the area of numbness had regressed significantly, paresthesias had resolved, and the LFCN SNAP had recovered (Fig. 1C, lower traces). A recent population-based study indicated that MP is associated with obesity, advanced age, and diabetes mellitus.2 The authors inferred a strong linear correlation between BMI and the occurrence of MP, and they could not find any underweight patients among the 262 with MP. Physicians often do not search for other causes of MP in diabetic patients. However, the patient described here demonstrates that there may be value in doing so if the patient is underweight and has abdominal pain on the side of the neurological symptoms. Kyung-Ha Noh, MD Dae-Seong Kim, MD, PhD Jin-Hong Shin, MD, PhD Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, Republic of Korea 1. Wartenberg R. Meralgia paresthetica. Neurology 1956;6:560–562.
2. Parisi TJ, Mandrekar J, Dyck PJ, Klein CJ. Meralgia paresthetica: relation to obesity, advanced age, and diabetes mellitus. Neurology 2011; 77:1538–1542.
Published online 11 May 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24701
--------------------------------------------------------PARANEOPLASTIC MYELONEUROPATHY IN A MAN WITH BREAST CANCER We report a 75-year-old man with a 2-year history of progressive lower extremity weakness associated with stiffness and numbness that limited ambulation. He subsequently developed hand numbness and weakness, bowel and bladder urgency, and progressive right-eye visual loss secondary to non-arteritic anterior ischemic optic neuropathy. His weight decreased by 30 pounds over a year. Physical examination revealed a cachectic, wheelchair-bound man. A hard
FIGURE 1. (A) Sagittal view. Posterior cord T2 intense signal extending throughout the cervical cord. (B) Axial view. The signal mainly involves the dorsal columns. (C) Follow-up MRI of the cervical spine after >6 months of cancer treatment showing improvement of the T2 signal. Letters to the Editor
MUSCLE & NERVE
October 2015
685
mass in the right breast, approximately 4 cm in diameter, was noted. Neurologically, mental status was normal. He had binocular visual loss; counting fingers in the right eye and 20/100 in the left. The lower extremities were spastic. Strength based on the Medical Research Council scale was 4/5 in the hands and 5/5 in proximal upper limbs. Lower limb strength was 2/5 proximally and 3/5 distally. Reflexes were hyperactive except for absent ankle jerks. Plantar responses were upgoing bilaterally. Sensory examination showed loss of vibratory sensation at the toes and ankles, absent proprioception at the toes, and decreased pinprick sensation in the distal lower limbs. Tests for vitamin B12, erythrocyte sedimentation rate (ESR), complete blood count (CBC), vitamin E, thyroidstimulating hormone (TSH), copper, methyl malonic acid, and homocysteine were normal. Tests for syphilis, HIV, and human T-cell lymphotropic virus (HTLV-1) were negative. Immunofixation revealed an IgM monoclonal gammopathy in serum and urine. An oncologist determined that this was of uncertain significance. Paraneoplastic [anti-glial neuronal antibody-1 (AGNA-1); anti-neuronal nuclear antibody (ANNA)-1, -2, and -3; voltage-gated calcium channel (VGCC) N and P/Q types; Purkinje cell cytoplasmic antibody (PCA)-1, -2, and -Tr types; acetylcholine receptor (AChR)-ganglionic and binding; voltage-gated potassium channel (VGKC); collapsing-response mediator protein-5 (CRMP-5)–IgG; amphiphysin; striated muscle] and neuromyelitis optica (NMO) autoantibodies were normal. Cerebrospinal fluid was normal for cell count, protein, cytology, varicella zoster virus polymerase chain reaction (PCR), cytomegalovirus culture, viral culture, fungal culture, anti–Borrelia burgdorferi IgG antibodies, oligoclonal bands, and paraneoplastic (similar to serum) autoantibodies. Skeletal survey X-rays were normal. Cervical spine MRI revealed abnormal T2 signal located primarily in the posterior columns (Fig. 1A and B). Nerve conduction studies are listed in Table S1 (see Supplementary Material, available online). Needle EMG revealed fibrillation potentials and positive sharp waves in the left tibialis anterior, gastrocnemius, vastus lateralis, biceps