Localized hypertrophic mononeuropathy involving the femoral nerve.

The authors report a case of localized hypertrophic mononeuropathy involving the femoral nerve in a 20-year-old woman referred because of progressive weakness and atrophy of the left thigh. MRI showed an enlarged femoral nerve and biopsies of fascicles displayed a concentric pattern of cells resembling an onion bulb. These cells were positive for epithelial membrane antigen immunostaining and had an incomplete basal lamina.

Pathologic findings of silent, small hyperintense foci in the basal ganglia and thalamus on MRI.

We analyzed the pathologic features of silent, small hyperintense foci in the basal ganglia and thalamus on MRI T2-weighted images (T2WI). Eight foci were histologically lacunar infarcts (LI), and 21 were dilated perivascular spaces (DPS). The foci with smooth margins were mostly DPS, whereas those with irregular margins were mostly LI (p = 0.018). Most putaminal foci were DPS, whereas most thalamic foci were LI (p = 0.001). The mean areas of LI and DPS overlapped below 19.6 mm2. The shape and site of hyperintense foci on T2WI are important for differentiating LI from DPS.

Paramyotonia congenita without cold paralysis: a case report.

A 27-year-old-woman with paramyotonia congenita was reported. She began to suffer from myotonia since infancy. Myotonia was aggravated by cold, but with intense cooling myotonia did not change to flaccid paralysis. Four generations of her family showed the same symptoms that suggested autosomal dominant inheritance. Neurological examinations revealed no impairments of mental function, cranial nerves and sensory system. Muscular atrophy or hypertrophy was not observed. Percussion myotonia of the tongue and thenar muscles could be elicited at room temperature. Myotonia was aggravated by cold. An oral intake of potassium chloride (7 grammes) did not provoke any muscle weakness or flaccid paralysis. Routine laboratory data and findings of head CT scan, cerebrospinal fluid and electroencephalogram were normal. The nosological distinction between paramyotonia congenita and hyperkalemic periodic paralysis has been debated since 1956. Paralysis induced by cold is thought to be a feature of paramyotonia congenita, thus raising a possible relationships to hyperkalemic periodic paralysis. In our case cold paralysis never occurred spontaneously and could not be provoked by immersion in ice water or by potassium loading. This finding confirms the existence of paramyotonia congenita without cold paralysis and may provide a nosological distinction between paramyotonia congenita and hyperkalemic periodic paralysis.

Spastic paraplegia with amyotrophy of the legs: a rare case of motor and sensory neuropathy.

A 36-year-old man who suffers from gait disturbance is reported. He noticed deformity of his feet at the age of 15. When he was 32 years old, he complained of heaviness in his lower extremities. Since then his legs have been always stiff. He had no previous illness or familial neuromuscular diseases. Neurological examination revealed no impairment of mental function or cranial nerves. Marked weakness and wasting of the feet were noted. The legs showed an inverted champagne bottle shape and pes cavus deformity was evident. Deep tendon reflexes were normal in the arms but abnormally brisk in the legs. Bilateral Babinski sign and ankle clonus were elicited. The patient tended to walk on his toes and the legs scissored. The motor nerve velocities were less than normal. Sural nerve biopsy showed reduced myelinated fiber density and increased endoneurial connective tissue. Electron microscopy showed axonal swellings filled with neurofilaments. Distal wasting and weakness involving the legs more than the arms resembled that of Charcot-Marie-Tooth disease. According to the classification by Dyck, this disorder could be referred to as hereditary motor and sensory neuropathy type V. Spastic paraplegia with amyotrophy is rare, but should be identified as a distinct disorder. Recognition of this disorder would imply the clinical and genetic heterogeneity of Charcot-Marie-Tooth disease.

Rapid changes in pial arterial diameter and cerebral blood flow caused by ipsilateral carotid artery occlusion in rats.

We investigated rapid changes in pial arterial diameter and in cerebral blood flow (CBF) caused by transient ipsilateral common carotid artery occlusion (CCA-O) in anesthetized rats in order to elucidate how the cerebral circulation reacts to acute stem artery occlusion. In separate groups of rats, pial arterial diameter was recorded through a closed cranial window and CBF was recorded by laser-Doppler flowmetry. CCA-O was performed for 5 minutes under normotension and normocapnia (control) and under graded hypotension, hypercapnia and hypocapnia. In the control condition, pial arterial diameter increased rapidly, triggered by CCA-O. It took 12 +/- 3 s to reach the maximum of 204 +/- 42% of the value before CCA-O, and 60 +/- 24 s to become stable at 131 +/- 11%. CBF decreased rapidly to 66 +/- 11%, then increased reactively to 135 +/- 9%, and again decreased to 91 +/- 3%. The reactive increase in CBF caused by CCA-O decreased in parallel with the degree of hypotension, and also became barely detectable under hypercapnia. Our data suggest that active vascular dilation in the territory of the occluded artery is important for inducing collateral circulation.

