Dermatomyositis sine dermatitis (DMSD) is a rare autoimmune disease. It's distinguished from classical dermatomyositis (DM) by a lack of skin involvement. DM is known to have a variety of extramuscular manifestations, including interstitial lung disease, myocarditis, and dysphagia. However, peripheral nervous system involvement in DM termed "Neuromyositis" is less often encountered. Neuromyositis diagnosis is controversial due to its rarity, unknown mechanism, and heterogeneity of nerve pathology findings. A 53-year-old woman presented to our hospital following a fall. Six months prior to presentation, she had a sensory disturbance in her right foot. On admission, she had a right foot drop that progressed to right then left lower extremity weakness. A biopsy of the superficial peroneal nerve and long peroneal muscle showed large fiber nerve axonal loss, CD20 B-cell and CD4 T-cell predominant inflammatory infiltrate involving the perimysial connective tissue of the muscle, as well as myocyte hypertrophy and fibrosis with type I fiber predominance. These findings were compatible with dermatomyositis with neuropathic features. Electrophysiological studies of lower extremities revealed severe widespread axonal dysfunction, as evidenced by decreased tibial compound muscle action potential (CMAP), no peroneal motor responses, absent sural sensory nerve action potential (SNAP), and extensive active denervation throughout the left lower extremity. Three months later, she developed bilateral upper extremity weakness. A biopsy of the deltoid muscle that was done eight months after admission showed CD20 B-cell and CD4 T-cell predominant inflammatory infiltrates involving the perimysial connective tissue. These findings were pathologically similar to the first biopsy. Subsequently, a repeat electromyography (EMG) of upper extremities revealed myopathic changes with normal nerve conductions. She ultimately became quadriplegic and ventilator-dependent nine months after admission. She never exhibited any skin findings throughout her course of illness. An extensive imaging and laboratory workup did not reveal any occult malignancy, inflammation, or nutritional deficiency. Our patient did not respond to steroids or intravenous immunoglobulin (IVIg) and ultimately passed away. The clinical, pathological, and electrophysiological features suggested the presence of neuromyositis. This case illustrates the importance of recognizing peripheral nervous system involvement as a significant and yet underreported extramuscular manifestation of DM. There are currently no formal management guidelines for neuromyositis.

lobes (endobronchial spread of infection in right middle lobe and right lower lobe). She was admitted to our hospital for further evaluation.Infectious diseases and Pulmonary were involved in the case, she was in airborne isolation and bronchoscopy was performed to rule out pulmonary tuberculosis. The acid-fast bacillus sputum culture (AFB) was negative x3, patient underwent bronchoalveolar lavage (BAL), which was negative for PCP and AFB. Serum Cyptocococcal neoformans capsular polysaccharide antigen was positive CRAg (1:2560), repeated was1:20, Absolute CD4 count was7. Initial CSF opening pressure was 36, WBCs 0, RBCs 2, colorless, protein 18.8, glucose 43, CSF fungal culture showed positive encapsulated yeast, India ink microscopy.Meanwhile, the patient was receiving treatment with Flucytosine, Amphotericin B and Cefepime. She underwent serial therapeutic lumbar puncture for 14 days. Despite the treatment with antifungal and serial LP, the patient had persistently raised CSF pressure. She did not complain of neuropsychiatric symptoms or signs such as neck stiffness, headache, dizziness, photophobia, lethargy, altered mental status, personality changes or memory loss. Brain CT was negative for hydrocephalus. Neurosurgery was involved for placement of lumbar drain or ventricular drain, either directly draining externally or a ventricular-peritoneal shunt. Ophthalmology was consulted to assess for papilledema, which was negative. As the patient was asymptomatic denied headaches, visual changes, or symptoms associated with elevated intracranial pressure (ICP) V-P shunt was not performed. The WBCs was serially followed, which showed a drop of all cell line after induction treatment with Flucytosine, Amphotericin B and Cefepime. The patient remained asymptomatic during the entire clinical course. The final report of BAL analysis: Grocott stain positive for yeast form fungal microorganisms, Cryptococcus. The mucicarmine stain showed highlights organism with positive capsular staining supporting the possibility of Cryptococcus infection. The patient didn't complete the treatment, she left the facility against medical advised (AMA) and was lost follow-up.

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