Abstract
Background/Aim: An 80-year-old male patient had complained of proximal paresis of the left leg, pain and sensory disturbances in the left abdomen, exanthema in the left lower abdomen, coprostasis, and severe abdominal pain, as well as a progressive deterioration of his general condition for weeks. The patient had already presented to three other medical centers. Colonoscopy and computed tomography of the abdomen could not explain the pronounced symptomatology. In addition, there was acute elevator paresis of the left leg and severe pruritic rash on both sides of the trunk. Case Report: At the Israelitisches Krankenhaus Hamburg (IKH), laboratory parameters of urine, stool, and blood, ultrasound, electrocardiogram, and transthoracic echocardiography diagnosis showed no abnormalities. Esophago-gastro-duodenoscopy revealed patchy erythema and moderately severe chronic low-activity Helicobacter-positive gastritis. Colonoscopically, two polyps were ablated. A neurological examination with magnetic resonance imaging and electroneurography also showed normal findings. Evidence of autoimmune or rheumatoid disease was also absent. Finally, analysis of the cerebrospinal fluid revealed a lympho-granulocytic cell count (32/3 lymphocytes, 21/3 granulocytes) and an elevated Borrelia-specific IgG index (Ai) of 20.82. This finding was confirmed by a complementary serological diagnosis, in which Borrelia-specific IgM and IgG antibodies were detected. In sum, Bannwart’s syndrome was assumed to be the cause of the neurological symptoms. The 21-day borreliosis therapy included doxycycline administration and analgesia with novaminsulfone and pregabalin as needed. Conclusion: A complex symptomatology of leg paresis, lower abdominal pain and sensory disturbances, exanthema, and coprostasis in combination with a long-lasting poor general condition were found to be the consequences of atypical neuroborreliosis.
Lyme borreliosis is an infectious disease transmitted by ticks. The disease was first described in 1977 as ‘Lyme arthritis’ in children with juvenile rheumatoid arthritis. The three most common Lyme pathogens belong to the genus Borrelia (B. burgdorferi, B. afzelii, B. garinii) (1). Lyme borreliosis is often asymptomatic but can also show a wide range of clinical symptoms. Depending on the pathogen, patients also show different degrees of severity. Clinical manifestation generally occurs in three phases, although they may occur independently. These include (i) early localized disease, (ii) early disseminated disease, and (iii) late disease. Early Lyme borreliosis occurs a few days to weeks after infection. Early localized disease includes erythema migrans and nonspecific findings, such as fever, headache, and general ill feeling with limb pain and fatigue (2, 3).
Neurologic complaints in the sense of Bannwarth syndrome and radicular pain may also occur. Early disseminated Lyme borreliosis usually occurs weeks to several months after the tick bite and is associated with neurologic symptoms (4). The classic triad of acute neurologic abnormalities consists of meningitis, cranial neuropathy, and motor or sensory radiculoneuropathy. In this regard, any of these findings may occur alone (5, 6). Late Lyme borreliosis is rare. Symptomatology develops insidiously and may be associated with a symptom-free latency period of months to years. For example, acrodermatitis chronica atrophicans Herxheimer (ACA) often does not appear for years. It typically presents as an inflammatory disease of the central nervous system (encephalomyelitis) with spastic-ataxic gait disturbance and bladder dysfunction. In rare cases, it may present as isolated meningitis (7).
Case Report
The 80-year-old male patient presented to the Israelitisches Krankenhaus Hamburg (IKH) in September 2022. In addition to proximal left leg paresis, he complained of pain and sensory disturbances in the area of the left abdomen. On admission to the hospital, the patient described a reduction in his general condition that had been present for 4-5 weeks. This was accompanied by exanthema in the left lower abdomen, coprostasis, and severe abdominal pain. Due to the abdominal pain, the patient had already presented to three other medical centers. During these visits, a colonoscopy and computed tomography scan of the abdomen were conducted, each of which showed coprostasis. Complete evaluation of the colon was not possible with residual contamination. However, a correlate for the pronounced symptomatology could not be found. A few days before presentation at the IKH, the pain exacerbated, so that an inpatient admission was made. At the same time, an acute elevator paresis of the left leg appeared, so that the patient, who had previously been completely self-sufficient, could no longer mobilize himself independently from a lying position. In addition, there was a severe pruritic rash on both sides of the trunk. The patient’s medical history included the following known disorders: Inguinal hernias bilaterally, distal symmetric polyneuropathy due to folic acid/vitamin B12 deficiency, vertebral body fracture, appendectomy, arterial hypertension, coronary 3-vessel disease with ST elevation myocardial infarction (STEMI) in 2017 and implantation of two stents in the right anterior descendens (RAD) and left anterior descendens (LAD). Due to comorbidities, regular use of acetylsalicylic acid, atorvastatin, bisoprolol, ramipril, pregabalin, and pantoprazole has been ongoing for years. There was no intake of new drugs in temporal relation to the mentioned complaints. At the time of presentation, the patient was living as a self-supporting pensioner and did not express any other complaints beyond those mentioned.
Differential diagnosis, investigations, and treatment. In the clinical examination, the patient presented an unremarkable general and normosomal nutritional condition with unremarkable abdominal findings, already subsiding skin rash still on the left side at the thoracoabdominal junction, and a left leg elevator paresis, strength grade 2/5. The cutaneous rash manifested as a patchy, pruritic redness with pustules, which was particularly prominent on the left flank and abdomen.
