Takuhiro Yoshida

CPPD crystal deposition disease of the cervical spine: A common cause of acute neck pain encountered in the neurology department

BACKGROUNDnCalcium pyrophosphate dihydrate (CPPD) crystal deposition disease is one of the most common forms of crystal-associated arthropathy in the elderly. However, CPPD deposition on the cervical spine is less well known, and only a limited number of cases have been reported to date. Here, we report our recent clinical experience with CPPD crystal deposition disease of the cervical spine and describe the clinical features of this disease.nnnMETHODSnFourteen patients with clinically diagnosed CPPD crystal deposition disease of the cervical spine at our department during the period from January 2005 to December 2008 were analyzed retrospectively.nnnRESULTSnPatients ranged in age from 54 to 92 (mean+/-SD, 77.5+/-8.5). Chief symptoms of patients were acute posterior neck pain and fever. All patients had markedly restricted neck rotation. Serum CRP level was highly elevated in all patients (10.16+/-5.35 mg/dL). Computed tomography of the cervical spine demonstrated linear calcific deposits in the transverse ligament of atlas (crowned dens syndrome) in all patients. Calcific deposits were also found in other periodontoid structures and the ligamenta flava in some patients. Posterior neck pain, fever, and increased serum inflammatory indicators were relieved within 1 to 3 weeks by nonsteroidal antiinflammatory drugs (NSAIDs) or a combination of NSAIDs and prednisolone. Most of the patients were misdiagnosed as having other diseases before consultation.nnnCONCLUSIONSnCPPD crystal deposition disease of the cervical spine is one of the most common underrecognized causes of acute neck pain in the neurology department, especially in elderly patients.

Primary AL amyloid polyneuropathy successfully treated with high-dose melphalan followed by autologous stem cell transplantation

We report 2 patients with polyneuropathy associated with amyloid derived from light chains (AL) who were treated successfully with high‐dose melphalan followed by autologous peripheral blood stem cell transplantation (HDM/SCT). Neuropathic symptoms improved in conjunction with normalization of serum‐free light chains. In addition to amyloid deposits in tissues, an amyloidogenic light chain itself produced by abnormal plasma cells might be harmful to peripheral nerve function, and thus HDM/SCT seems to be a promising therapy for primary AL amyloid polyneuropathy. Muscle Nerve, 2010

Slaughtered aged cattle might be one dietary source exhibiting amyloid enhancing factor activity

It has been shown that experimental murine AA amyloidosis can be enhanced by dietary ingestion of amyloid fibrils, and it is known that systemic AA amyloidosis occasionally develops in aged cattle. In this study, we examined amyloid deposits in renal and muscular tissues simultaneously obtained from slaughtered aged cattle; from both tissues when affected, amyloid-enhancing activity was also investigated. On histopathology, renal amyloid deposition was seen in nine of the 293 cattle with no history of disease, and minute amyloid deposition in muscular tissue was detectable in one of these nine. All these amyloid deposits were immunohistochemically demonstrated to be AA. Extracts, which might contain amyloid fibril fractions, were isolated from renal and muscular tissues in five of these nine cattle. On SDS-PAGE and Western blot analysis, protein bands immunoreactive to anti-AA serum were detected in the kidney fractions obtained from four of the five latter cattle, but no bands were seen in the muscle fractions of any of the five cattle. Amyloid fibril fractions from two cattle were intravenously injected into group of seven experimentally designed mice for induction of AA amyloidosis. All seven mice injected with kidney fraction developed severe AA amyloidosis, whereas only one of the seven mice given muscle fraction showed slight amyloid deposition in the spleen. These data suggest that food products made from aged cattle possess amyloid-enhancing potential.

AL amyloidosis manifesting as systemic lymphadenopathy

We report three patients with AL amyloidosis manifesting as systemic lymphadenopathy, mainly in the cervical and supraclavicular regions. Histopathology of lymph nodes showed massive deposition of AL amyloid with no abnormal findings suggestive of lymphoproliferative disorders. Two of the patients were considered to be classifiable as primary systemic AL amyloidosis based on the presence of M-protein in serum and abnormal plasma cells or lymphoplasmacytoid cells in the bone marrow probably producing the precursor immunoglobulin, although no visceral organs were affected. The size of the involved lymph nodes in these two patients increased gradually, and one was treated with rituximab and VAD (vincristine, doxorubicin and dexamethasone) followed by high-dose melphalan with autologous peripheral blood stem cell transplantation (auto-PBSCT). The remaining patient showed no obvious change in the size of lymph nodes or detectable M-protein in serum. The prognosis of AL amyloidosis manifesting as lymphadenopathy is usually good as long as there are no hematological malignancies or rapid increases in the size of lymph nodes, but in cases of the systemic type, intensive chemotherapy, such as high-dose melphalan with auto-PBSCT, should be actively considered in order to avoid possible involvement of visceral organs.