femoris, and L5–S1 paraspinal muscles, but it was normal in an upper limb, consistent with a lumbosacral polyradiculoneuropathy. Somatosensory-evoked potential testing revealed absent median N13 and N20 potentials and absent tibial P37 potentials bilaterally. On visual-evoked potential testing, P100 potentials were absent bilaterally. Sural nerve biopsy demonstrated mild axonal loss without evidence of amyloid deposition, inflammation, or vasculitis. The patient underwent right breast mass excision; biopsy showed mucinous (colloid) carcinoma. Fluorodeoxyglucose–positron emission tomography (FDG-PET) imaging revealed no dissemination. He received adjunctive tamoxifen treatment. Serial neurological examinations showed improved strength beginning 3 months after treatment. He was no longer wheelchair-bound and was able to ambulate with a walker. Proximal lower limb weakness improved to at least 3/5. There was radiological improvement in the T2 signal (Fig. 1C) after 6 months. This case is most consistent with myeloneuropathy, and the clinical and radiological improvement after treatment of the carcinoma strongly suggests a paraneoplastic 686
Letters to the Editor
etiology. Although a paraneoplastic cause for myelopathy has been reported in gastrointestinal cancers attributed to pernicious anemia and vitamin B12 malabsorption,1,2 this patient differs because there was no evidence of pernicious anemia or low vitamin B12 level. In conclusion, a paraneoplastic etiology of myeloneuropathy should be considered in patients without evidence for other causes. Mohammad Alsharabati, MD Shin J. Oh, MD Neurology Department, Neuromuscular Disease Division, University of Alabama at Birmingham Hospital, Birmingham, Alabama 1. Hirata A, Nomoto N, Konno S, Nakazora H, Sugimoto H, Nemoto H, et al. Subacute combined degeneration of the spinal cord concomitant with gastric cancer. Intern Med 2006;45:875–877. 2. Madrid A, Casado JL, P erez-Errazquin F, Vazquez A, Garz on F, et al. Subacute combined degeneration of the spinal cord: manifestations of esophageal epidermoid carcinoma. Rev Neurol 1997;25:1098– 1101.
Additional Supporting Information may be found in the online version of this article. Published online 14 May 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24706
--------------------------------------------------------PHARYNGEAL–CERVICAL–BRACHIAL/ MILLER–FISHER OVERLAP: A POSSIBLE CENTRAL VARIANT After recovering from a diarrheal illness 1 week earlier, a 52-year-old woman presented with dysarthria, tongue numbness, and clumsiness. Neurological examination revealed weakness in finger abduction [grade 4/5 on Medical Research Council (MRC) scale], hypesthesia to light touch in the face, absent proprioception, and ataxia. Over the next 3 days, she developed global areflexia, gaze limitation in all directions, bilateral facial and tongue weakness, and dysphagia, with an absent gag reflex. From day 8, the power of shoulder abduction and elbow flexion were 2/5 (MRC scale) bilaterally, whereas neck flexion, finger abduction, and lower limb movements were 4/5 bilaterally. Her reflexes progressed to symmetrical hyperreflexia on day 12. An edrophonium test was negative. Routine biochemical tests, cerebrospinal fluid results, and brain and spinal imaging were normal. Antinuclear, anti-neutrophil cytoplasmic, and anti-neuronal antibodies, as well as rheumatoid factor, extractable nuclear antigens, and human immunodeficiency virus antibodies, were negative. Thyroid function testing and vitamin B12 and folate levels were normal. Antiacetylcholine receptor antibody was negative. Serum anti-cytomegalovirus IgG and IgM antibodies were positive. IgG antibodies against GQ1b were strongly positive. Nerve conduction study on day 6 showed minimal changes. On day 18 bilateral sural sensory nerve action potentials were absent, and motor studies were normal (Table 1). Blink reflex and facial studies on day 21 showed bilaterally absent R1 and R2 responses and decreased motor amplitudes in the nasalis bilaterally MUSCLE & NERVE
October 2015
2. Parisi TJ, Mandrekar J, Dyck PJ, Klein CJ. Meralgia paresthetica: relation to obesity, advanced age, and diabetes mellitus. Neurology 2011; 77:1538–1542.