Congenital hemophagocytic reticulosis.

A fatal case of an apparently congenital form of hemophagocytic reticulosis is reported. The onset was manifested by hyperbilirubinemia and hepatosplenomegaly which were present at birth and persisted throughout life. Fever, anemia and pancytopenia developed at 1 month of age and became progressively worse. A splenectomy was performed at the age of 3 months, but the child died one day later with disseminated intravascular coagulation and pulmonary hemorrhage. The literature is reviewed with regard to the relationship of this case to (familial) hemophagocytic reticulosis and malignant histiocytosis (histiocytic medullary reticulosis). It is suggested that congenital hemophagocytic reticulosis, as described here, (familial) hemophagocytic reticulosis in infants, and malignant histiocytosis in adults all represent the same basic disorder with different ages of onset and clinicopathologic manifestations.

Detrimental effects of exogenous glutamate on spinal cord neurons during brief ischemia in vivo.

BACKGROUND: Paraplegia remains a serious complication of thoracoabdominal aortic operations. However, despite growing in vitro evidence, it has been difficult to demonstrate glutamate neurotoxicity in vivo because of the reuptake activity that occurs. We hypothesized that glutamate can be toxic to the spinal cord under metabolic stress. METHODS: Infrarenal aortic isolation was performed in New Zealand white rabbits. Group A animals (n = 7) then received a segmental infusion of glutamate (50 mmol/L) for 5 minutes. Group B animals (n = 7) received saline as a negative control. Group C animals (n = 6) were pretreated with a segmental infusion of 2,3-dihydroxy-6-nitro-7-sulfamoyl-benzo(f)-quinoxaline (4 mg/kg), a competitive alpha-amino-3-hydroxy-5-methylisoazole-4-propionic acid/kainate antagonist, followed by the segmental infusion of glutamate (30 mmol/L) for 4 minutes. Group D animals (n = 6) received the vehicle agents only, followed by the same glutamate infusion (30 mmol/L) as in group C as a control for group C. Neurologic status was assessed at 12, 24, and 48 hours after operation and scored using the Tarlov system. RESULTS: Group A animals exhibited paraplegia or paraparesis with marked neuronal necrosis. Group B animals recovered fully. Group C animals had better neurologic function than group D animals (p = 0.0039). CONCLUSIONS: Exogenous glutamate can have detrimental effects on spinal cord neurons during a brief period of ischemia. This model may be useful for the purpose of assaying a glutamate receptor antagonist in vivo.

Occurrence and distribution of substance P receptors in the cerebral blood vessels of the rat.

The distribution of immunoreactivity to the receptor for substance P was examined in the cerebral blood vessels of the rat. Substance P immunoreactivity has been demonstrated in the nerve fibers of the cerebral blood vessels. Recently, the production of substance P receptor specific antibody has enabled the detection of localization of the substance P receptor in the central nervous system. In this study, we examined the existence of nerve fibers with substance P receptor immunoreactivity in the cerebral blood vessels and the cranial ganglia innervating the cerebral blood vessels. Sprague-Dawley rats were perfused with fixative and the pial arteries and the cranial ganglia known to innervate the cerebral blood vessels, i.e., trigeminal, sphenopalatine, internal carotid, otic and superior cervical ganglia, were dissected. All specimens were incubated with anti-substance P receptor IgG, then stained by the avidin-biotin-peroxidase complex method. Numerous nerve fibers with varicosities forming plexuses, with substance P receptor immunoreactivity were observed on the walls of the major extracerebral arteries forming the circle of Willis and its branches. Substance P receptor immunoreactivity was also detected in the endothelium of the cerebral arteries. Substance P receptor immunoreactivity was positive in many neurons of the sphenopalatine ganglion, otic ganglion, trigeminal ganglion, superior cervical ganglion and internal carotid ganglion. The present study demonstrated the existence of nerve fibers with substance P receptor immunoreactivity in the cerebral blood vessels and the cranial ganglia that innervate the cerebral blood vessels. These findings are important in understanding the responsiveness of the cerebral blood vessels to substance P.

Creutzfeldt-Jakob disease: a case with extensive white matter degeneration and optic atrophy.

