Diabetic cervical radiculoplexus neuropathy: a distinct syndrome expanding the spectrum of diabetic radiculoplexus neuropathies.

Diabetic lumbosacral radiculoplexus neuropathy is a subacute painful, asymmetrical lower limb neuropathy due to ischaemic injury and microvasculitis. The occurrence of a cervical diabetic radiculoplexus neuropathy has been postulated. Our objective was to characterize the clinical features and pathological alterations of diabetic cervical radiculoplexus neuropathy, to see if they are similar to diabetic lumbosacral radiculoplexus neuropathy and due to ischaemic injury and microvasculitis. We identified patients with diabetic cervical radiculoplexus neuropathy by review of the Mayo Clinic database from 1996 to 2008. We systematically reviewed the clinical features, laboratory studies, neurophysiological findings, neuroimaging and pathological features and compared the findings with a previously published diabetic lumbosacral radiculoplexus neuropathy cohort. Eighty-five patients (56 males, 67 with Type 2 diabetes mellitus) were identified. The median age was 62 years (range 32-83). The main presenting symptom was pain (53/85). At evaluation, weakness was the most common symptom (84/85), followed by pain (69/85) and numbness (56/85). Neuropathic deficits were moderate (median motor neuropathy impairment score 10.0 points) and improved at follow-up. Upper, middle and lower brachial plexus segments were involved equally and pan-plexopathy was not unusual (25/85). Over half of patients (44/85) had at least one additional body region affected (30 contralateral cervical, 20 lumbosacral and 16 thoracic) as is found in diabetic lumbosacral radiculoplexus neuropathy. Recurrent disease occurred in 18/85. Neurophysiology showed axonal neuropathy (80/80) with paraspinal denervation (21/65), and abnormal autonomic (23/24) and sensory testing (10/13). Cerebrospinal fluid protein was elevated (median 70 mg/dl). Magnetic resonance imaging showed brachial plexus abnormality in all (38/38). Nerve biopsies (11 upper and 11 lower limbs) showed ischaemic injury (axonal degeneration, multifocal fibre loss 15/22, focal perineurial thickening 16/22, injury neuroma 5/22) and increased inflammation (epineural perivascular inflammation 22/22, haemosiderin deposition 6/22, vessel wall inflammation 14/22 and microvasculitis 5/22). We therefore conclude that (i) diabetic cervical radiculoplexus neuropathy is a predominantly monophasic, upper limb diabetic neuropathy with pain followed by weakness and involves motor, sensory and autonomic fibres; (ii) the neuropathy begins focally and often evolves into a multifocal or bilateral condition; (iii) the pathology of diabetic cervical radiculoplexus neuropathy demonstrates ischaemic injury often from microvasculitis; and (iv) diabetic cervical radiculoplexus neuropathy shares many of the clinical and pathological features of diabetic lumbosacral radiculoplexus neuropathy, providing evidence that these conditions are best categorized together within the spectrum of diabetic radiculoplexus neuropathies.

Proinflammatory cytokines in the cerebrospinal fluid of patients with lumbar radiculopathy.

In pathologic radicular pain of lumbar spinal stenosis, cytokines such as tumor necrosis factor-alpha (TNF-α) and interleukins (ILs) play a crucial role in the pathogenesis of nerve degeneration and pain. We investigated TNF-α and IL-6 levels in the cerebrospinal fluid (CSF) of patients with radicular pain caused by lumbar spinal stenosis (LSS). A total of 30 LSS patients and 10 age-matched controls were examined. CSF samples were obtained adjacent to the level of stenosis in 30 LSS patients, and at the L4-L5 level in the 10 control patients. TNF-α and IL-6 levels in the samples were analyzed using enzyme-linked immunosorbent assays (ELISA). We compared the amounts of TNF-α and IL-6 with severity of pain (low back and leg pain), walking ability, and severity of stenosis (cross-sectional area of dural space). The concentration of IL-6 was significantly higher in LSS patients than in controls, but TNF-α levels were beneath the limit of detection. There was no correlation between IL-6 levels and severity of pain or walking ability (p > 0.05). However, there was a significant correlation between IL-6 levels and severity of stenosis (p < 0.05). The current study showed that the increased CSF IL-6 levels in LSS patients with radicular pain were not correlated with pain severity; although not proven in this study, the increase in CSF IL-6 concentration could indicate pathological nerve damage or degeneration of lumbar radiculopathy represented by the severity of stenosis.