Published online 11 May 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24701
--------------------------------------------------------PARANEOPLASTIC MYELONEUROPATHY IN A MAN WITH BREAST CANCER We report a 75-year-old man with a 2-year history of progressive lower extremity weakness associated with stiffness and numbness that limited ambulation. He subsequently developed hand numbness and weakness, bowel and bladder urgency, and progressive right-eye visual loss secondary to non-arteritic anterior ischemic optic neuropathy. His weight decreased by 30 pounds over a year. Physical examination revealed a cachectic, wheelchair-bound man. A hard
FIGURE 1. (A) Sagittal view. Posterior cord T2 intense signal extending throughout the cervical cord. (B) Axial view. The signal mainly involves the dorsal columns. (C) Follow-up MRI of the cervical spine after >6 months of cancer treatment showing improvement of the T2 signal. Letters to the Editor
MUSCLE & NERVE
October 2015
685
mass in the right breast, approximately 4 cm in diameter, was noted. Neurologically, mental status was normal. He had binocular visual loss; counting fingers in the right eye and 20/100 in the left. The lower extremities were spastic. Strength based on the Medical Research Council scale was 4/5 in the hands and 5/5 in proximal upper limbs. Lower limb strength was 2/5 proximally and 3/5 distally. Reflexes were hyperactive except for absent ankle jerks. Plantar responses were upgoing bilaterally. Sensory examination showed loss of vibratory sensation at the toes and ankles, absent proprioception at the toes, and decreased pinprick sensation in the distal lower limbs. Tests for vitamin B12, erythrocyte sedimentation rate (ESR), complete blood count (CBC), vitamin E, thyroidstimulating hormone (TSH), copper, methyl malonic acid, and homocysteine were normal. Tests for syphilis, HIV, and human T-cell lymphotropic virus (HTLV-1) were negative. Immunofixation revealed an IgM monoclonal gammopathy in serum and urine. An oncologist determined that this was of uncertain significance. Paraneoplastic [anti-glial neuronal antibody-1 (AGNA-1); anti-neuronal nuclear antibody (ANNA)-1, -2, and -3; voltage-gated calcium channel (VGCC) N and P/Q types; Purkinje cell cytoplasmic antibody (PCA)-1, -2, and -Tr types; acetylcholine receptor (AChR)-ganglionic and binding; voltage-gated potassium channel (VGKC); collapsing-response mediator protein-5 (CRMP-5)–IgG; amphiphysin; striated muscle] and neuromyelitis optica (NMO) autoantibodies were normal. Cerebrospinal fluid was normal for cell count, protein, cytology, varicella zoster virus polymerase chain reaction (PCR), cytomegalovirus culture, viral culture, fungal culture, anti–Borrelia burgdorferi IgG antibodies, oligoclonal bands, and paraneoplastic (similar to serum) autoantibodies. Skeletal survey X-rays were normal. Cervical spine MRI revealed abnormal T2 signal located primarily in the posterior columns (Fig. 1A and B). Nerve conduction studies are listed in Table S1 (see Supplementary Material, available online). Needle EMG revealed fibrillation potentials and positive sharp waves in the left tibialis anterior, gastrocnemius, vastus lateralis, biceps femoris, and L5–S1 paraspinal muscles, but it was normal in an upper limb, consistent with a lumbosacral polyradiculoneuropathy. Somatosensory-evoked potential testing revealed absent median N13 and N20 potentials and absent tibial P37 potentials bilaterally. On visual-evoked potential testing, P100 potentials were absent bilaterally. Sural nerve biopsy demonstrated mild axonal loss without evidence of amyloid deposition, inflammation, or