A 52-year-old woman is described, whose clinical features were typical of Creutzfeldt-Jakob disease except for the presence of optic atrophy. Serial CT scans showed rapid development of brain atrophy early in the course. Postmortem examination revealed extensive degeneration of the cerebral and cerebellar white matter and of the optic nerves in addition to the classic findings of Creutzfeldt-Jakob disease. It is suggested that both the grey and white matter may undergo a severe destructive process early in the course of the disease, and the possibility is discussed that the white matter involvement is not a result of neuronal loss.

Controlled ultraviolet irradiation generates endothelial damage without affecting the nerve terminals of the cerebral artery in cats.

The vesicles of adventitial autonomic nerve terminals were examined quantitatively under an electron microscope in controlled ultraviolet ray (UV)-irradiated cerebral vessels. Five cats whose basilar arteries were irradiated with UV (UV group) and 5 cats whose basilar arteries were irradiated with visible rays (control group) were compared. Endothelial vacuolation was observed only in the UV group. There was no statistically significant difference in the diameters of the dense-cored vesicles, related to noradrenaline, and clear vesicles, related to acetylcholine, between the two groups. It is concluded that controlled UV irradiation which generates endothelial damage does not affect the vascular adventitia ultrastructurally.

Cerebrovascular NADPH diaphorase-containing nerve fibers in the rat.

Recently, neuronal nicotinamide adenine dinucleotide phosphate (NADPH)-diaphorase has been elucidated to be the nitric oxide synthase (NOS) per se. In order to examine the existence and distribution of cerebrovascular nerve fibers containing these substances, NADPH-diaphorase histochemistry was applied to the cerebral blood vessels and the cranial ganglia known to innervate the cerebral vessels in the rat. Numerous nerve fibers with varicosities forming plexuses were observed in the circle of Willis and its branches. In addition, thick nerve bundles were seen to run along the wall of the internal ethmoidal artery. NADPH-diaphorase reaction was prominent in neurons of the sphenopalatine, otic and internal carotid ganglia. This study demonstrated, for the first time, the NADPH-diaphorase-containing nerve fibers in the cerebral vessels and ganglion cells in the parasympathetic and sensory ganglia known to innervate the cerebral vessels.

Moderate hypoglycemia induces ultrastructural changes in perivascular nerve terminals of cat cerebral arteries.

A quantitative morphological analysis of the perivascular nerve terminals of cerebral arteries during moderate hypoglycemia was performed. 5-Hydroxydopamine (5-OHDA) was applied to discriminate dense-cored vesicles, related to noradrenaline, and clear vesicles, related to acetylcholine, under the electron microscope. Five hypoglycemic and 5 normoglycemic cats, all receiving 5-OHDA, were compared. In both the middle cerebral artery and vertebral artery, the dense-cored vesicles were significantly smaller and clear vesicles were significantly larger in hypoglycemia than in normoglycemia. These morphological changes in the vesicles may indicate hyperactivity of the sympathetic system and hypoactivity of the parasympathetic system of the cerebral vessels during hypoglycemia.

Effects of locus ceruleus lesions on the pericapillary nerve terminals in the feline brain.

The effects of bilateral locus ceruleus (LC) lesions on the pericapillary nerve terminals were investigated in the feline brain parenchyma using electron microscopy. LC lesions were induced stereotaxically and the animals were sacrificed after intravenous administration of 5-hydroxydopamine (5-OHDA). The diameter and number of dense-cored vesicles (DCVs) and clear vesicles (CVs) in the pericapillary nerve terminals were measured. The number of DCVs in the nerve terminal was significantly decreased by bilateral LC lesions. The diameters of the DCVs and CVs decreased significantly as compared with those in the non-operated control group. These data suggest that the LC is closely related to the pericapillary nerve terminals in the brain parenchyma and that not only nerve terminals with DCVs but also those with CVs are affected by LC lesions.

Pathologic findings of silent hyperintense white matter lesions on MRI.

The pathologic features of silent hyperintense white matter lesions in T2-weighted images on MRI were studied in patients with no neurologic signs or symptoms. The small patchy hyperintense white matter lesions represented myelin pallor associated with vessels showing hypertension and arteriosclerotic changes. 'Caps' also showed myelin pallor with dilated perivascular spaces. There were no lacunar infarcts in these lesions. Some of 'caps' was shown to be elongated normal lateral ventricle. 'Rims' of early stage revealed subependymal gliosis that was a part of normal aging processes.

Choroidal epithelial cyst. Case report.

