Rosai-Dorfman disease presenting with multiple orbital and intracranial masses

Rosai-Dorfman disease is an idiopathic histocytic proliferative disorder typically characterized by painless cervical adenopathy, fever, and weight loss. Extranodal manifestations are responsible for presentation in approximately 25% of patients. Orbital involvement has been described in about 10% of patients. There have been only 16 reported cases of Rosai-Dorfman disease presenting with an intracranial mass. We report an unusual case of a patient presenting with bilateral orbital tumors as well as multiple intracranial masses. Clinical, magnetic resonance imaging, and histopathological features are discussed. Received: 13 October 1995 / Revised, accepted: 22 November 1995     

Multiple myeloma presenting with unilateral abducens and trigeminal nerve palsies

Petrous apex masses can manifest with neurologic symptoms due to their involvement of various structures, including cranial nerves (CN) V and VI. The differential diagnosis of petrous masses is broad and includes a variety of both non-neoplastic and neoplastic lesions. We report a rare case of multiple myeloma confined to the right petrous apex, presenting with ipsilateral abducens and trigeminal nerve palsies. A 63-year-old woman presented with a 6–8 week history of facial numbness and a 2 week history of diplopia, with examination showing right-sided facial hypoesthesia in the CN V1–V3 region and right-sided lateral rectus palsy. MRI of the brain showed a solitary 2.0 cm lesion confined to the right petrous apex involving the right cavernous internal carotid artery and Meckel’s cave. A transnasal biopsy showed a proliferation of plasmacytoid cells, which showed diffuse immunoreactivity with antibodies to CD138 and kappa, consistent with a plasma cell dyscrasia. A bone scan subsequently revealed multiple lytic bone lesions involving the skull, left humerus, bilateral femurs and possibly the L4 vertebral body. Bone marrow biopsy and serum laboratory results confirmed the diagnosis of kappa-type multiple myeloma. Although rare, multiple myeloma may initially present with petrous involvement and associated cranial nerve deficits.     

IgDK multiple myeloma presenting as unilateral proptosis

A 72-year-old woman presented with painful proptosis of the right eye and a large destructive tumour of the middle cranial fossa. A diagnosis of IgDK multiple myeloma was made, based on histopathologic and immunologic studies of the biopsy. Biochemistry and bone marrow examination further confirmed the myeloma as IgDK type. The clinical, radiological, and pathological findings are presented. The patient was treated with radiotherapy with satisfactory results.     

Multiple myeloma presenting as solitary mass in the posterior fossa

Intracranial plasma cell tumors are extremely rare and can either be solitary lesions or part of systemic multiple myeloma. We report a 42-year-old woman who presented with a posterior fossa mass and successfully underwent surgical resection, leading to the diagnosis of multiple myeloma. To our knowledge, this is the first reported case of multiple myeloma presenting as a posterior fossa mass lesion. This report highlights the importance of maintaining plasma cell tumor in the differential of intracranial mass with bony involvement. Furthermore, once the diagnosis is established, further work up is critical to evaluate for systemic disease.     

Palpable orbital subcutaneous masses in chronic inflammatory demyelinating polyneuropathy. MRI and neurophysiological study of multiple peripheral nerve swelling]

We report a 46-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom swelling of the first branch of the trigeminal nerves in the bilateral orbits were observed as subcutaneous masses in the upper eyelids. The disease developed when the patient was 33 years old, and weakness of the four limbs, double vision, unilateral hypoglossal neuroparalysis, and unilateral facial paralysis frequently occurred during the course of the illness. On nerve conduction studies, conduction block was detected in the motor nerves. Steroid therapy and immunoglobulin treatment improved the symptoms. At 43 years old, subcutaneous phymas were noted in the bilateral upper eyelids, and fat-suppressed MRI detected the phymas localized along the upper region of the superior straight muscle in the bilateral orbits, and the muscles were slightly compressed downward. The masses branched in the orbits, and were diagnosed as nerve swelling of the supraorbital nerve, the first branch of the trigeminal nerve. Fat-suppressed MRI also identified nerve swelling of the extracranial maxillary and mandibular nerves. However, the patient had no subjective sensory disturbance in the trigeminal nerve region. Blink reflex did not induce R1 and R2 exhibited low amplitude and delayed latency. MRI confirmed asymmetric nerve swelling in the regions of the bilateral median nerves with motor nerve conduction block. Lumbar MRI detected nerve swelling in the peripheral nerves distant from the lumbar ganglion. MRI detected no nerve swelling in the arachnoid space such as the cervical cord, thoracic cord, or cauda equina. Aggravation of CIDP was treated with steroids and immunoglobulin. Diplopia occasionally appeared, but was not consistent with aggravation of CIDP. The bilateral supraorbital nerves remained unchanged for three years on MRI.     

Multiple focal cortical dysplasias presenting with intractable epilepsy: case report]

A 14-year-old female, who had intractable epilepsy associated with multiple focal cortical dysplasias (FCD), was reported. She developed intractable epilepsy at the age of 7 and was diagnosed as having frontal lobe epilepsy based on the seizure semiology and interictal EEG. MRI revealed multiple lesions in the right frontal, bilateral occipital and left parietal lobes. EEG demonstrated that ictal discharge was preceded by spike on the right frontal region and FDG-PET showed hypometabolic area in the right frontal lobe. Chronic subdural electrode recordings from the right frontal lobe indicated that ictal onset zone was located around the right frontal lesion, especially frontal tip and base, and these areas including the lesion were resected. Postoperatively, residual seizure was noted although seizure frequency was decreased. It is well known that, postoperatively, satisfactory seizure outcome can be obtained in patients with FCD. However, further investigation in terms of surgical indication and strategies for multiple FCD should be needed.     

