Age and duration related changes in muscle sympathetic nerve activity in Parkinson's disease.

OBJECTIVE: To clarify the characteristics of sympathetic vasomotor function in Parkinson's disease by sympathetic neurographic analysis. METHODS: Muscle sympathetic nerve activity (MSNA) was recorded using a microneurographic technique at rest and during head up tilt in 18 patients with idiopathic Parkinson's disease and 21 healthy controls. RESULTS: Heart rate and blood pressure at rest did not differ between index and control subjects. The increase in these variables and MSNA in response to tilting was slightly blunted in the Parkinson's group. Resting MSNA showed a negative correlation with age in patients with Parkinson's disease (p<0.05) and a positive correlation with age in controls (p<0.01). There was a negative correlation between duration of disease or disability levels and MSNA (p<0.01). CONCLUSIONS: Sympathetic vasomotor function may be related to age and disease duration in Parkinson's disease.

Ipsilateral thalamic MRI abnormality in an epilepsy patient.

In a 19-year-old patient with status epilepticus arising in the right parietal neocortex, unenhanced ictal MRI showed abnormalities mainly in the right cerebral cortex, contralateral cerebellum, and ipsilateral thalamus. The thalamus is considered a key site of functional abnormality in this patient.

[A case of Guillain-Barré syndrome after Campylobacter jejuni enterocolitis: anti-ganglioside antibody levels with or without Guillain-Barré syndrome].

A 38-year old man developed enterocolitis one day after he had ingested raw chicken. Nine days later, his grip strength weakened. Eleven days later, he was admitted to our hospital with weakness of four limbs, dysphagia and dysarthria. Serum anti-Campylobacter jejuni antibody and anti-ganglioside antibodies (GM1, GD1a, GD1b, GalNAc-GD1a) were positive, and motor action potentials were not evoked at all extremities. He was diagnosed as having Guillain-Barré syndrome. After receiving immune absorption therapy and plasma exchange therapy, the patient improved. Another person who had also consumed the same raw chicken developed colitis only. Five weeks later, the anti-GalNAc-GD1a-IgG antibody titers (O.D. 490 nm) of the patient and the other man who developed colitis were 0.324 and 0.118, respectively. It was suggested that the pathogenesis of Guillain-Barré syndrome after Campylobacter jejuni enterocolitis may be related to the type and titer of anti-ganglioside antibodies and also to the sensitivity of the individual.

A concise overview of recent breakthroughs in imaging of ALS.

Numerous attempts have been made to visualize the motor cortex and pyramidal tract lesions in patients with ALS using magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT) and positron emission tomography (PET). This paper briefly reviews the applicability of these imaging modalities in ALS.

Muscle sympathetic activity in cerebellar degeneration with or without mild autonomic dysfunction.

To evaluate autonomic function in sporadic cases of cerebellar degeneration (CD) with or without mild autonomic dysfunction, we measured muscle sympathetic nerve activity (MSNA) by microneurography in 12 CD patients and 18 healthy subjects. The burst incidence and mean amplitude of MSNA at rest was significantly decreased in CD patients (P < 0.01). There were no significant differences between patients with CD and controls in heart rate or blood pressure at rest. During head-up tilting, increases in blood pressure and mean amplitude of MSNA were significantly lower in CD patients. In conclusion, a decrease in muscle sympathetic outflow can be observed even in CD patients who are clinically excluded from multiple system atrophy (MSA). It might be difficult to distinguish other CD syndromes from MSA in recordings of muscle sympathetic activity.

An aggressive familial amyloidotic polyneuropathy caused by a new variant transthyretin Lys 54.

Histologic examination of sural nerve of a 32-year-old man with an aggressive polyneuropathy associated with autonomic failure demonstrated amyloid deposition, and familial amyloidotic polyneuropathy (FAP) was diagnosed. Immunohistochemical staining showed transthyretin (TTR) staining of the amyloid deposits in nerve. Sequencing revealed G to A transition in the codon 54 causing TTR Lys 54. This is a new variant TTR associated with aggressive FAP.

Prolonged sympathetic reflex latency on skin nerves in sporadic cerebellar degeneration.

