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Cytomegalovirus polyradiculopathy in HIV-infected patients.

Cytomegalovirus polyradiculopathy, a late complication of HIV infection, is characterized by lower extremity weakness, urinary retention, and sacral dysesthesias. We describe four patients (mean CD4 T-cell count = 25 cells/mm3) who developed this "infectious cauda equina syndrome." The characteristic cerebrospinal fluid (CSF) findings, notably atypical for a viral infection, included polymorphonuclear leukocytosis (mean white blood cell count = 1512 cells/mm3, 72% polymorphonuclear leukocytes), elevated protein level (mean = 370 mg/dl), and hypoglycorrhacia (mean = 28 mg/dl). Physicians who treat patients with HIV should be familiar with this syndrome because early intervention, prior to microbiologic confirmation, provides the best hope for improving neurologic function.

AIDS-Related Opportunistic Infections↗

Diabetic thoracic polyradiculopathy.

Diabetic thoracic polyradiculopathy usually causes severe, chronic abdominal pain in patients with type 2 diabetes of variable duration. Other diabetic complications, weight loss and paretic abdominal wall protrusion are common. Sensory, motor and autonomic functions are affected. The diagnosis can be made from the characteristic history, physical examination findings, paraspinal electromyography, and other procedures. The differential diagnosis includes postherpetic neuralgia, abdominal wall pain, malignancy, and other spinal disorders. The pathology appears to be immune-mediated neurovasculitis resulting in ischemic injury. Traditional therapy is symptomatic, but recent pathological findings and clinical experience suggest that immunotherapy may be effective.

Abdominal Pain↗

Subclinical lumbar polyradiculopathy, polyneuritis and ganglionitis in aged wild and exotic mammalians.

Subclinical lumbar polyradiculopathy was present in the intradural dorsal and ventral nerve rootlets of 19 aged individuals of the following wild and exotic mammalian species: woodrat, raccoon, mink, lynx, reindeer, red deer, musk ox, scimitar-horned oryx, Arabian oryx, hybrid waterbuck, Persian onager, Przewalski's wild horse, Malayan sun bear, Asian elephant, East African river hippopotamus, vervet monkey and rhesus monkey. It was characterized by mild to severe multifocal ballooning of myelin sheaths. Occasionally, ballooned myelin sheaths contained thin strands of myelin and macrophages surrounding distorted axons. Additionally, a mild incidental lymphocytic polyneuritis was present in intradural nerve rootlets of the Malayan sun bear, and a moderate lymphocytic spinal ganglionitis in the East African river hippopotamus.

Age Factors↗

Late cytomegalovirus polyradiculopathy following haploidentical CD34+-selected hematopoietic stem cell transplantation.

A 55-year-old man with acute myeloid leukemia in second relapse presented 4 months after haploidentical CD34+-selected hematopoietic stem cell transplantation (HSCT) with symmetric, progressive neurological deficits of the lower extremities. Although there was no molecular evidence for drug resistance in the cerebral-spinal fluid, antiviral combination therapy failed to control the rapidly progressing CMV polyradiculopathy (PRP) and encephalitis, which were confirmed by autopsy studies. Late CMV PRP as an unusual manifestation of CMV disease should be kept in mind in patients with suggestive neurological symptoms after HSCT.

Acute Disease↗

Cytomegalovirus polyradiculopathy in patients with AIDS.

Using the MEDLINE database, we evaluated 103 cases of AIDS-related cytomegalovirus (CMV) polyradiculopathy (PRP). In 13% of cases, PRP was the initial manifestation of AIDS. Hyporeflexia was present in 100% of cases; lower limb weakness, in 100%; urinary retention, in 94%; paresthesia, in 79%; sensory loss, in 80%; and a Babinski's sign, in 16%. Mean cerebrospinal fluid (CSF) parameters +/- SD were as follows: white blood cell count, 651 +/- 1,053 x 10(6)/L; protein level, 2.28 +/- 1.78 g/L; and CSF/serum glucose ratio, 0.48 +/- 0.17. Gadolinium enhancement of meninges on a magnetic resonance image and abnormalities on a myelogram were noted in 31% and 17% of cases, respectively. Mean survival time +/- SD was 5.4 +/- 1.8 weeks for untreated patients and 14.6 +/- 9.4 weeks for patients treated with ganciclovir (P < .0001), but it was only 7.2 +/- 3.0 weeks for patients receiving ganciclovir treatment at the onset of PRP. CMV-related PRP is an uncommon but distinctive complication of AIDS. Early diagnosis is possible, and other causes can be excluded by lumbar magnetic resonance imaging and by the presence of typical CSF changes, as shown by polymerase chain reaction of CMV. Retrospectively, survival time for naive patients was increased by ganciclovir therapy and may even be underestimated in this evaluation of historical reports.