High-resolution nuclear magnetic resonance spectroscopic study of metabolites in the cerebrospinal fluid of patients with cervical myelopathy and lumbar radiculopathy.

There have been few reports describing substances related to oxidative and intermediary metabolism in the cerebrospinal fluid (CSF) in patients with spinal degenerative disorders. This study investigated whether the concentrations of metabolites in the CSF differed between patients with spinal degenerative disorders and controls, and whether the concentrations of these metabolites correlated with the severity of symptoms. CSF samples were obtained from 30 patients with cervical myelopathy (Group M), 30 patients with lumbar radiculopathy (Group R), and 10 volunteers (control). Metabolites in these CSF samples were measured by nuclear magnetic resonance spectroscopy. There were no differences in the concentrations of lactate, alanine, acetate, glutamate, pyruvate, or citrate between Groups M and R, between Group M and the control, or between Group R and the control. In Group M, neither symptom duration nor the Japanese Orthopaedic Association score correlated with the concentration of any metabolite. In Group R, the symptom duration positively correlated with the concentration of lactate, glutamate, and citrate in CSF. The duration of nerve root block showed a negative correlation with the concentrations of acetate in CSF of the patients in Group R. In patients with lumbar radiculopathy, there is a possibility of increased aerobic metabolic activity or decreased gluconeogenic activity in patients with shorter symptom duration, and increased aerobic metabolic activity in patients with severe inflammation around a nerve root.

Clinical and MRI phenotypes of sarcoidosis-associated myelopathy.

OBJECTIVE: To determine the characteristic clinical and spinal MRI phenotypes of sarcoidosis-associated myelopathy (SAM), we analyzed a large cohort of patients with this disorder. METHODS: Patients diagnosed with SAM at a single center between 2000 and 2018 who met the established criteria for definite and probable neurosarcoidosis were included in a retrospective analysis to identify clinical profiles, CSF characteristics, and MRI lesion morphology. RESULTS: Of 62 included patients, 33 (53%) were male, and 30 (48%) were African American. SAM was the first clinical presentation of sarcoidosis in 49 patients (79%). Temporal profile of symptom evolution was chronic in 81%, with sensory symptoms most frequently reported (87%). CSF studies showed pleocytosis in 79% and CSF-restricted oligoclonal bands in 23% of samples tested. Four discrete patterns of lesion morphology were identified on spine MRI: longitudinally extensive myelitis (n = 28, 45%), short tumefactive myelitis (n = 14, 23%), spinal meningitis/meningoradiculitis (n = 14, 23%), and anterior myelitis associated with areas of disc degeneration (n = 6, 10%). Postgadolinium enhancement was seen in all but 1 patient during the acute phase. The most frequent enhancement pattern was dorsal subpial enhancement (n = 40), followed by meningeal/radicular enhancement (n = 23) and ventral subpial enhancement (n = 12). In 26 cases (42%), enhancement occurred at locations with coexisting structural changes (e.g., spondylosis). CONCLUSIONS: Recognition of the clinical features (chronically evolving myelopathy) and distinct MRI phenotypes (with enhancement in a subpial and/or meningeal pattern) seen in SAM can aid diagnosis of this disorder. Enhancement patterns suggest that SAM may have a predilection for areas of the spinal cord susceptible to mechanical stress.

Tumor necrosis factor-alpha, interleukin-1beta, and interleukin-6 in the cerebrospinal fluid of patients with cervical myelopathy and lumbar radiculopathy.

