Perineuritis and ulcerative colitis

We describe the association of chronic polyneuropathy with ulcerative colitis. Electrophysiologic studies disclosed a severe neuropathy with both axonal and demyelinating features. The CSF protein content was 875 mg/dl. Sural nerve biopsy revealed perineuritis. Peripheral neuropathy with perineuritis may be an immunologically mediated extraintestinal manifestation of ulcerative colitis.     

A case of sensory perineuritis with Bowen disease]

A 60-year-old man was admitted because of subacutely progressive sensory disturbance characterized by fluctuating dysesthesia and light pain on the skin of his hands and feet. He exhibited mononeuritis multiplex, and a plaque with sloughing was observed on the left side of his back. Sural nerve biopsy revealed marked thickening of the perineurium with vascularization, and no necrotizing vasculitis. The pathological features of the nerve biopsy were compatible with sensory perineuritis. A biopsy from his skin lesion revealed Bowen's disease. There have been several reported cases of sensory perineuritis accompanied by malignant tumors. In these cases, immune dysfunctions were considered a common underlying cause in both diseases. This is the first reported case of sensory perineuritis associated with Bowen's disease.     

Case-of-the-month: perineuritis presenting as mononeuritis multiplex.

Perineuritis unassociated with other significant pathological changes is a rare finding. A patient is described with mild, non-insulin dependent diabetes mellitus who presented with the clinical picture of mononeuritis multiplex, and in whom perineuritis but no occlusive vascular disease was found on sural nerve biopsy. Treatment with prednisone and plasma exchange resulted in some improvement. We conclude that the focal perineuritis, rather than the diabetes, was responsible for the clinical picture mimicking mononeuritis multiplex. In conjunction with previous reports, this suggests that perineuritis may be a treatable neuropathy.     

Simultaneous occurrence of neuroretinitis and optic perineuritis in a single eye.

A 52-year-old woman presented with pain, decreased visual acuity, decreased color vision, and visual field loss in the OD. Fundus examination revealed optic disc edema and later a macular star figure consistent with neuroretinitis. Magnetic resonance imaging showed enhancement of the orbital optic nerve sheath and perineural fat suggestive of optic perineuritis. The patient was not treated. At 11 months, visual function and fundus examination had returned to normal. This unusual simultaneous occurrence of neuroretinitis and optic perineuritis suggests that these two entities may have a similar pathogenesis.     

Sensory perineuritis.

A case of sensory perineuritis is described, affecting individual cutaneous nerves in the extremities and with a chronic inflammatory exudate confined to the perineurium in a sural nerve biopsy. No cause was found. The condition slowly resolved on steroid treatment.     

Optic perineuritis: A further cause of visual loss and disc edema in children

Background: Optic perineuritis is a rare form of orbital inflammatory pseudotumor in which the specific target tissue is the optic nerve sheath. Patients are mainly represented by adult women. Differential diagnosis with demyelinating optic neuritis is essential in terms of prognosis and treatment. Case presentation: An 8-year-old Caucasian girl presented with bilateral loss of vision, disc edema, eye movement impairment, and diplopia. Brain MRI findings were suggestive of optic perineuritis. The patient received steroid pulse therapy followed by prolonged course of oral steroid therapy. The visual acuity recovered dramatically within 2 days. Two months later, a new MRI investigation was normal. No clinical relapse was observed at the follow-up. Discussion: We first report on a child affected by optic perineuritis. Our observation suggests that optic perineurits should be considered in the differential diagnosis of children presenting with visual loss and disc edema. An early and correct diagnosis may lead to an appropriate therapeutic approach with very good outcome.     

Perineuritis in mononeuritis multiplex with cryoglobulinemia

A case with mononeuritis multiplex associated with mixed cryoglobulinemia and mild persistent hepatitis is reported. Sural nerve biopsy specimens revealed thickening of the perineurium and vascular alterations with inflammatory cells infiltrating into the perineurium as well as the epineurium and endoneurium, thus having the features of perineuritis. Most myelinated and unmyelinated nerve fibers were lost. A therapeutic trial of corticosteroids decreased the serum titer of cryoglobulin without any improvement of clinical features.     