AIDS-Related Opportunistic Infections↗

Cytomegalovirus polyradiculopathy caused by a ganciclovir-resistant strain.

Polyradiculopathy caused by cytomegalovirus (CMV) is a rare but serious neurological disorder that occurs late in the course of HIV-1 infection and is potentially treatable with antimicrobial agents active against CMV. We describe a patient with CMV infection caused by a strain that was resistant to ganciclovir. This case demonstrates one potential mechanism of therapeutic failure and illustrates the potential pathogenicity of strains that are resistant to antiviral drugs.

AIDS-Related Opportunistic Infections↗

Acute lumbosacral polyradiculopathy due to cytomegalovirus in advanced HIV disease: CSF findings in 17 patients.

OBJECTIVES: To describe the abnormalities in CSF from HIV infected patients with acute lumbosacral polyradiculopathy (ALP) caused by cytomegalovirus (CMV) infection. METHODS: Retrospective case notes and laboratory records were reviewed for 17 consecutive patients with CMV associated ALP admitted to specialist HIV/AIDS units at UCL Hospitals and Chelsea and Westminster Hospital. RESULTS: Infection with CMV was confirmed by detection of CMV DNA by polymerase chain reaction amplification in 15 patients (all of whom were negative by culture), by culture in one patient, and by objective clinical response to anti-CMV treatment in one patient. Only nine patients had a CSF pleocytosis 28-1142 (median 150) cells/mm3; in seven there was a polymorphonuclear (PMN) leucocyte preponderance. Protein concentrations in CSF were moderately or considerably raised in 13 patients; CSF: plasma glucose ratios were < or = 50% in five patients. Two patients had no pleocytosis, normal CSF: plasma glucose, and normal or near normal protein values. CONCLUSIONS: Abnormalities in CSF in CMV associated ALP are varied: only 50% of patients have a "typical" PMN preponderant pleocytosis. The diagnosis of this condition should not rely on demonstration of a PMN preponderant pleocytosis, but on identification of CMV DNA in CSF and the exclusion of other opportunistic infections and lymphoma in order that specific anti-CMV treatment may be instituted.

AIDS-Related Opportunistic Infections↗

Vasculitic polyradiculopathy in systemic lupus erythematosus.

A 22 year old woman with recently diagnosed systemic lupus erythematosus presented with subacute progressive areflexic paraparesis, electrophysiologically identified as a pure axonal polyradiculopathy. Sural nerve biopsy disclosed necrotising vasculitis. A striking radiological feature was marked enhancement of the cauda equina with gadolinium.

Adult↗

Early syphilis presenting as a painful polyradiculopathy in an HIV positive individual.

Neurosyphilis and neurological complications from syphilis may be commoner in HIV disease. With outbreaks of early syphilis in HIV positive individuals being observed over recent years, rare neurological manifestations of secondary syphilis will be observed more commonly. We describe a case of an HIV positive individual whose first presenting feature of early syphilis was a polyradiculopathy.

HIV Infections↗

Post-irradiation polyradiculopathy mimics leptomeningeal tumor on MRI.

Three patients with a remote history of Hodgkin's disease treated with total or subtotal lymphoid radiation 17 to 24 years earlier developed lumbosacral polyradiculopathy associated with nodular meningeal enhancement of the conus medullaris and cauda equina on MRI. None had evidence of recurrent Hodgkin's disease or second malignancy, and the MRI findings may be sequelae of radiation therapy.

Adult↗

Subclinical lumbar polyradiculopathy in aged domestic, laboratory, and exotic mammalian species--a light and selected electron microscopic study.