There have been few reports describing cytokines in the cerebrospinal fluid (CSF) of patients with spinal degenerative disorders. This study investigated whether interleukin-1beta (IL-1beta), interleukin-6 (IL-6), and tumor necrosis factor-alpha (TNF-alpha) could be detected in CSF of patients with cervical myelopathy or lumbar radiculopathy and whether the concentrations of those cytokines correlated with the severity of disease conditions. CSF samples were obtained from 21 patients with cervical myelopathy (Group M) and 19 patients with lumbar radiculopathy (Group R), and six volunteers (control). The concentration of IL-6 was significantly higher in Groups M and R than in the control, possibly demonstrating spinal cord and nerve root damage, respectively. However, TNF-alpha was lower than the detection limit. IL-1beta was detected in only five samples from three patients in Group M and two volunteers in the control. The concentrations of IL-6 did not show any correlation with symptom duration, the scoring system by the Japanese Orthopaedic Association, or the duration of nerve root block. There is a possibility that the concentration of inflammatory cytokines in CSF can indicate certain pathological aspects of cervical myelopathy or lumbar radiculopathy.

Schistosomal Myeloradiculopathy - A case report.

The most common neurological impairments related to schistosomiasis involve the lower portions of the medulla and the cauda equina. A 22-year-old woman, with no history, signs, or symptoms of hepatointestinal schistosomiasis, presented with lumbar pain associated with acute paresthesia and paresis of the right lower limb. Spinal schistosomiasis was suspected based on the disease progression and radiological findings, and the diagnosis was confirmed after cerebrospinal fluid analysis. The authors emphasize this pathology as important as a differential diagnosis in similar clinical scenarios, especially in endemic areas, because both early diagnosis and treatment are essential to avoid permanent sequelae.

Viral ascending radiculomyelitis with severe hypoglycorrachia.

Ascending radiculomyelitis in a 52-year-old woman was accompanied by transient marked hypoglycorrachia and polymorphonuclear pleocytosis of the CSF. Examination of the CSF later in the course of illness revealed albuminocytologic dissociation. This unusual sequence of findings was associated with serologic evidence of a recent cytomegalovirus (CMV) infection. In addition, the illness was associated with a nonthrombocytopenic purpuric rash. We proposed that the rash and neurologic disease were consequences of vasculitis caused by CMV infection.

The immune response at onset and during recovery from Borrelia burgdorferi meningoradiculitis.

BACKGROUND: Borrelia burgdorferi causes a wide range of neurologic syndromes. In Europe, acute meningoradiculitis is the most common manifestation. OBJECTIVE: To address the nature of the immune response during the course of B burgdorferi meningoradiculitis, with special respect to the early and late changes in cerebrospinal fluid (CSF). METHODS: Serial immunophenotyping was performed and cytokine measurements were obtained in the peripheral blood and CSF of 12 European patients with definite B burgdorferi meningoradiculitis. RESULTS: Early during infection and before initiation of treatment, we observed high levels of interleukin (IL) 10, IL-6, and IL-8, and large numbers of B cells and plasma cells in the CSF of most patients. At the same time, we found a mainly unspecific intrathecal antibody synthesis. During resolution of the infection, cytokine levels normalized rapidly and plasma cells disappeared from the CSF. In parallel, the percentage of B cells in the CSF increased over several months, accompanied by rising levels of intrathecally produced B burgdorferi-specific antibodies. CONCLUSIONS: Our findings demonstrate that the early phase of B burgdorferi meningoradiculitis is characterized by a well-coordinated immune response involving specific cytokine release and plasma cell recruitment, followed by a long-lasting, antigen-specific B-cell response in the central nervous system.

Predominance of neutrophils in the cerebrospinal fluid of AIDS patients with cytomegalovirus radiculopathy.

Cytomegalovirus (CMV) radiculopathy has been associated with both viral cytopathic inclusions and an increased number of neutrophils in the cerebrospinal fluid (CSF) of patients with AIDS. The significance of these findings is unknown. To evaluate this, the authors reviewed all CSF cytology specimens from patients with a history AIDS or HIV infection over a 9-year period. Of 193 specimens identified, 42 (22%) had neutrophils present. Neutrophils were rare (<6 per slide) in the majority of specimens (57%). Occasional neutrophils (<2/hpf) were observed in three patients; one with suspected CMV myelitis, one with bacterial meningitis, and one with cryptococcal meningitis. All 6 cases (3 patients) with numerous neutrophils (>10/hpf) had positive CMV CSF cultures and symptoms of radiculopathy. Definite viral inclusions were not seen. The prognosis was poor in all cases. The authors conclude that diagnostic CMV inclusions are quite rare. However, the presence of elevated numbers of neutrophils in the CSF of a patient with AIDS without an identified infectious agent is highly suggestive of CMV radiculopathy.