BACKGROUND: Many physiological or pharmacological evaluations of autonomic function have been performed in patients with various types of cerebellar degeneration (CD). Few studies have been done, however, using neurographic recordings in patients with CD, especially of sudomotor or vasoconstrictor nerves. OBJECTIVE: To confirm the clinical importance of sympathetic reflex latencies on the skin nerves of patients with various types of CD. DESIGN AND SETTING: Case-comparison study at an academic center. PARTICIPANTS: We studied 12 patients who had sporadic CD (cerebellar cortical atrophy, olivopontocerebellar atrophy, and Shy-Drager syndrome) and 15 healthy volunteers as controls. METHODS: Skin sympathetic nerve activity (SSNA), sympathetic skin response, and skin vasomotor reflex were simultaneously recorded using randomly administered electrical stimuli. RESULTS: In controls, SSNA reflex latency ranged from 640 to 864 milliseconds. Patients with cerebellar cortical atrophy exhibited slight but significant (P<.01) prolongation of reflex latency to the onset of reflex bursts. In patients with olivopontocerebellar atrophy, latency to the onset and the peak of reflex bursts was significantly prolonged (P<.001). Patients with Shy-Drager syndrome had no SSNA, sympathetic skin response, or skin vasomotor reflex, even with supramaximal electrical stimuli. CONCLUSIONS: Prolonged reflex latency or the absence of reflex bursts on SSNA can be observed in patients with CD with various forms of autonomic dysfunction. The measurement of SSNA reflex latency may be a useful method of evaluating sympathetic function, including the central pathway.

[A case of neuroleptic malignant syndrome associated with reversible leukoencephalopathy].

We report a case of neuroleptic malignant syndrome associated with reversible leukoencephalopathy. The patient was a 60-year-old woman. Soon after ingesting an antipsychotic drug, the patient developed neuroleptic symptoms. After hydration and dantrolene sodium were administered, muscular rigidity gradually improved and serum levels of CK became normal. On the 7th hospital day, however, she fell into coma and showed tetraplegia. Although brain CT was normal on admission, diffuse low density areas were observed on the parieto-occipital cerebral white matter. The same lesions were observed on T2-weighted MRI. On the 20th hospital day, after giving her glycerol and adrenocorticosteroid, not only abnormal neurological findings but also abnormal CT and MRI findings disappeared. There were only two case reports of leukoencephalopathy with malignant syndrome in the literature. This is a rare case of reversible leukoencephalopathy with neuroleptic malignant syndrome due to the antipsychotic drug.

Sleep apnoea in well-controlled myasthenia gravis and the effect of thymectomy.

We conducted overnight polysomnographic sleep studies of 16 patients (5 men and 11 women) with clinically well-controlled myasthenia gravis (MG). The subtypes of MG were IIA (3 patients), IIB (11 patients), IV (1 patient) and V (1 patient). Twelve patients were found on polysomnography to have obstructive and/or central types of the sleep apnoeas (SA). Their mean age was 42.4, SD 16.4 years, and the mean duration of MG was 7.4, SD 6.96 years. SA was not detected in 4 patients whose mean age was 30.8, SD 10.71 years and who had manifested MG for a mean duration of only 0.9, SD 0.65 years. Thus, patients with a longer duration of MG tended to have more SA. In 9 of the 12 SA patients, polysomnographic studies were repeated following thymectomy. SA had resolved in 6 patients, but persisted in 3. These findings suggest that SA is a possible clinical manifestiation of MG and that nocturnal dysfunction of both peripheral and central colinergic systems may be involved.

Endovascular treatment of excessive spasticity from spinal cord injury. A case report.

SUMMARY: We report a new treatment for excessive spasticity using an endovascular technique. A 52-year-old woman with intractable spasticity of the hip joints and abdominal muscles was treated with an injection of 70% alcohol into Adamkiewicz's artery, which destroyed the spinal cord fed by Adamkiewicz's artery and cut the reflex arcs of excessive spasticity. Immediately after the treatment a significant change was noticed in muscle tonicity of the lower extremities, and intractable spasticity disappeared. Skin necrosis and muscle damage occurred because of the alcohol that leaked into the muscle branch of the subcostal artery. However, these complications could be treated by simple necrotomy. No recurrence was observed during the follow-up period. We believe this endovascular treatment will become a new strategy for excessive spasticity arising from spinal cord injury.

Decreased sympathetic outflow to muscles in patients with cervical spondylosis.

To clarify the pathogenesis of neurologic symptoms of a vascular nature that may accompany cervical spondylosis (CS), we measured the sympathetic outflow to muscles (muscle sympathetic nerve activity: MSNA) in 8 ambulatory patients with CS and 10 healthy volunteers (controls). The burst rate and incidence of MSNA at rest were significantly lower in patients with CS than in controls (P < 0.01). There were no differences between the 2 groups in either resting heart rate or blood pressure. During a head-up tilting, the changes in MSNA were slightly greater in CS patients than controls due to reduced baseline levels of MSNA in CS patients. The burst incidence of MSNA in CS was significantly negatively correlated with the severity of pyramidal tract symptoms (P < 0.05). The decreased MSNA at rest in CS patients may be due to a spinal cord compression resulting from posterior spondylotic changes in the cervical spine.

