Hypertrophic pachymeningitis (HP) is thought to have an autoimmune etiology but its precise cause and treatment remains to be elucidated. Here, we report the clinical details and therapeutic responses of 3 patients with HP and reviewed 66 previously reported cases in the literature. Among these patients, headache was the most frequent complaint. Cranial nerve involvement was also frequently observed, with the optic nerve being the most frequently impaired followed by the oculomotor, trochlear, and abducens nerves in frequency. Elevated C-reactive protein levels and erythrocyte sedimentation rates were found in approximately 97% of the patients. Steroids were the most commonly prescribed therapy, but no definite protocols for the standard dose and duration in HP have been proposed thus far. The average initial dose of prednisolone (PSL) was 42.7mg/day, and the average maintenance dose was 12.4mg/day in the chronic stage. Recurrence occurred in many patients when the dose of PSL was reduced to under 20mg/day. Therefore, steroids should be tapered extremely slowly.

View full abstract

We report 13 patients presenting with predominantly sensory strokes due to brainstem infarction, without any other brainstem symptoms such as hemiparesis, dysarthria or vertigo. All of them had lacunar infarctions localized at the medial lemniscus and/or spinothalamic tract, at the pontine (12 patients) or midbrain (1 patient) tegmentum. The presenting symptom was dysesthesia with a variety of distributions for all cases, and a thalamic-pain-like unpleasant dysesthesia persisted in 4 patients. The lesion on brain MRI was usually very small, and was sometimes overlooked by radiological evaluation, which led to a long delay in the correct diagnosis of a stroke in two cases. Median nerve somatosensory evoked potentials showed a depressed N20 amplitude or a loss of the P15 potential unilaterally with preserved P13/14 potential in 7 out of 10 cases examined, and was useful in localizing the lesion intracranially. During the 4-year study period, 10 patients with brainstem infarctions were admitted to our department as acute sensory stroke cases (2.1% of all acute strokes), whereas 11 patients with thalamic infarctions (2.3%) were admitted due to similar symptoms. Cases with brainstem infarctions had sensory symptoms localized below the neck more frequently (5/10) than cases with thalamic infarctions (1/11), thus would be more likely to be confused with cervical or peripheral nerve disorders. The relative frequency of brainstem infarction as compared to thalamic infarction was higher than that in previous reports, implying that some cases with brainstem infarction might have been overlooked due to difficulty in obtaining the correct diagnosis. One should always keep this syndrome in mind when assessing patients with acute-onset sensory symptoms.

View full abstract

A 45-year-old female was positive for anti-aquaporin-4 antibody with disturbance of consciousness, respiratory failure, and ophthalmoplegia associated with extensive brain stem involvement with intractable hiccup and nausea as an initial manifestation. Her level of consciousness and state of respiration worsened approximately one month later. There was no abnormality in the cerebrospinal fluid examination. A lesion was found in the medullary tegmentum on brain MRI. The patient received steroid pulse therapy and her level of consciousness improved the next day. However, her state of respiration worsened, and she had extensively clinical involvement of the brain stem. Her symptoms gradually improved with intravenous administration of prednisolone and intravenous immunoglobulin therapy (IVIg). The patient had almost completely recovered, but she relapsed with cervical myelitis extending over 3 vertebral segments approximately 10 months later. She underwent steroid pulse therapy, oral prednisolone, and IVIg again and improved.

View full abstract

A 22-year-old man with a previous uveitis episode was admitted to our hospital because of persistent hiccup. On admission, he presented right-upper quadrantanopia, mydriasis and lack of the light reflex in the left eye, left-sided hemiplegia, and bilateral pathologic hyperreflexia. The MR fluid attenuated inversion recovery images showed left side dominant, high intensity lesions on the brainstem and the diencephalon. The HLA-B51 was positive. The CSF IL-6 was extremely elevated (998pg/ml: reference value <=6.0pg/ml). Based on these, we concluded he had the neuro-Behçet's disease and treated him by high dose intravenous corticosteroids. This treatment improved his symptoms and MRI lesions, and decreased the CSF IL-6 levels initially. On 13th day after the first his discharge, however, dysarthria appeared and the CSF IL-6 levels elevated again. In addition to the high dose intravenous corticosteroids therapy for acute attack, 15mg/week of methotrexate was started to prevent the recurrence. Even with this prevention, meningitis related to neuro-Behçet's disease occurred within six weeks. We administered 5mg/kg of infliximab intravenously at 0, 2, 6, and 14 weeks. After the infliximab treatment, his symptoms improved and the IL-6 levels decreased, and no recurrence has occurred. This case supports that infliximab, anti-TNF-α agent, is a good candidate for neuro-Behçet's disease treatment when it is resistant to conventional immunosuppressive agents such as corticosteroids or methotrexate.