Eosinophilic radiculomyeloencephalitis: an angiostrongyliasis outbreak in American Samoa related to ingestion of Achatina fulica snails.

For the first time in American Samoa an outbreak of eosinophilic radiculomyeloencephalitis was related to eating giant African snails (Achatina fulica) infected with Angiostrongylus cantonensis. Among 24 Korean fisherman sharing the same infective meal, 16 who ate raw or partially cooked snails became ill; five who ate boiled snails and three who ate none remained well. The ensuing illnesses began within 1-6 days, persisted up to 10 weeks, and were characterized by both peripheral blood and spinal fluid eosinophilia, severe pains, weakness and hyporeflexia of the legs, and dysfunction of the bladder and bowels. Eight patients also had transient hypertension and/or lethargy, and three became comatose. One man died 17 days after eating the infected snails, and maturing larvae of A. cantonensis were found in his spinal cord. Enzyme-linked immunosorbent assay titers for antigens of A. cantonensis were elevated to 1:64 or greater (mean 1:128) in all 10 patients tested. Treatment with thiabendazole had no appreciable effect on the clinical course of the illness.

Autoradiographic demonstration of proliferating cells in cerebrospinal fluid.

The proliferative activity of cells, isolated from 82 human CSF specimens, was examined by 3H-thymidine autoradiography. High labelling indices (LI) were found in acute viral meningitis (up to 8 per cent) and radiculitis (up to 6 per cent). CSF cell proliferation was also shown in the subacute stages of viral diseases and in other inflammatory processes (LI ranging from 0.5 per cent to 3 per cent). Most of the cells labelled from these CSF specimens were large lymphocytes, "lymphoid cells" and plasmacytes. Their presence in CSF is presumed to indicate an immune reaction. By the demonstration of a proliferative activity of these cells, aseptic inflammatory processes can be differentiated from "unspecific" pleocytosis. Because of a correlation between the LI of CSF cells and the stages of some inflammations, this method is suggested for an assessment of pregression or remission of chronic processes, e.g. "chronic meningitis" and multiple sclerosis. It can also be used in experimental research: the same type of mononuclear cells was labelled after having been cultured for 23 hours prior to the incubation with 3H-thymidine. Proliferating tumor cells as well as proliferating non-neoplastic mononuclear cells were demonstrated in CSF from various neoplastic diseases. In the clinical diagnosis of these processes, the method is of limited value. It proved very useful, however, for an assessment of the therapeutic effects of intrathecal cytostatic therapy. CSF specimens from non-inflammatory and non-neoplastic diseases regularly contained very few proliferating cells (LI: less than 0.1).

Abnormal CSF immunoglobulin components detected by isoelectric focusing.

The results from thin-layer isoelectric focusing of 4000 paired CSF and serum samples were examined for the occurrence of abnormal CSF immunoglobulin components. Such changes, not referable to serum protein abnormalities, were detected in 630 samples from 465 patients. All but 3 of these subjects had symptoms and signs of an affection of the nervous system, predominantly MS and encephalitic or myelitic/radiculitic disorders of known or presumed infectious etiology. The abnormal, microheterogeneous immunoglobulin components were mostly focused in the pI range approximately 7.5--9 with generally a shift towards pI-values > 8 pH units; a distribution differing from that of M components in plasma cell dyscrasias.

Demonstration of oligoclonal immunoglobulin G in Guillain-Barré syndrome and lymphocytic meningoradiculitis by isoelectric focusing.

