Lymphedema: strategies for management.

An important sequela of cancer treatment is lymphedema. Management of this condition must be based on the physiologic functioning of the lymphatic system and tailored to the individual patient's presentation of the disease. Early diagnosis and treatment are essential to prevent worsening of the condition and to help assuage the psychologic impact of the disease. A review of the normal and impaired lymphatic system is presented in this article. Current assessment and treatment options are described, including education of patients in precautions, positioning, exercise, compression garments and bandages, pneumatic pumps, and lymphatic massage. Also included is a discussion of the psychologic impact of the disease as well as management of psychologic symptoms. Various strategies for management of the physical aspects of lymphedema are available. Studies have shown that use of physical treatments such as exercise, compression, and lymphatic massage are effective in reducing the amount of swelling in affected limbs. The specific type, amount, and combination of these treatments continues to be debated. The development of lymphedema is commonly associated with significant psychologic distress that can impact on compliance with physical treatments. Because there is no consensus as to the optimal treatment approach for management of lymphedema, additional research must ensue to determine the efficacy of existing treatments and to develop new management techniques. Clinicians must be attuned to the signs and symptoms of lymphedema in order to make prompt referrals for treatment.

Paraneoplastic cerebellar degeneration as the first evidence of cancer: a case report.

Paraneoplastic cerebellar degeneration (PCD) is the most frequently seen paraneoplastic syndrome affecting the brain. PCD is most commonly associated with cancers of the ovary, breast, and lung. The anti-Purkinje cell antibodies (anti-Yo) that specifically damage the Purkinje cells of the cerebellum are found in the patient's serum and cerebrospinal fluid. The typical presentation of PCD includes limb and truncal ataxia, often along with dysarthria. This report describes the case of a 47-year-old woman without significant medical history who developed new onset of unsteady gait, headache, and vertigo. The imaging studies suggested rhombencephalitis. The patient initially responded to corticosteroid treatment. Unfortunately, her gait ataxia worsened and she developed dysarthria, neither of which responded to increasing dosages of corticosteroids. Extensive imaging studies showed no evidence of tumor, but the patient was found to have positive anti-Yo antibodies and elevated cancer antigen 125 (CA-125). Pathology results from exploratory laparotomy revealed stage III C adenocarcinoma of the ovary. This case demonstrates that PCD may be the presenting symptom of an occult malignancy. The pathogenesis, diagnosis, and treatment of PCD, and its rehabilitation implications, are reviewed.

Lymphedema of the limb. An overview of treatment options.

In developed countries, prevalence is increasing of malignancy-related secondary lymphedema, usually resulting from lymph node resection or irradiation. Untreated lymphedema usually increases over time, and the physical and psychological sequelae in even mild cases demand appropriate diagnosis and treatment. Patients should be referred to a clinic familiar with the treatment of lymphedema, and therapy should be customized to optimize patient compliance. Treatment programs do exist, but clinicians who are unfamiliar with them may find it difficult to determine the best program for individual patients. Currently, most lymphedema clinics favor the use of MLT-CPT for significant lymphedema.

Spinal accessory nerve palsy as a cause of pain after whiplash injury: case report.

Spinal accessory nerve injury is most commonly reported following surgery in and around the posterior cervical triangle. Pain, impaired ability to raise the ipsilateral shoulder, and scapular winging on abduction of the arm are the most frequently noted clinical manifestations. We report the case of a collegiate swimmer who developed left-sided neck and shoulder pain secondary to a spinal accessory nerve palsy (SANP) after a "whiplash injury," which we believe to be the first such reported case in the English language literature. We review the clinical manifestations, diagnostic pitfalls, and therapeutic approaches to SANP. A high index of suspicion for SANP following whiplash-type injury will ensure its earlier detection and treatment and improve the chances of a better functional outcome.

Antibodies against the calcium channel beta-subunit in Lambert-Eaton myasthenic syndrome.

The sera of patients with Lambert-Eaton myasthenic syndrome (LEMS) contain autoantibodies against several extracellular and intracellular components of the voltage-gated calcium channel (VGCC)/synaptic vesicle release complex. An example of the latter are anti-beta-subunit antibodies (anti-MysB antibodies). We constructed a full-length cDNA clone of a human VGCC beta-subunit to produce purified beta-subunit fusion protein (MysB protein). Using this protein, we demonstrated that anti-beta-subunit antibodies are present in the sera of 23% of LEMS patients and only, in low titer, in 2% of small cell lung cancer patients without LEMS. The presence of anti-beta-subunit antibodies was closely associated with high titers of P/Q- and N-type VGCC antibodies. Immunization of rats with the purified MysB protein induced high antibody titers, but no signs of neurologic dysfunction were found. We conclude that anti-beta-subunit antibodies are not likely to interfere with ion channel function, but their presence could explain the cross-reactivity of LEMS sera with several subtypes of VGCCs and the lack of correlation between anti-VGCC antibody titer and clinical severity of disease.

Multiple sclerosis presenting as a spinal cord tumor.