View full abstract

We report a 59-year-old immunocompetent man presenting with slowly progressive gait unsteadiness, dysarthria, and clumsiness in writing over 6 months. There were bilateral pyramidal signs, pseudobulbar palsy, and attention deficits. Cerebrospinal fluid examination showed mild mononuclear pleocytosis, and magnetic resonance imaging revealed pachymeningeal pattern of contrast enhancement beneath the calvarium and the posterior cranial fossa. Interferon-gamma release assay in whole blood after stimulation by specific tuberculosis antigens was positive and repeat polymerase chain reaction assay detected Mycobacterium tuberculosis genome in the cerebrospinal fluid. After combination therapy with anti-tuberculous agents and corticosteroids, the patient's pachymeningitis regressed. Tuberculous cranial pachymeningitis may present with chronic diffuse brain dysfunction without headache, fever, or cranial nerve dysfunction.

View full abstract

We visualized ponto-cerebellar tracts projecting through the middle cerebellar peduncle of 3 patients with early stage multiple system atrophy (MSA), 3 patients with advanced stage MSA, and a healthy control using diffusion tensor imaging (DTI) on 1.5T magnetic resonance imaging (MRI). We also examined whether the location of the degenerated ponto-cerebellar tracts coincided with that of the transverse part of the so-called "hot cross bun sign (HCB)" on MRI. DTI successfully demonstrated the degeneration of ponto-cerebellar tracts in MSA patients. The tracts in advanced stage MSA patients appeared more sparse than those in patients of the early stage. High apparent diffusion coefficient (ADC) values and low fractional anisotropy (FA) values also indicated the degeneration of the ponto-cerebellar tracts in MSA patients. The tracts in the ventral pons were more sparse than those in the central pons. The location of the degenerated ponto-cerebellar tracts of the central pons appeared to coincide with that of the transverse part of HCB. Visualization of degenerated ponto-cerebellar tracts that cross the ventral pons using DTI might be useful for the early diagnosis.

View full abstract

We report a Japanese patient with short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUCNT) responsive to gabapentin. A 29-year-old man presented with sudden-onset intermittent left-sided orbital headache, which was not accompanied by lacrimation and conjunctival injection. We diagnosed trigeminal neuralgia at first and administered carbamazepine and loxoprofen. However, these medications were entirely ineffective at all and 6 days later, autonomic symptoms including conjunctival injection and tearing appeared. Diagnosis of SUNCT was made and gabapentin was started at up to 800mg per day. Soon after, the headache and autonomic symptoms disappeared. Gabapantin at 800mg per day was continued for 3 months and then reduced to 400mg per day. Soon he had only a slight headache without tearing and conjunctival injection. He has continued to take gabapenin at 400mg per day until now. This case indicated that headache and autonomic symptoms in SUNCT did not always emerge simultaneously, but they sometimes appear with time lag. Furthermore, the long-term clinical course and therapeutic outcome in SUNCT remain unknown. A therapeutic strategy and optimal dosage of medications including gabapentin should be established for the treatment of SUNCT.

View full abstract

We report the case of disseminated metastatic tumor at dorsal surface of medulla oblongata presenting intractable hiccups. A 73-yaer-old man has a history of for metastatic lung tumor of the left tempral lobe. Although 3 surgeries and 4 radiotherapies were performed in the last 8 years, residual tumor grew slowly. He presented with intractable hiccups. His hiccups continued for 30 minutes, sometimes for 3 hours with obstruction of eating. Contrast-enhanced Magnetic resonance (MR) imaging demonstrated the dissemination of metastatic lung tumor at dorsal surface of medulla oblongata and ventral surface of midbrain. Some literatures reported the patients with intractable hiccups caused by dorsal medullary lesions. Therefore, we thought that the small disseminated tumor at dorsal surface of medulla oblongata caused the hiccups. Evaluation of dorsal medullay area by MR imaging is important to reveal the cause of intractable hiccups.

View full abstract

A 32-year-old woman, who had developed head tremor and paresthesia of the right upper limb for several months, was admitted to our hospital. The diagnosis of multiple sclerosis was made because the serial MRI showed multiple lesions in both the cerebral white matter and the cervical cord. Oligoclonal IgG band was positive. Her symptoms were improved by intravenous methylprednisolone and an antiepileptic drug (MEPM 1g/day and CZP 1mg/day). The head tremor was the so-called "yes-yes" type which shakes back and forth. Although this type of tremor has been considered to be developed by the lesions in the cerebellum, our patient seemed to develop the tremor by cervical cord lesion. Further investigation is needed to confirm the association of the head tremor and the cervical lesions in MS.

View full abstract