Isoelectric focusing (IEF) of serum and CSF revealed oligoclonal IgG in 13 out of 16 patients with the Guillain-Barré syndrome (GBS), in 10 of them in serum only and in 3 in serum and CSF. Seventeen out of 19 patients with lymphocytic meningoradiculitis (LMR) showed oligoclonal IgG, 12 of them in CSF only. These findings, together with additional results, mean that in GBS oligoclonal IgG is synthesized mainly outside the CNS and in LMR within the CNS. Follow-up studies revealed changes in the oligoclonal IgG during the course of GBS and LMR. After treatment by plasma exchange the disappearance of oligoclonal IgG bands was followed by an improvement of GBS symptoms. The oligoclonal IgG bands returned in correlation with worsening of the disease. We were not able to elucidate the antibody character of oligoclonal IgG in GBS or LMR. No antibodies against the myelin basic protein (fragment 89-169) were detectable in the sera and CSF in any of the patients tested.

Meningoradiculitis and encephalomyelitis due to Borrelia burgdorferi: a follow-up study of 72 patients over 27 years.

In 1987, follow-up studies were conducted on 72 patients who had had meningoradiculitis and encephalomyelitis (8 patients) due to Borrelia burgdorferi 5-27 years previously. These patients had not been treated with antibiotics, either during the acute disease or during the interval prior to follow-up studies. The patients had exhibited the typical symptoms of Bannwarth's syndrome during the acute phase. At the follow-up studies, 33 patients showed no, and 23 only mild, clinical residual symptoms including normal CSF findings and low-positive serum IgG borrelia antibody titres (IFT; ELISA). Three patients without sequelae exhibited persistent intrathecal secretion of oligoclonal B. burgdorferi-specific CSF IgG antibodies (Immunoblot; positive borrelia CSF IgG antibody titres). Thirteen patients exhibited mild-to-medium sequelae with persistent intrathecal formation of oligoclonal B. burgdorferi-specific CSF IgG antibodies, up to 21 years after the acute illness. This persistence can be interpreted as an "immunological scar syndrome". Our follow-up studies appear to indicate that neurological manifestations of B. burgdorferi infections are generally (with few exceptions) of a benign nature. Most patients can be classified as having been cured without antibiotic therapy. No late manifestations of chronic progressive CNS borreliosis comparable to that of neurosyphilis have been seen following acute untreated neuroborreliosis.

Radiculomyelitis complicating acute haemorrhagic conjunctivitis. A clinical study.

Fourteen patients with radiculomyelitis following acute haemorrhagic conjunctivitis (AHC) were seen in Bangkok during October to December 1974. Most patients developed weakness of extremities 2 weeks after AHC. Prodromal symptoms consisted of fever and malaise for a few days, followed by the acute onset of root pain in the legs and flaccid paralysis. Knee and ankle reflexes were absent or diminished. Cerebrospinal fluid examination revealed lymphocytosis and an increase in protein. Electromyographic findings were consistent with anterior horn cell or motor root lesions. Ten of the 12 cases in which virological studies were performed showed definite serological evidence of AHC virus infection. Six patients received corticosteroid treatment but apart from relief of pain no significant improvement was seen. Motor weakness in 10 patients was less at the end of 2 months, but in 4 it remained unchanged. The occurrence of disabling neurological sequelae calls for effective public health control of AHC outbreak.

Superoxide dismutase activity in cerebrospinal fluid and its relation to compression of the lumbosacral nerve root.

In the pathophysiology of lumbosacral radiculopathy, inflammation of the nerve root is of critical importance. Additionally, free radicals have been shown to be associated with some inflammatory process. This study was designed to investigate whether free radicals participate in the pathophysiology of nerve root involvement. We measured superoxide dismutase (SOD) activity in cerebrospinal fluid (CSF) of 31 patients with unilateral lumbosacral radiculopathy caused by a herniated disc using electron spin resonance (ESR) spectrometry. Then SOD activity was compared with the type of nerve root compression as seen on preoperative myelography. SOD activity in the normal control group was 7U/ml, while that in the hernia group remarkably decreased. The concentration gradient of SOD activity was different between central herniation and centrolateral herniation. Our findings indicate that free radicals are generated after nerve root compression. Under severe deficiency of SOD activity in CSF, serum SOD penetrates into CSF after further compression. In addition, SOD in CSF may play an important role in protecting against nerve root involvement.