Lumbar polyradiculopathy, characterized by ballooning myelin sheaths and axonal distortion, was documented in multiple, aged, domestic, laboratory, and exotic mammalian species. Animals studied that exhibited this nerve rootlet change included the goat, sheep, pig, white-tailed deer, gerbil, vole, ferret, beaver, hedgehog, chinchilla, North Chinese leopard, lion, cheetah, mountain lion, llama, East African eland, Uganda giraffe, polar bear, and drill. Ultrastructurally, lumbar nerve rootlets from the North Chinese leopard revealed prominent and often multiple lamellar separations in the area of myelin bubbling. Clefts in myelin sheaths contained macrophages with engulfed fragments of myelin. Axons were sometimes variously attenuated, and the axoplasm contained densely packed neurofilaments. In all species, the lesion was subclinical and considered an incidental age-related finding.

Aging↗

[A case of acquired immune deficiency syndrome presenting acute lumbosacral polyradiculopathy due to opportunistic infection of cytomegalovirus].

We reported a case of acquired immune deficiency syndrome (AIDS) with acute lumbosacral polyradiculopathy (ALSP), resulting from the opportunistic infection of cytomegalovirus (CMV). A 22-year-old Thai woman noticed weakness of the both legs, and two weeks later, she became unable to walk and had the difficulty of voiding. Neurological examination revealed flaccid paraplegia, sensory disturbance of the both legs, areflexia of the patella and ankle, and urinary retention. She could not move the legs on either side except for ankle flexion or extension, and the passive elevation of the leg brought about severe sacral pain. Radiological examinations, including lumbar MRI, failed to reveal abnormal findings. The needle EMG showed an acute denervation of the lower leg muscles, and the lumbar puncture yielded a colorless fluid containing 2,097/cu mm WBC (polynuclear 88%), 412 mg/dl protein and 45 mg/dl glucose. The serum HIV-1 antibody was positive with a marked loss of CD4 lymphocytes (31/cu mm). In CSF, the DNA of CMV was detected in the polymerase chain reaction (PCR) method. In addition, large round cells with intranuclear or cytoplasmic inclusions showing immunopositivity for the CMV antibody were present. Ganciclovir (daily dose: 400 mg, every 12 h) was administered for two weeks, but the painful numbness gradually extended to the trunk. For AIDS patients, ALSP caused by the CMV infection is a rare neurological complication, and this is the first case report in Japan. Progressive flaccid paraplegia with sensory disturbance, radicular pain, or bladder dysfunction are characteristic symptoms, and CSF pleocytosis with elevated protein or hypoglycorrhachia provides a diagnostic clue for clinicians. In addition, the CMV-DNA amplification in the PCR method or immunohistochemical approach from CSF is a useful procedure.

AIDS-Related Opportunistic Infections↗

Subacute polyradiculopathy with optic and auditory nerve involvement.

Two patients are described, who, after an otherwise trivial viral upset, presented with an acute syndrome consisting of polyradiculopathy affecting all four limbs, with additional severe optic and auditory nerve involvement and other central nervous system signs. A combination of some of the features of the Guillain-Barré syndrome and a simultaneous acute episode of demyelination in the central nervous system were seen in these patients.

Adult↗

Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness.

OBJECTIVE: To describe four syndromes of acute regional weakness with clinical, spinal fluid, and electrophysiologic similarities to the acute immune polyneuropathy of Guillain-Barré syndrome. DESIGN: Case series of personally examined patients. RESULTS: Seven patients are described: four with facial diplegia and distal limb paresthesias, one with sixth nerve palsy and distal paresthesias, one with bilateral lumbar polyradiculopathy, and one with combined Fisher's syndrome and pharyngeal-cervical-brachial weakness. These self-limited illnesses, which evolved over days or weeks, involved acellular cerebrospinal fluid with raised protein concentration and electrophysiologic findings that were consistent with a demyelinating polyneuropathy. CONCLUSIONS: The first three regional variants of Guillain-Barré syndrome may cause diagnostic difficulty, particularly at the onset of illness, and the fourth links Fisher's syndrome with the typical syndrome. The consistently bilateral weakness of Guillain-Barré syndrome and its regional variants and the absence of a monoparetic or hemiparetic pattern suggest that the pathologic process occurs in the same single or contiguous groups of nerves on both sides of the sagittal plane but is not randomly distributed in the peripheral nervous system.

