy of the fascia of quadriceps femoris showed perivascular mononuclear cell infiltration. A muscle biopsy showed mononuclear cell infiltration mainly in the perimysium extending to the endmysium. Eosinophilic cells were not found. Perifascicular atrophy was observed. Corticosteroid therapy improved clinical symptoms and serum aldolase level. We diagnosed him as non-eosinophilic myofasciitis and synovitis with perifascicular atrophy. The serum aldolase activity is usuful for diagnosis and for monitoring the disease activity.

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A 57-year-old previously healthy woman visited our clinic complaining of frequent muscle cramps and progressive weakness in the hands and fingers for 3 years. On examination, cranial nerves were unremarkable. There were moderate weakness and mild muscle wasting with fasciculation in the left thumb flexor and interossei on both sides. Tendon reflexes were hypoactive. There were no pathologic reflexes or sensory deficit. The cerebrospinal fluid was unremarkable. Nerve conduction studies demonstrated conduction block in the right ulnar nerve. Compound muscle action potential in the left median nerve was low-normal. Distal motor latencies, motor and sensory nerve conduction velocities were normal in all nerves tested. A diagnosis of multifocal motor neuropathy was made. Two courses of intravenous immunoglobulin infusion gave no beneficial effects. The patient had IgM kappa monoclonal gammopathy of undetermined significance. Her serum IgM reacted with GM2, GM1, and GA1 but not with GD1a, GD1b, GD3, GalNAc-GD1a, GT1b, GQ1b, galactocerebroside, or sulfated glucuronyl paragloboside. IgM kappa paraprotein reacted exclusively with GM2. Only IgM lambda bound to GM1 and GA1, suggesting the possibility that another paraprotein, though undetectable by immunoelectrohoresis, had a reactivity with GM1 and GA1. This case showed previously unreported antigenic specificity of paraproteins in cases of MMN.

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A 23-year-old Japanese man with rheumatoid arthritis experienced attacks of the right hand weakness during the latest month. Megaloblastic anemia was diagnosed and he was admitted in the department of hematology. Seven days after admission, he developed mild consciousness disturbance and hemiparesis on the right side. Brain MRI revealed the cortical watershed infarction in the left cerebral hemisphere. The cause of the infarction was diagnosed spontaneous dissection of the left internal carotid artery. We detected 11 microembolic signals (MES) during 30 minutes' monitoring in the left middle cerebral artery using transcranial Doppler. MES decreased gradually with anticoagulant therapy. However, surgical therapy was performed to prevent progression of dissection or arterial rupture. MES detected on transcranial Doppler would be important to assess the risk of embolism and to discuss therapeutic strategy.

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