Brief report: stroke in multiple myeloma patient treated with thalidomide]

We presented a patient suffered from stroke related to thalidomide therapy. The patient was a 74-year-old man who had about two-year history of multiple myeloma and treated with 100 mg of oral thalidomide daily. He was diagnosed as having cryptogenic stroke attributable to patent foramen ovale, when he admitted to our hospital with sudden onset left-side hemiparesis. Antiplatelet and neuroprotective therapies were commenced along with the use of elastic stocking to prevent further embolic event. Then, warfarin was selected as secondary prevention to reduce the risk of paradoxical embolism during thalidomide therapy. Although the risk of deep vein thrombosis on thalidomide therapy has been well documented, only a few cases have been noted documenting the risk of stroke during thalidomide therapy. We need to be careful about the risk of deep vein thrombosis on thalidomide therapy, even as monotherapy, and consider using anticoagulant therapy while prescribing thalidomide.     

Multiple myeloma with cauda equina infiltration

We report a case of a 51-year-old man with multiple myeloma who presented with lumbar pain and left limb paresis. Cerebrospinal fluid, brain and spinal cord resonance imaging revealed a diffuse infiltration of the cauda equina without any cranial leptomeningeal enhancement. An infiltration limited to the cauda equina is extremely rare.     

Extraosseous epidural IgD myeloma presenting with compression myelopathy

An 85-year-old man presented with acute transverse myelopathy: flaccid paraplegia, superficial and deep sensory disturbance below Th6 and loss of urinary sensation. Spinal magnetic resonance image showed an epidural mass compressing the spinal cord at the level of C7 to Th2. Immunoelectrophoresis revealed the presence of immunoglobulin D (IgD)-lambda M protein. Chemotherapy markedly improved both the haematologic aspect and tumor size, but not the motor deficit. In IgD myeloma, extraosseous spreads occur frequently, but extraosseous epidural tumors causing compression myelopathy are relatively rare. To our knowledge, this is the fourth report of cases.     

Necrotizing myelopathy associated with multiple myeloma

A case of progressive necrotizing myelopathy occurring in the context of multiple myeloma is described, and the pathology illustrated. This association has not been previously reported.     

Multiple sclerosis presenting with fantastic confabulation

Multiple sclerosis (MS) is the most common inflammatory, demyelinating disease of the central nervous system. Besides neurological signs and symptoms, the disease may be associated with various psychiatric manifestations, and uncommonly, psychiatric manifestations may be the presenting symptom. On the other hand, confabulation is defined as falsification of memory occurring in clear consciousness in association with an organically derived amnesia or as spontaneous narrative reports of events that never happened. We report an unusual association of confabulation with MS. A 23-year-old man was admitted to a hospital, telling unusual stories. After evaluation and a detailed workup, the patient was given the diagnosis of MS with fantastic confabulations. Neuropsychological evaluation revealed mild frontal lobe dysfunction. Confabulation has been reported after frontal lobe lesions and is also hypothesized to be associated with a deficit primarily in the retrieval of more than one of the following: encoding, consolidation or storage. Clinicians' awareness of this kind of rare association may further enlighten the neural basis of confabulation.     

Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma.

Multiple myeloma and plasmacytoma are rare causes of mass lesions at the skull base and cavernous sinus. Sixth nerve palsy, in isolation or in combination with other cranial neuropathies, may occur rarely as the initial presenting feature of multiple myeloma. We report the neuro-ophthalmologic, radiologic, and pathologic findings for two patients who developed sixth nerve palsies as an initial manifestation of intracranial plasmacytoma and multiple myeloma. One patient presented with an isolated sixth nerve palsy in the setting of multiple vasculopathic risk factors. Treatable skull base lesions, including plasmacytoma and multiple myeloma, must be considered in patients with sixth nerve palsies, especially among those who demonstrate a progressive course or multiple cranial neuropathies.     

Intracranial plasmocytoma manifesting multiple myeloma: apropos of a case]

Neurological complications of myeloma are multiple and various. Cranial and intracranial locations have been rarely reported. They can be classified into three clinical groups: (1) cranial nerve palsies secondary to single or multiple lesions in the base of the skull, (2) intraorbital tumors, (3) intracranial tumors, either cranial myeloma extending intracranially or pure intracranial tumor (dural and/or cerebral). In our case, macroscopic and microscopic examination of the brain showed dural and cerebral involvement, confirmed by immunohistochemical studies. The radiological features are discussed (CT scan, MRI, angiography). To our knowledge, a single report of intracranial plasmacytoma documented by MRI has been reported in the literature.     

Progressive multifocal leucoencephalopathy associated with multiple myeloma

Summary A case of progressive multifocal leucoencephalopathy is described associated with multiple myeloma (Kahler's disease).

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Résumé Un cas de leucoencéphalopathie progressive multifocale associée avec de myélome multiple (maladie de Kahler) est rapporté.

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With 2 Figures in the Text     

Multiple intracranial aneurysms presenting with epistaxis.

Although epistaxis secondary to rupture of a post-traumatic, false aneurysm is well documented, true intracavernous carotid artery aneurysms presenting with epistaxis are very rare. We present such a case in a patient with multiple aneurysms. The aneurysm was treated by endovascular techniques and the patient made a good recovery. This case highlights the need to distinguish between true and false aneurysms for both diagnostic and therapeutic reasons.