Seronegative Sjögren syndrome with asymptomatic autoimmune sclerosing pancreatitis

We report two elderly patients with seronegative Sjögren syndrome who showed benign swelling of the pancreas on computed tomography. Immunostaining of the biopsied lip tissue or serum examination confirmed an increase in production of IgG4, leading to a diagnosis of autoimmune sclerosing pancreatitis (ASP) as a cause of the asymptomatic swelling of the pancreas. Sicca symptoms and ASP spontaneously improved in one patient, and the other responded well to oral prednisolone. Seronegative Sjögren syndrome and ASP can concurrently occur as a clinical manifestation of the IgG4-related systemic disorder, particularly in elderly subjects, and, in such a case, corticosteroid may be a potent therapeutic option.

Severe cranial nerve involvement in a patient with monoclonal anti-MAG/SGPG IgM antibody and localized hard palate amyloidosis

We report a patient with severe cranial polyneuropathy as well as sensory limb neuropathy. Biclonal serum IgM-kappa/IgM-lambda gammopathy was found and serum anti-myelin-associated glycoprotein (MAG)/sulfoglucuronyl paragloboside (SGPG) IgM antibody was also detected. Immunofluorescence analysis of a sural nerve biopsy specimen revealed binding of IgM and lambda-light chain on myelin sheaths. No amyloid deposition was detected in biopsied tissues except for the hard palate, suggesting that the amyloidosis was of the localized type and had no relation to the pathogenesis of cranial neuropathy. Our observations indicate that the anti-MAG/SGPG IgM antibody may be responsible for this patients cranial polyneuropathy, which is a rare manifestation in anti-MAG/SGPG-associated neuropathy.

Sepsis Caused by Newly Identified Capnocytophaga canis Following Cat Bites: C. canis Is the Third Candidate along with C. canimorsus and C. cynodegmi Causing Zoonotic Infection

Sepsis caused by a Capnocytophaga canis infection has only been rarely reported. A 67-year-old female with a past medical history of splenectomy was admitted to our hospital with fever and general malaise. She had been bitten by a cat. She showed disseminated intravascular coagulation and multi-organ failure because of severe sepsis. On blood culture, characteristic gram-negative fusiform rods were detected; therefore, a Capnocytophaga species infection was suspected. A nucleotide sequence analysis revealed the species to be C. canis, which was newly identified in 2016. C. canis may have low virulence in humans; however, C. canis with oxidase activity may cause severe zoonotic infection.

Motor Branch Biopsy of the Pronator Teres Muscle in a Patient with Painful Forearm Neuropathy

Histological evaluation of a peripheral nerve is often the final diagnostic work-up for a neuropathy of unknown origin, and a distal sensory nerve is usually biopsied. Here, we report the case of a female patient with painful unilateral neuropathy in the upper arm. According to the histological evaluation of the pronator teres motor branch, vasculitis seemed to be the most probable cause of the condition, and steroid therapy improved the patients symptoms. A biopsy of the motor branch of the pronator teres muscle nerve may be considered a valuable diagnostic option in selected cases with neuropathy affecting the upper limb, when performed in cooperation with neurologists and orthopedic surgeons.

Survey of epidemiology, clinical picture and current treatments for elderly-onset (≥ 65 years) patients with myasthenia gravis in Nagano Prefecture, Japan

Our previous population‐based study in 1982–2001, Nagano Prefecture, Japan, showed increased numbers of patients with elderly‐onset (≥ 65 years) myasthenia gravis.

Bortezomib–dexamethasone versus high-dose melphalan for Japanese patients with systemic light-chain (AL) amyloidosis: a retrospective single-center study

Bortezomib–dexamethasone (BD) and high-dose melphalan (HDM) are effective for systemic light-chain (AL) amyloidosis, but have not been compared in detail. We retrospectively investigated patients treated with BD or HDM at our center between September 2001 and June 2016. Among 234 patients, 20 were treated with BD and 30 received HDM. With the exception of age, transplant eligibility, and previous history of other chemotherapy, there were no significant differences in most background parameters between the two groups. Median age was higher (63.2 vs. 55.8, Pxa0=xa00.001), number of transplant-eligible patients was lower (60.0 vs. 96.7%, Pxa0=xa00.002), and number of previously treated patients was higher (35.0 vs. 0.0%, Pxa0<xa00.001) in the BD group. The BD group showed trends toward lower treatment-related mortality (5.0 vs. 10.0%, Pxa0=xa00.641), greater hematological response (partial response or better) (90.0 vs. 73.3%, Pxa0=xa00.279), higher complete response (60 vs. 50%, Pxa0=xa00.487), and similar survival with the HDM group (neither reached, Pxa0=xa00.705). In conclusion, BD was as effective and safe as HDM. Notably, BD achieved this outcome among patients with poorer clinical backgrounds compared with HDM.