A supratentorial cyst in the leptomeninges of an 8-month-old infant was studied with the electron microscope. The lining of the cyst consisted of a single layer of peg-shaped epithelial cells rich in organelles and glycogen. Their free border had numerous microvilli but no cilia, and their basal portions rested on a basement membrane. Tight junctions and interdigitations were frequent between contiguous cells. The blood vessels were fenestrated. Since these features characterize developing choroidal epithelial cells, we felt the diagnosis of choroidal epithelial cyst was justified. Cysts lined by choroidal epithelium may continue to secrete cerebrospinal fluid after surgical extirpation when this is incomplete. Accumulation of the fluid in the partially excised cyst bed may therefore account for recurrence of symptoms.

Calbindin-D28k in cerebrovascular extrinsic innervation system of the rat.

Calbindin-D28k, one of the calcium-binding proteins, belongs to the EF hand family and is commonly found in neurons. It serves as a representative neuronal marker for neuroanatomical investigations. The authors' knowledge of its precise function, however, is yet very limited. In this study, we examined the existence of nerve fibers with calbindin-D28k immunoreactivity in the cerebral blood vessels and ganglia that innervate the cerebral blood vessels in the rat. Numerous nerve fibers with calbindin-D28k immunoreactivity were observed on the walls of the major extracerebral arteries forming the circle of Willis and its branches. Calbindin-D28k immunoreactivity was seen in many neurons of the trigeminal, dorsal root and jugular ganglia. A small number of neurons showed calbindin-D28k immunoreactivity in the otic and superior cervical ganglia. Calbindin-D28k immunoreactivity was not detected in the sphenopalatine or internal carotid ganglia. Pericellular basket-like formations of nerve terminals with calbindin-D28k immunoreactivity were observed in the sphenopalatine, otic, internal carotid and superior cervical ganglia. The present study demonstrated calbindin-D28k immunoreactivity in the cerebrovascular nerve fibers as well as in their origins--the cranial ganglia. These findings are significant in understanding the calcium-mediated mechanism of the neural control of the cerebral blood vessels.

Characteristic form of standing up from squatting in Miyoshi's distal muscular dystrophy.

A 27-year-old woman with Miyoshi's distal muscular dystrophy devised a unique form of standing up from a squatting position; She held her ankles with her hands to support the weight transfer, fixed the heels, extended the knees to elevate the hips, raised the upper half of the body, and finally stood up. This strategy illustrates the characteristic and specific distribution of the wasted muscle in this disease.

Sarcomas metastatic to the brain: case report of a metastatic fibrosarcoma, and review of literature.

A case of fibrosarcoma with metastases to the brain is presented with a review of the pertinent literature. This review demonstrates that the incidence of brain metastases in patients with sarcoma is probably higher than previously indicated.

Portal systemic encephalopathy presenting with dressing and constructional apraxia.

We report a case with portal systemic encephalopathy who presented with dressing and constructional apraxia and subtle weakness of the left hand. We initially suspected a cerebrovascular attack in the right cerebral hemisphere, but brain T1-weighted magnetic resonance (MR) imaging revealed high intensity in the basal ganglia and hyperammonemia was detected. We performed abdominal MR angiography, which visualized an intrahepatic portal systemic shunt. Cerebral blood flow, measured by xenon-enhanced computed tomography, was decreased in the bilateral, but more dominantly right-sided, parietal watershed regions. We speculate that these boundary territories might be susceptible to damage by toxic metabolites of hepatic encephalopathy.

[Intravascular malignant lymphomatosis].

Based on our personal experience of two cases of intravascular malignant lymphomatosis (IML) and a review of 128 cases in the literature, the possibilities for clinical diagnosis and successful treatment of this condition are discussed. IML usually presents with hemiparesis or progressive dementia and then with disturbances of consciousness. Paraparesis and dysuria due to spinal cord involvement are not rare. Since increased levels of blood sedimentation rate, CRP, serum LDH, and CSF protein are often observed in the patients, a diagnosis of IML must be included among the list of differential diagnoses when these findings are encountered in a patient presenting with clinical features similar to those of cerebrovascular diseases or multiinfarct dementia. The diagnosis must be confirmed histologically, so that an appropriate treatment can be initiated early in the course of the disease. Skin biopsy is indicated when skin eruption is present. Autopsy records of patients have revealed involvement of intravascular lymphoma to the kidney, adrenals, lungs and liver in more than 80% of cases. It is suggested therefore that biopsies are performed for these organs. Muscle biopsy can also be useful for making a diagnosis. Since autopsy studies have revealed swelling of the adrenals in some cases, including ours, CT or MRI images of the adrenals might be of importance. Regarding treatment, chemotherapy must be indicated. However, this has so far shown either no or only a temporary efficacy, partly because of the delay in reaching a diagnosis. A cure for IML could be expected through early initiation of combined treatment with various chemotherapeutic agents in the near future.