Standard laboratory chemistry examination of urine, feces and blood were without pathological findings. Cardiac examination including electrocardiogram and transthoracic echocardiography was unremarkable. Abdominal ultrasonography also revealed no pathologic findings. Esophago-gastroduodenoscopy revealed a patchy erythema and a moderately severe chronic, low-activity Helicobacter-positive gastritis (Figure 1). Colonoscopically, two polyps were ablated, which could be histologically classified as low-grade intraepithelial neoplasia and sessile serrated lesion (Figure 2).
Esophagogastroduodenoscopic examination of the stomach. Patchy erythema without edema on the entire surface of the stomach. Foldrielef and mural behavior are normal.
Endoscopic image of a broad-based polyp (approx. 10 cm) in the coecum (arrow; A). For removal by polypectomy snare (EPE), the polyp was injected with NaCl solution and positively lifted (arrow; B). Mucosal lesion after complete endoscopic removal of the polyp (macroscopic R0 resection). Subsequently, wound closure with a clip was carried out for prophylaxis (arrow; C).
Computed tomography morphology revealed inguinal hernias on both sides without signs of incarceration. However, correlation with abdominal pain and left leg elevator paresis was excluded. For further evaluation, a consultant neurologic co-evaluation was performed with subsequent unremarkable magnetic resonance imaging of the neurocranium, thoracic spine, and lumbar spine. Electroneurography also showed normal findings consistent with age. There was no serological evidence of autoimmune or rheumatoid disease. A skin biopsy of the rash with suspected herpes zoster infection histologically revealed only an uncharacteristic exanthema of unspecified etiology.
A cerebrospinal fluid (CSF) puncture was performed. The CSF findings revealed a lympho-granulocytic cell count (32/3 lymphocytes, 21/3 granulocytes) and an elevated Borrelia-specific IgG index (Ai) of 20.82. A supplementary serological diagnosis revealed Borrelia-specific IgM and IgG antibodies, consistent with the symptomatology of neuroborreliosis.
In summary, a Bannwart syndrome could be assumed as the cause of the neurological symptoms. Doxycycline therapy was initiated for 21 days with as-needed analgesia with novaminsulfone and a dose increase of pregabalin.
Outcome and follow-up. The social services department suggested follow-up treatment, which the patient also started. However, due to the enormous improvement in his physical and locomotor condition, this was terminated prematurely. Furthermore, an eradication therapy was performed for the gastritis and based on the histological colonoscopy findings, a recommendation was made to perform another colonoscopy in two years. After finishing the treatment, the patient was able to resume his home exercise program within two months and since then he has been on the same physical level as before.
Discussion
The procedure for diagnosing patients with suspected Lyme borreliosis depends on the stage of the disease and is also influenced by previous infections. Lyme borreliosis diagnosis is based on two pillars. The first is characterized by the typical clinical presentation as well as the typical course of the disease. The second consists of the corresponding laboratory tests (8). In the early stage and with a characteristic pattern of an erythema migrans, antibiotic therapy should not be delayed by further diagnostics (9). Serological tests are neither necessary nor recommended in such patients. However, if there is doubt about the cause of the skin lesion and empiric antimicrobial therapy is not being administered, serologic testing is reasonable. The test can be performed at the time of presentation, and if negative, a second test can be performed two to three weeks later. However, calculated antibiotic therapy is reasonable when symptoms appear. In CSF diagnostics, the clinical diagnosis of suspicion can be confirmed by the detection of Borrelia-specific antibodies in association with pleocytosis (10).
Due to the initially inconspicuous clinical findings as well as the inconspicuous laboratory values (urine, feces, blood) and apparative diagnostics (sonography), the abdominal pain was initially located in the context of constipation. This could have been promoted by the left leg elevator paresis during the prolonged immobilization. The pain improved under laxative measures. Constipation in seniors is common (11) and was successfully treated with stool-regulating measures (macrogol 3,350 + electrolytes 13.8 g bag, 2× daily). Because the leg elevator paresis did not improve and fluctuated in intensity during the inpatient stay, a comprehensive neurological diagnosis was performed. Finally, a diagnosis of neuroborreliosis was made, which initially manifested itself with autonomic symptoms (constipation) and severe abdominal pain, accompanied by a skin rash and a left-sided leg paresis that manifested itself only later. Abdominal pain and constipation are not classic symptoms of neuroborreliosis. The patient’s constipation is likely to be due to the autonomic involvement of the disease, as it improved rapidly with therapy with doxycycline and laxative measures. Early case reports described similar symptomatology (12).
The rash was characterized by a flat, itchy redness with pustules, especially on the left flank and abdomen. The skin rash manifested rather atypically for Lyme borreliosis. Classical for acute neuroborreliosis is meningeal or facial affection of the nerves (13). In the past, spastic paraparesis has also been observed as a neurological symptom (10). However, the patient’s leg paresis was not spastic at any time, but rather flaccid. However, the serological tests were finally conclusive for Lyme borreliosis, so that the abdominal pain and coprostasis were evaluated as autonomic, and the leg paresis as neurological involvement of neuroborreliosis.
Footnotes
Authors’ Contributions
Patient treatment: H.A., C.F., D.J., A.A., M.W., P.L., U.R. Images: H.A., M.B.S., A.A. Draft: H.A., C.F., D.J., A.A., M.W., P.L., U.R., M.B.S. Final manuscript: AH.A., M.B.S. Proofreading: H.A., C.F., D.J., A.A., M.W., P.L., U.R., M.B.S.
Conflicts of Interest
The Authors declare that they have no competing interests in relation to this study.
- Received October 2, 2023.
- Revision received December 16, 2023.
- Accepted December 18, 2023.
- Copyright © 2024 The Author(s). Published by the International Institute of Anticancer Research.
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY-NC-ND) 4.0 international license (https://creativecommons.org/licenses/by-nc-nd/4.0).
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