Multiple sclerosis, a disorder of central nervous system demyelination, is a leading cause of disability in young people. Lesions of the spinal cord are usually less than two vertebral body segments long, peripherally located, and found in the cervical region. A 30-year-old woman had a 2-month history of back pain, urinary incontinence, and bilateral lower extremity weakness. Magnetic resonance imaging (MRI) of the spine showed an intramedullary spinal cord tumor from T4 to T8 with an intramedullary cyst from T1 to T4. After thoracic decompressive surgery, findings from biopsy of the cord lesion were consistent with multiple sclerosis. Postoperatively, the patient required an intensive rehabilitation program. This is the first reported case of histopathologically confirmed spinal cord demyelination presenting as an intramedullary thoracic cord tumor. Physiatrists should be alerted that demyelinating disease can mimic a spinal cord tumor, even on MRI, and must be considered in the differential diagnosis of a symptomatic spinal cord mass.

Increased phantom limb pain as an initial symptom of spinal-neoplasia.

Phantom limb pain is a common sequela of amputation. Studies suggest that over time, there is a decrease in frequency and intensity of phantom pain. Persistently increased phantom pain has been seen in benign lesions affecting the peripheral and central nervous system. We present a 74-year-old woman who had a left above-knee amputation for leiomyosarcoma of the foot 24 years previously. She had been free of disease and ambulated independently until 1 month before hospitalization, when she noted increasing pain in her phantom foot. At the time of admission, she had developed increasing low back pain and was diagnosed with adenocarcinoma of unknown primary. Work-up confirmed involvement of the L4 vertebral body with epidural and paraspinal disease. We believe this is the first reported case of worsening phantom limb pain resulting from a spinal metastasis. We review the literature on the potential implications of increased phantom pain.

Tethered thoracic cord resulting from spinal cord herniation.

Tethered cord syndrome (TCS) usually involves tethering of the lower cord at the conus medullaris from dural abnormalities, but may occur after spinal cord herniation. A tethered thoracic spinal cord is rare. We present an unusual case of a 30-year-old woman with a history of myelopathy presumed to be secondary to T6 cord compression resulting from T6-T8 arachnoid cyst. She continued to deteriorate after partial excision of the cyst. Repeat magnetic resonance imaging suggested recurrence of the presumed arachnoid cyst with cord compression and showed tethering at T6-T8. Surgical exploration revealed myelocele with cord herniation through the anterior thoracic dura. Pathologic diagnosis showed neural tissue with gliosis. After physical therapy treatments, the patient had increased lower extremity strength, ambulated with a cane, and regained some bladder control. Progressive myelopathy may represent tethering of the cord resulting from cord herniation. Early recognition of TCS, even in patients with minimal neurologic deficits, could prevent progressive disability.

Upper limb reflex sympathetic dystrophy associated with occult malignancy.

Reflex sympathetic dystrophy, characterized by pain, swelling, vasomotor instability, and trophic changes in an extremity, has been infrequently described in patients with occult malignancy. Two cases of reflex sympathetic dystrophy associated with local tumor involvement are reported. Both patients had a history of cancer in clinical remission. Despite aggressive physical therapy measures, the patients' symptoms persisted. Workup of the first patient found an apical paravertebral mass in the lung; biopsy revealed recurrent breast carcinoma. In the second case, workup found an axillary mass contiguous with the lower brachial plexus. Biopsy revealed lymphoma, a second primary malignancy. In both cases, medical treatment of the tumor was instituted, with consequent improvement of hand and shoulder function. Both patients required prolonged hospitalization and multiple procedures that might have been avoided if malignancy had been suspected. Spontaneous development of reflex sympathetic dystrophy in patients with a history of cancer should alert the physician to the possibility of occult malignancy.

Balint's syndrome arising from bilateral posterior cortical atrophy or infarction: rehabilitation strategies and their limitation.

Balint's syndrome is characterized by faulty visual scanning, dysmetria secondary to a visual perceptual deficit, and an inability to recognize more than one object at a time. We report three cases of Balint's syndrome and the individualized rehabilitation they received. One patient developed symptoms of Balint's syndrome caused by bilateral occipitoparietal infarcts. In the two other cases the symptoms arose secondary to posterior cortical atrophy, a slowly progressive dementia with alexia and agraphia. All three patients initially responded to a multicontext treatment approach with intensive verbal cueing and organizational strategies with subsequent improvement in visual recognition, reaching and scanning. Continued deterioration from posterior cortical atrophy or a second infarction resulted in worsening dementia in two patients. Cognitive remediation was required as visual perceptual rehabilitation became ineffective. Clinicians should be familiar with Balint's syndrome and its various aetiologies. Lack of awareness of this syndrome may lead to a misdiagnosis and resulting inappropriate or inadequate treatment.

Pelvic insufficiency fractures after irradiation: diagnosis, management, and rehabilitation.