Abducens Nerve↗

Polyradiculopathy complicating acute lymphoblastic leukemia.

A patient who presented with a symmetrical polyradiculopathy 6 months before the onset of acute lymphoblastic leukemia (ALL) is described. This case shows that bone marrow aspiration should be considered in such presentations when no other cause has been identified, even if the CSF is not initially diagnostic and the full blood count appears normal.

Adult↗

Polyradiculopathy in leptomeningeal metastasis: the role of EMG and late response studies.

Leptomeningeal metastasis (LM) of systemic malignancy often give rise to multifocal involvement of the central (CNS) and peripheral nervous system. Signs of CNS dysfunction such as seizures and confusion often overshadow radicular deficits. We report clinical and laboratory evaluations in 10 patients with LM who presented with isolated polyradiculopathy. Our data suggest that electromyography and late response studies are sensitive indicators of radicular dysfunction in patients with LM. These studies are helpful in the early documentation of root involvement when computerized tomography, MRI and myelography are normal.

Adult↗

Acute polyradiculopathies in HIV-infected patients.

We studied 17 consecutive cases of acute polyradiculopathy (PR) diagnosed in HIV-infected patients to investigate the possible causes of this syndrome in our milieu. Sixteen patients presented with lumbosacral PR and one patient had predominantly cervical PR. Electrophysiological study showed a predominantly motor axonal neuropathy in all patients examined. Six patients had a laboratory-confirmed aetiology for the PR: cytomegalovirus (CMV) was isolated from cerebrospinal fluid (CSF) in three cases, meningeal lymphomatosis was diagnosed by CSF cytology in two cases, and one patient had cryptococcal meningitis. Another patient was thought to have acute axonal polyradiculoneuritis associated with HIV infection. CMV and Mycobacterium tuberculosis were the probable agents in four and three patients, respectively. Finally, in three patients a cause could not be foscarnet were effective in the treatment of definite or probable CMV PR. The present study confirms that acute lumbosacral PR in HIV-infected patients must be considered a syndrome with different causes. CMV and M. tuberculosis infections were the most frequent causative agents in our series (41% and 18% of the cases, respectively). Early empirical therapy is often necessary as definite diagnosis may be delayed or never achieved. Our experience suggests that, at least in our milieu, anti-tuberculous drugs should be considered in some cases together with ganciclovir or foscarnet in the empirical therapy for PR in HIV-infected patients.

Acute Disease↗

Inflammatory sensory polyradiculopathy and reactivated peripheral nervous system infection in a genital herpes model.

To study effects of herpesvirus reactivation on the nervous system, mice with latent genital herpes simplex virus type 2 (HSV-2) infections were immunosuppressed. Reactivated infection was detected by virus isolation or by antigen screening in histological sections of spines that contained cords, roots, and dorsal root ganglia. In ganglia, viral antigen was restricted to 2 distinct groups at T9-L2, and L6-S2 levels. In some ganglia, antigen was found in up to 4% of non-contiguous neurons, in their axons, and in endoneurial and satellite cells. Nerve roots distal to ganglia contained a few antigen-positive endoneurial cells and axons, but convincing antigen was not seen in proximal roots. Rare foci of anterior horn cells in the lower cord were the only central sites found to contain antigen. Comparison of antigen-containing ganglionic neuron counts to vaginal culture data indicate that peripheral virus shedding may depend on the number of neurons with reactivated infection. In non-immunosuppressed, latently infected mice, virus recovery was restricted to ganglionic explants from lower thoracic and lumbosacral regions; these ganglia contained no infectious virus in homogenates and no detectable antigen. These mice had an inflammatory polyradiculopathy in a similar distribution to virus found in latent and reactivated infection. The data show that genital HSV-2 infection can result in more extensive ganglionic latency and peripheral nervous system disease than has previously been recognized. Immunosuppression leads to reactivation and widespread, anatomically restricted antigen expression in many ganglia, consistent with the innervation of the genitourinary tract. Decalcified spine preparations provide a sensitive and simple way to detect virus reactivations and disease in these neural tissues.

Animals↗