[Neurologic forms of Lyme disease. 12 cases]. / Les formes neurologiques de la maladie de Lyme: 12 cas.

Twelve cases of Lyme's disease with neurological complications are reported. Seven patients had meningoradiculitis of the Garin-Bujadoux-Bannwarth type, with facial palsy in 2 cases. In 1 case the radiculitis involved only the cauda equina. Two more patients had meningomyelitis. Of the remaining 3, 1 had subacute inflammatory polyneuritis with albumino-cytologic dissociation, 1 had probable dorsal epiduritis, and the last one developed parkinsonism and communicating hydrocephalus after an otherwise classical meningoradiculitis. Three patients recalled a tick bite but only one a cutaneous eruption. No arthritis or cardiac involvement were observed. In 2 cases the CSF contained pseudo-neoplastic cells. Severe pain was a prominent feature in most cases. Pain consistently and rapidly improved on high-dose intravenous penicillin, while other signs or symptoms (e.g. paresthesias or fatigue) often lasted several months. Parkinsonism and hydrocephalus were not influenced by penicillin, and both required specific therapy. Isolated neurological (both central and peripheral) involvement is not unusual in Lyme's disease and may give rise to a wide range of signs and symptoms. This diagnosis is to be considered even when other features of Borrelia burgdorferi infection are lacking.

[Meningomyeloradiculopathy in schistosomiasis mansoni. Clinical and cerebrospinal fluid evaluation in 16 cases]. / Meningomielorradiculopatia na esquistossomose mansônica. Avaliação clínica e do líquido cefalorraqueano fm 16 casos.

Data on 16 patients with spinal cord involvement by Schistosomiasis mansoni are evaluated as to the clinical course and the evolution of cerebrospinal fluid changes. According to evidences of radicular involvement cases were divided in two groups: myelitis (9 cases) and radiculomyelitis (7 cases). Cerebrospinal fluid changes were evaluated as to cytology, total protein content and gammaglobulins. Partial remission of clinical symptomatology was more common among patients of the second group than among those of the first group. There was not relationship of CSF changes and their course with the clinical course of the disease. Cerebrospinal fluid changes and their course were not related to clinical aspects of the disease and their course. Remission of hypercytosis was more common than the remission of protein changes along the evolution in the two groups of cases considered.

[Primary acute and subacute meningomyeloradiculopathies: study of the cerebrospinal fluid]. / Meningomielorradiculopatias agudas e sub-agudas primárias: estudo do líquido cefalorraqueano.

Forty four patients with primary acute and subacute meningomyeloradiculopathies were studied in respect to the evolutive aspects of the cerebrospinal fluid (CSF) cell-protein dual in several periods of the disease. The tendency of CSF hypercytosis to normal values of cell count occurred in most cases (96%) at the end of the period in which the study was performed (60 days). The CSF protein levels had a similar but slower behavior pattern when compared with hypercytosis. The comparative analysis of clinical improvement of two groups of patients (treated and not treated with ACTH or corticosteroids) showed a statistically significant difference between on the two groups favouring the former. This fact enhances the possibility that an auto-immune process plays an important role in cases of primary meningomyeloradiculopathies.

[Lymphocytic meningoradiculitis (Bannwarth's syndrome). Neurologic involvement of Lyme disease]. / Meningoradiculitis linfocitaria (síndrome de Bannwarth). Expresión neurológica de la enfermedad de Lyme.

We describe a patient with Lyme's disease who showed neurologic symptoms of meningoradiculitis (Bannwarth's syndrome) and had no previous history of exposure to tick bite or chronic erythema migrans. He had longterm fever and bilateral facial paralysis. Antibody titres for Borrelia Burgdorferi in serum and cerebrospinal fluid (CSF) were increased. The CSF changes showed an intrathecal production of immunoglobulins (presence of plasmatic cells and immunoblasts; IgG-CSF: IgG-serum ratio much higher than albumin-CSF: Albumin serum ratio, according to Reiber's diagram; presence of oligoclonal bands). Therapeutic response to high doses of penicillin was resolutive. The second day of treatment the patient developed uveitis, that was treated with corticoids.