Insufficiency fractures of the pelvis are commonly overlooked as causes of severe hip and low back pain. Predisposing factors include postmenopausal osteoporosis, corticosteroids, and local irradiation. Differential diagnosis includes metastatic disease to bone. We present the case of a 65-year-old woman who had a two-month history of low back pain and left groin pain. Her medical history included osteoporosis and endometrial cancer that was treated with radiation therapy to the pelvis 1 year prior to presentation. Despite bed rest, analgesics, and therapeutic modalities, her pain remained intractable and prevented ambulation. Plain radiographs showed no fracture. Computed tomography (CT) and magnetic resonance imaging showed fractures of the pelvis but were suggestive of malignancy. CT-guided bone biopsy was consistent with radiation osteonecrosis. After diagnosis and continued therapy, the patient progressed to ambulation with moderate discomfort. Failure to diagnose insufficiency fractures could lead to further pelvic irradiation, compromising already weakened bones and causing prolonged disability.

Lumbosacral radiculopathy secondary to L5 metastatic melanoma of unknown primary.

Lumbosacral radiculopathy secondary to spinal malignancy is rare. Spinal melanoma without cutaneous manifestations is even more unusual. We present the case of a 45-year-old physician with a history of degenerative disease of lumbar spine and chronic back pain who presented with increasing back pain with right radiculopathy despite conservative management for 6 months. Computed tomography showed a destructive lesion of the L5 vertebral body. Results from a biopsy guided by computed tomography suggested neoplasm of unknown origin. The patient underwent anterior vertebrectomy with instrumentation and fusion. Surgical pathology study results showed metastatic melanoma of unknown primary. The patient had no cutaneous manifestation of the disease. This is the first reported case of radiculopathy due to melanoma metastatic to the lumbar spine. In view of the atypical presentation of our patient's malignancy, we emphasize the importance of including malignancy of lumbar spine in the differential diagnosis of progressive lower back pain with radiculopathy.

Neurosarcoidosis of the conus medullaris and cauda equina presenting as paraparesis: case report and literature review.

Sarcoidosis is a multi-system, non-caseating granulomatous disease of unknown etiology. Although spinal cord involvement is rare, it may cause severe neurological complications. We report the case of a 52-year-old woman who developed low back pain and a progressive flaccid paraparesis whose investigations revealed pulmonary and spinal sarcoidosis. To our knowledge, this is the first reported case of sarcoidosis of the conus medullaris and cauda equina diagnosed by an unusual enhancement pattern observed on magnetic resonance imaging with gadolinium. Biopsy of the lumbar nerve roots and of the lung revealed a non-caseating granuloma. Treatment with steroids and intensive rehabilitation resulted in remarkable functional improvement. Although rare, sarcoidosis of the conus medullaris and cauda equina should be considered in the differential diagnosis of flaccid paraparesis, particularly in patients with non-diagnostic evaluations.

Severe polyneuropathy: initial manifestation of Castleman's disease associated with POEMS syndrome.

Castleman's disease is a rare clinicopathological entity characterized by multicentric angiofollicular lymph node hyperplasia. Peripheral neuropathy has infrequently been described in patients with Castleman's disease. A patient is described who presented initially with severe painful sensorimotor polyneuropathy of his lower limbs diagnosed as a plasma cell variant of Castleman's disease associated with features of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin change). The patient was treated with plasmapheresis, immunosuppressive agents, and intensive rehabilitation. His functional status improved from being wheelchair bound to ambulating independently with crutches. Clinicians should be alert to the relationship of Castleman's disease and mixed polyneuropathy because physical improvement is possible with treatment. Also, the evaluation of patients presenting with peripheral neuropathy of unknown etiology and lymphadenopathy should include lymph node biopsy to rule out Castleman's disease.

Complications associated with intermittent pneumatic compression.

The intermittent pneumatic compression device (IPCD) is prophylaxis for prevention of deep-venous thrombosis (DVT). This pneumatic leg sleeve has been used extensively in high-risk surgical patients, without complication. We describe two cases, one with peroneal neuropathy and the other with compartment syndrome, associated with IPCD use during surgery. Case 1 involves a patient with pancreatic cancer and weight loss who developed bilateral peroneal nerve palsies during surgery. Case 2 involves a patient with bladder cancer who developed lower leg compartment syndrome during prolonged surgery in the lithotomy position. These cases are unusual for several reasons. First, patients wearing IPCDs during surgery are at increased risk of neurovascular compression. Second, significant weight loss may predispose the peroneal nerve to injury from intermittent compression garments. Third, patients undergoing surgery in the lithotomy position are at risk of compartment syndrome. Therefore, physicians may wish to use another method of DVT prophylaxis in surgical patients with cancer or significant weight loss, or those who are undergoing procedures in the lithotomy position.

Lower extremity weakness as the initial manifestation of lung cancer.

A 55-year-old woman had progressive lower extremity weakness. She had an 80-pack-per-year history of cigarette smoking. Electromyographic findings were consistent with the Lambert-Eaton myasthenic syndrome. Despite a normal chest x-ray, normal laboratory studies, and negative bronchoscopy, investigation for lung cancer was pursued. The final thoracotomy and pathology reports indicated a diagnosis of oat cell carcinoma. After treatment with chemotherapy and radiation therapy, clinical and electrophysiologic findings have resolved. Two years after diagnosis, the patient remains in good health without evidence of recurrence.