vasculitis. The patient underwent right breast mass excision; biopsy showed mucinous (colloid) carcinoma. Fluorodeoxyglucose–positron emission tomography (FDG-PET) imaging revealed no dissemination. He received adjunctive tamoxifen treatment. Serial neurological examinations showed improved strength beginning 3 months after treatment. He was no longer wheelchair-bound and was able to ambulate with a walker. Proximal lower limb weakness improved to at least 3/5. There was radiological improvement in the T2 signal (Fig. 1C) after 6 months. This case is most consistent with myeloneuropathy, and the clinical and radiological improvement after treatment of the carcinoma strongly suggests a paraneoplastic 686
Letters to the Editor
etiology. Although a paraneoplastic cause for myelopathy has been reported in gastrointestinal cancers attributed to pernicious anemia and vitamin B12 malabsorption,1,2 this patient differs because there was no evidence of pernicious anemia or low vitamin B12 level. In conclusion, a paraneoplastic etiology of myeloneuropathy should be considered in patients without evidence for other causes. Mohammad Alsharabati, MD Shin J. Oh, MD Neurology Department, Neuromuscular Disease Division, University of Alabama at Birmingham Hospital, Birmingham, Alabama 1. Hirata A, Nomoto N, Konno S, Nakazora H, Sugimoto H, Nemoto H, et al. Subacute combined degeneration of the spinal cord concomitant with gastric cancer. Intern Med 2006;45:875–877. 2. Madrid A, Casado JL, P erez-Errazquin F, Vazquez A, Garz on F, et al. Subacute combined degeneration of the spinal cord: manifestations of esophageal epidermoid carcinoma. Rev Neurol 1997;25:1098– 1101.
Additional Supporting Information may be found in the online version of this article. Published online 14 May 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24706
--------------------------------------------------------PHARYNGEAL–CERVICAL–BRACHIAL/ MILLER–FISHER OVERLAP: A POSSIBLE CENTRAL VARIANT After recovering from a diarrheal illness 1 week earlier, a 52-year-old woman presented with dysarthria, tongue numbness, and clumsiness. Neurological examination revealed weakness in finger abduction [grade 4/5 on Medical Research Council (MRC) scale], hypesthesia to light touch in the face, absent proprioception, and ataxia. Over the next 3 days, she developed global areflexia, gaze limitation in all directions, bilateral facial and tongue weakness, and dysphagia, with an absent gag reflex. From day 8, the power of shoulder abduction and elbow flexion were 2/5 (MRC scale) bilaterally, whereas neck flexion, finger abduction, and lower limb movements were 4/5 bilaterally. Her reflexes progressed to symmetrical hyperreflexia on day 12. An edrophonium test was negative. Routine biochemical tests, cerebrospinal fluid results, and brain and spinal imaging were normal. Antinuclear, anti-neutrophil cytoplasmic, and anti-neuronal antibodies, as well as rheumatoid factor, extractable nuclear antigens, and human immunodeficiency virus antibodies, were negative. Thyroid function testing and vitamin B12 and folate levels were normal. Antiacetylcholine receptor antibody was negative. Serum anti-cytomegalovirus IgG and IgM antibodies were positive. IgG antibodies against GQ1b were strongly positive. Nerve conduction study on day 6 showed minimal changes. On day 18 bilateral sural sensory nerve action potentials were absent, and motor studies were normal (Table 1). Blink reflex and facial studies on day 21 showed bilaterally absent R1 and R2 responses and decreased motor amplitudes in the nasalis bilaterally MUSCLE & NERVE
October 2015