Apoptosis of perineurial cells in sensory perineuritis

A 71-year-old man presented a 6-month history of progressive paresthesia of all four limbs. Sural nerve biopsy specimens showed dense mononuclear infiltrates in the perineurium and subperineurium, indicating sensory perineuritis. One section revealed disruption of the perineurial barrier. Perforin and granzyme B were present in the infiltrates, and apoptosis of perineurial cells was indicated by a terminal deoxynucleotidyl-transferase-mediated dUTP-digoxigenin nick end-labeling (TUNEL) method. These findings suggest T cell-mediated apoptosis of the perineurium and nerve injury caused by perineurial damage. Received: 5 May 1999 / Revised, accepted: 12 July 19993     

Paraneoplastic Peripheral Neuropathy Associated With Anti-hu Antibodies. A Clinical And Electrophysiological Study Of 20 Patients

Peripheral neuropathy is a rare, yet treatable manifestation of sarcoidosis, a multisystem disorder characterized by the presence of non‐caseating granulomas that are seldom found in nerve biopsy specimens. In order to learn more about the subject, we reviewed our clinical and pathological findings in a series of 11 patients (six men and five women aged 26‐83 years) with symptomatic neuropathy associated with characteristic granulomas in nerve biopsy specimens. Only two patients were known to have sarcoidosis before the occurrence of the neuropathy. The neuropathy was focal or multifocal in six patients, including one with a multifocal neuropathy associated with conduction blocks, and one with a multifocal axonal motor deficit. Four patients had a distal symmetrical deficit and one patient had a Guillain‐Barré‐like syndrome with facial diplegia and respiratory failure. Serum angiotensin‐converting enzyme concentration was elevated in only two patients. Epineurial granulomas and perineuritis were present in all nerve specimens. The inflammatory infiltrates invaded the endoneurium, following connective tissue septae and blood vessels, in five patients. Multinucleated giant cells were found in eight patients and necrotizing vasculitis in seven. Inflammatory lesions were associated with variable, asymmetrical involvement of nerve fascicles and axon loss. A muscle specimen was sampled during the same procedure in 10 patients. It showed inflammatory infiltrates and granulomas in nine patients and necrotizing vasculitis in two. Immunolabeling showed a mixed inflammatory infiltrate of T cells (predominantly CD4+ cells) and macrophages, in keeping with a delayed hypersensitivity reaction. In addition to nerve involvement, all patients had at least one other tissue or organ affected, including muscle in nine patients, lungs and/or intrathoracic lymph nodes in eight, skin in three, arthritis in two, and peripheral lymph nodes, stomach and eye in one patient each. Most patients improved on corticosteroids. Two patients remain free of symptoms after 7 years. Severe side‐effects of long‐term treatment with corticosteroids occurred in two patients, leading to death in one. This study illustrates the wide range of clinical manifestations of sarcoid neuropathy and the frequent association of granulomatous inflammatory infiltrates with necrotizing vasculitis and with silent or symptomatic involvement of other organs.     

Nerve biopsy findings contribute to diagnosis of multiple mononeuropathy: 78% of findings support clinical diagnosis

Multiple mononeuropathy is an unusual form of peripheral neuropathy involving two or more nerve trunks. It is a syndrome with many different causes. We reviewed the clinical, electrophysiological and nerve biopsy findings of 14 patients who suffered from multiple mononeuropathy in our clinic between January 2009 and June 2013. Patients were diagnosed with vasculitic neuropathy(n = 6), perineuritis(n = 2), chronic inflammatory demyelinating polyradiculoneuropathy(n = 2) or Lewis-Sumner syndrome(n = 1) on the basis of clinical features, laboratory data, electrophysiological investigations and nerve biopsies. Two patients who were clinically diagnosed with vasculitic neuropathy and one patient who was clinically diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy were not confirmed by nerve biopsy. Nerve biopsies confirmed clinical diagnosis in 78.6% of the patients(11/14). Nerve biopsy pathological diagnosis is crucial to the etiological diagnosis of multiple mononeuropathy.     

Pseudotumor Cerebri in Lyme Disease: A Case Report and Literature Review

Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. Serum and cerebrospinal fluid Lyme antibodies were positive. Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.     

Carpal tunnel syndrome in Lyme borreliosis

Neurophysiologic evidence of median nerve entrapment in the carpal tunnel was present in 25% of patients with late Lyme borreliosis. Sixty-eight of 76 consecutive, prospectively studied patients with late Lyme underwent neurophysiologic testing. Nineteen reported intermittent hand paresthesias; 17 had neurophysiologically confirmed carpal tunnel syndrome. This was not consistently associated with clinically apparent wrist arthritis or with neurophysiologically evident peripheral neuropathy. We conclude that a significant proportion of patients with late Lyme borreliosis develop carpal tunnel syndrome.     