[Idiopathic hypertrophic cranial pachymeningitis associated with Horner's syndrome. A case report].

A 64-year-old man was admitted to our hospital for recurrence of visual disturbance and double vision experienced 1 year previously. He was alert and oriented. Bilateral light perception in a vision test, the third to the sixth cranial nerve palsies on the left side, and neck stiffness were observed. The patient exhibited left blepharoptosis, anisocoria and a left miotic pupil. After a pupillary drug test, a diagnosis of Horner's syndrome was made. Laboratory tests revealed hypoalbuminemia, elevated erythrocyte sedimentation rate, positive rheumatoid factor, and elevated p-ANCA. Examination of the cerebrospinal fluid showed increased initial pressure and pleocytosis, but its bacterial and fungal cultures were negative. Cranial magnetic resonance imaging after intravenous administration of gadolinium revealed mild brain edema and marked hypertrophy of the left tentorium cerebelli, bilateral frontal dura and falx. Marked hypertrophy of the dura accompanied by cellular infiltration was observed in biopsied dura specimens. The patient was given a diagnosis as idiopathic hypertrophic cranial pachymeningitis (IHCP). His ocular symptoms gradually improved with methylprednisolone pulse therapy and oral prednisolone. This is the first reported case of IHCP associated with Horner's syndrome. Previous anatomical findings of the cavernous sinus suggest that Horner syndrome in this patient might be due to a mechanism other than constrictive compression of the cranial nerves by the hypertrophic dura mater.

Unilateral tongue atrophy due to an enlarged emissary vein in the hypoglossal canal.

A 16-year-old girl presented to our clinic with right-sided tongue atrophy and fasciculations of 1-year duration. Enlargement of the outer opening of the hypoglossal canal was reveal by conventional and computed tomography of the skull. Magnetic resonance imaging disclosed an enlarged venous system extending from the jugular vein to the internal jugular vein on the right, with low signal density suggestive of a flow void. A right-sided occipital craniotomy was performed. When the hypoglossal canal was opened, an enlarged emissary vein compressing the hypoglossal nerve was identified. This is the first reported case of unilateral tongue atrophy and an enlarged hypoglossal canal due to an enlarged emissary vein.

Autopsy findings in Down's syndrome with cerebrovascular disorder.

We describe the vascular pathologic findings in a 20-year-old Japanese man with Down's syndrome (DS) who developed stroke-like symptoms and died of respiratory failure attributed to a cerebral infarction. The latter was verified at autopsy, which also revealed vascular abnormalities resembling those of moyamoya disease. There were occlusions or stenosis in the bilateral internal carotid arteries, middle cerebral arteries, left posterior communicating artery, and basilar artery due to fibrous hypertrophy of the arterial intimal walls. The tunica media was highly atrophic and the internal elastic lamina was tortuous and duplicate. However, unlike moyamoya disease, main trunks of the cerebral arteries were of normal size, and there was marked stenosis of the basilar artery. These cerebrovascular abnormalities may have been the result of an immune deficiency, thrombosis, maldevelopment of the skull and brain, or may have been linked to moyamoya disease. Further study of similar cases is necessary to clarify the association between DS and moyamoya disease, as well as the pathogenesis of that disease.

Decreased signal intensity of cerebral cortex on T2-weighted MR images.

To define the frequency of decreased signal intensity (DSI) in cerebral cortex on T2-weighted images relative to aging and to the incidence of identifying white matter pathology, T2-weighted MR brain images of 906 patients consecutively examined between July 1989 and June 1991 were reviewed. MR images of cerebral cortex were divided into five areas: frontal lobe (F), pre- and postcentral gyri (C), parietal lobe (P), occipital lobe (O) and temporal lobe (T). Each area was separately and independently evaluated for the presence or absence of DSI. The frequency of DSI in each area was plotted against patients' ages. The severity of leukoaraiosis was evaluated in relation with DSI in each cortical area. Patients with DSI in area F numbered 61 (5.7%), in area C 236 (26.0%), in area P 174 (15.9%), in area O 428 (47.2%), and in area T 10 (1.1%). The number of patients with DSI in each area increased with age. The greater the severity of leukoaraiosis, the greater the chance of finding DSI of the cerebral cortex. The incidence of DSI in the cerebral cortex increased with the patient's age as well as with the severity of co-existent white matter pathology.