Multiple mononeuropathy and inflammatory syndrome manifested in Lyme disease

Meningo-radiculitis is the most common peripheral nerve system involvement of Lyme disease. We report the observation of a 73 year-old woman presenting a subacute multiple mononeuropathy and a severe inflammatory syndrome. Diagnosis of Lyme disease was confirmed by a lymphocytic meningitis with positive serologic results in the cerebrospinal fluid. Nerve biopsy showed inflammatory cells spreading along the endoneurium. This case report emphasizes that Lyme disease may present as a multiple mononeuropathy mimicking a vasculitic neuropathy.     

Eosinophilia-myalgia syndrome (L-tryptophan-associated neuromyopathy)

Histopathologic study of skeletal muscle biopsy in a patient with eosinophilia-myalgia syndrome following L-tryptophan use showed prominent lymphocytic perineuritis, neuritis, and perimysial fasciitis. The presence of perineuritis and neuritis provides a histopathologic basis for clinical features of neuropathy in eosinophilia-myalgia syndrome and occurred in conjunction with a fasciitis or interstitial myositis that was predominantly perimysial and focally endomysial.     

Acute Cerebellar Ataxia in a Pediatric Case of Lyme Disease and a Review of Literature

A broad range of neurologic disorders have been described in children with Lyme disease, of which peripheral facial nerve palsy and aseptic meningitis are among the most common. In contrast, there are few reports of cerebellar involvement in pediatric Lyme disease patients. We report the case of a 5-year-old girl seropositive for antibodies against the causative Lyme disease pathogen Borrelia burgdorferi presenting with severe acute cerebellar ataxia from the in southern coast of Anatolia (Mediterranean region).     

Frequency and clinical significance of Lyme seropositivity in patients with isolated optic neuritis

We evaluated antibody reactivity against Borrelia burgdorferi in 20 consecutive patients with newly diagnosed isolated optic neuritis who resided in a region endemic for Lyme disease. Four (20%) patients had positive serology. All three patients who had follow-up serologies showed rising convalescent levels of Borrelia-specific IgM. One patient refused lumbar puncture, one had normal CSF constituents except for an elevated Lyme antibody index, and two had CSF lymphocytic pleocytosis that remained unexplained after extensive evaluations for causes other than Lyme disease. We treated both patients who had CSF pleocytosis with intravenous ceftriaxone; the pleocytosis and optic nerve function improved. The other two patients received oral antibiotics and showed excellent recovery of visual acuity. We believe that serologic testing for Lyme disease is warranted for individuals with optic neuritis who reside in an endemic region, and patients with rising convalescent antibody levels or unexplained CSF pleocytosis should receive antibiotic treatment for Lyme disease.     

Lower cranial nerve involvement as the initial manifestation of Lyme borreliosis

Lyme disease or Lyme borreliosis is an infectious disease transmitted by ticks and caused by Borrelia burgdorferi. Being clinically different from Relapsing Fever it may cause an array of symptoms, specially cutaneous and neurological but also musculoskeletal and cardiac ones. Within the neurologic manifestations of Lyme disease the affectation of low cranial nerves is, to our knowledge, extremely infrequent. We present the clinical case of a 35 years old male whose initial symptoms were low cranial nerve dysfunction with a cerebrospinal fluid compatible with meningitis. Serology against Borrelia burgdorferi both in serum and cerebrospinal fluid was positive.     

Isolated neuritis of the sciatic nerve in a case of Lyme disease

Lyme disease is an infectious disease caused by the spirocheteBorrelia burgdorferi. The course of the disease is divided into three stages, the second of which may include various types of peripheral nervous system disturbances. We report the case of a patient with persistent deficits caused by the prevalent involvement of the sciatic nerve, confirmed by electrophysiological and neuropathological findings. The most significant bioptic results were axonal degeneration and perivascular inflammation. Damage to a single peripheral nerve as the dominant clinical expression during the course of Lyme disease is an unusual finding that has been rarely described in the literature.     

Lyme disease: cause of a treatable peripheral neuropathy

Peripheral nerve dysfunction was demonstrated in 36% of patients with late Lyme disease. Of 36 patients evaluated, 14 had prominent limb paresthesias. Thirteen of these had neurophysiologic evidence of peripheral neuropathy; neurologic examinations were normal in most. Repeat testing following treatment documented rapid improvement in 11 of 12. We conclude that this neuropathy, which is quite different from the infrequent peripheral nerve syndromes previously described in this illness, is commonly present in late Lyme disease. This neuropathy presents with intermittent paresthesias without significant deficits on clinical examination and is reversible with appropriate antibiotic treatment. Neurophysiologic testing provides a useful diagnostic tool and an important measure of response to treatment.